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Annals of Clinical and Medical
Case Reports
Review Article Volume 5
ISSN 2639-8109
Sexual Function and Dysfunction among Patients with Systemic and Auto-Immune
Diseases
Frikha F1*
, Masmoudi J2
, Salah RB1
, Ghribi M1
and Bahloul Z1
1
Department of Internal Medicine Hedi Chaker Hospital 3029 Sfax-Tunisia. Medical University School of Sfax, Tunisia
2
Department of Psychiatry A. Hedi Chaker Hospital 3029 Sfax-Tunisia. Medical University School of Sfax, Tunisia
*Corresponding author:
Faten Frikha,
Department of Internal Medicine Hedi Chaker
University Hospital, Medical University School
of Sfax, Tunisia, E-mail: fetenfrikha@yahoo.fr
Received: 21 Jan 2021
Accepted: 18 Feb 2021
Published: 22 Feb 2021
Copyright:
©2021 Frikha F et al., This is an open access article dis-
tributed under the terms of the Creative Commons Attri-
bution License, which permits unrestricted use, distribu-
tion, and build upon your work non-commercially.
Citation:
Frikha F. Sexual Function and Dysfunction among
Patients with Systemic and Auto-Immune Diseases. Ann
Clin Med Case Rep. 2021; V5(11): 1-9
Keywords:
Sexuality; Sexual dysfunction; Systemic
Diseases; Auto-immune diseases
1. Summary
Systemic autoimmune diseases affect various organs and they can
determine sexual dysfunction in females and males patients par-
ticularly with rheumatoid arthritis (RA), systemic lupus erythe-
matosus (SLE), systemic sclerosis (SSc), Sjögren syndrome (SS),
antiphospholipid syndrome, Behçet’s disease and other vasculitis.
Sexual dysfunction among patients with rheumatic diseases is
multifactorial due to disease-related factors (chronic disease as-
pects, disease activity) as well as therapy.
There is little data regarding the impact of these diseases and
treatments, mainly with large population, on sexual function. In
rheumatoid arthritis patients, sexual dysfunction is principally
associated with pain and depression. In patients with SLE, Sjög-
ren’s syndrome and systemic sclerosis, vaginal discomfort or pain
during intercourse could be the principal factors contributing to
sexual dysfunction. Sexual dysfunction is probably associated
with psychological status and neurological involvement in patients
with BD.
This systematic review synthesizes the current literature concer-
ning sexual function and dysfunction in patients with systemic and
auto-immune diseases. Our attention was directed specifically on
the sexual function and dysfunction of patients affected by SLE,
systemic Sclerosis, primary Sjogren syndrome, antiphospholipid
syndrome and Behçet’s disease.
2. Introduction
Sexuality is a complex aspect of the human being’s life and is clo-
sely associated to the quality of life (QoL). Sexuality has been
cited as an important part of the whole person, and sexual expres-
sion has been described as a crucial part of personal’s self-identity
[1]. Normal sexual functioning is important for healthy and all in-
dividuals and consists of sexual activity with transition through
the phases from arousal to relaxation with no problems, and with a
feeling of pleasure, and satisfaction [2,3].
Sexual dysfunction refers to a term that encompasses problems
that affect any phase of sexual responses. Sexual dysfunction
in females mainly includes desire, arousal, orgasmic, as well as
sexual pain disorders whereas males encounter erectile dysfunc-
tion, diminished libido, and abnormal ejaculation [4,5].
Systemic autoimmune diseases affect various organs and they can
determine sexual dysfunction in patients particularly with rheuma-
toid arthritis (RA), systemic lupus erythematosus (SLE), systemic
sclerosis (SSc), Sjögren syndrome (SS), Behçet’s disease (BD)
and other vasculitis.
Sexual dysfunction among patients with rheumatic or systemic au-
to-immune diseases is multifactorial due to disease-related factors
(chronic disease aspects, disease activity) and drugs. Many factors
including pain, stiffness, fatigue, functional impairment, negative
body image, reduced libido, hormonal imbalance, depression and
anxiety, contribute to reduce an individual’s sexual interest and
lead to a less active and enjoyable sex life [6].
Because of sexuality may be a taboo subject to many people,
sexual health is rarely addressed by health professionals during
routine consultations and is rarely proactively reported by patients.
In other hand, sexual dysfunction can cause frustration and distress
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and can impact both a couple’s relationship and sexual satisfaction.
In routine clinical practice, the focus is typically on fertility, pre-
gnancy or contraception, whereas sexual function is not addressed.
There is little data regarding the impact of these diseases and treat-
ments, mainly with large population, on sexual function.
In rheumatoid arthritis, sexual dysfunction is principally asso-
ciated with pain and depression. In patients with SLE, Sjögren’s
syndrome and systemic sclerosis, vaginal discomfort or pain
during intercourse could be the principal factors contributing to
sexual dysfunction. Moreover, the disturbances of sexual function
may also be attributed to psychological and cultural factors [6,7].
To date, little is known about the prevalence and the characteristics
of sexual dysfunction among patients with systemic and auto-im-
mune diseases suche as SLE, SSc, SS, antiphospholipid syndrome
and Behçet’s disease. Thus, this paper aimed to investigate the im-
pact of those diseases on patients’s sexual function by performing
a systematic review of studies available in the literature.
3. Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is a connective-tissue disor-
der, chronic inflammatory autoimmune disease of unknown etio-
logy that can affect multiple organs (skin rash, arthritis, serositis,
nephritis, pneumonitis, cardiac disease, hematological problems
and neurological involvement) [8,9].
Various studies have revealed that SLE can exert detrimental in-
fluences on different aspects of patients’ life, leading to an impair-
ment of psychological health (depression and low self-esteem),
health-related quality of life, and sexual function [10,11,12] in
both males and females.
Sexual function is of particular significance for SLE patients be-
cause it occurs predominantly in women, especially young wo-
men, at a ratio of 9 :1 (women : men) and plays a vital role in
quality of life [13]. The impact of SLE on sexual functioning has
been studied in previous studies and more evidence suggests that
an important part of QoL, sexual function is affected by SLE. The
proportion of SLE patients reporting sexual dysfunction across
studies ranged between 15% to 85.9% [10,13-16].
Shen et al. Found that the frequency of self-reported impaired
sexual function among patients with SLE was significantly higher
than healthy individuals (64.10% vs. 35.7%, p<0.001) [13].
Curry et al. [17] found that patients with SLE, when compared
with controls, had a significantly higher rate of abstention, a lower
frequency of sexual activity among the sexually active, diminished
vaginal lubrication, poorer general sexual adjustment, and more
depression. In this study, sexual dysfunction in f SLE patients may
be associated with age, relationship status, weight concerns, pre-
morbid sexual adjustment, and depression
In a review, the author reported that sexual dysfunction in women
with SLE is apparently most associated to vaginal discomfort or
pain during intercourse [3].
In a cross-sectional study, Morales et al [18] found that women
with SLE had a lower desire, lubrication, and orgasm. The results
of a recent meta-analysis showed that female SLE patients have
a lower total FSFI score, compared with healthy controls, which
means that SLE had some influence on women’s sexual function
[8]. Male patients with SLE might be also more vulnerable to
sexual dysfunction [10]. In fact, an increased prevalence of impo-
tence has been suggested [19].
However, other studies did not find a negative relationship between
SLE and sexual dysfunction. Tseng et al. [20] reported that female
patients with SLE were not more inclined to experience impaired
sexual function than the control population (52.5% vs. 47.1%,
p¼0.206).
SLE touches on all aspects of life including sexuality and repro-
duction. The pathogenesis of sexual problems in SLE patients is
still unclear. The reasons for inhibited sexual functioning and inef-
ficient reproduction are multifactorial and comprises of disease-re-
lated factors as well as medication [6]. Previous studies have
found that sexual function is significantly related to depression in
female SLE patients [21] and depression affects about 40% of SLE
patients [22]. Recent studies have shown that impairment of the
normal structure and function of microglia, caused by autoimmune
diseases, can result in depression and associated impairments in
neuroplasticity and neurogenesis [23].
3.1. Disease-Related Factors
In SLE patients, two main disease-related factors account for the
sexual dysfunction : diminished sexual drive and difficulties in
performing sexual intercourse [10].
The impairment of motion, fatigue, stiffness and pain of knee or
hip joints also limited sexual activity [10].
It is recognized that the androgenic status could be related with
sexual function including sexual desire and satisfaction. Female
SLE patients often had a higher level of estrogen but a lower level
of testosterone and dehydroepiandrosterone. In addition, vaginal
dryness and dyspareunia associated with secondary Sjogren’s syn-
drome are frequently found in women with SLE [6,10].
In males, erectile dysfunction can result from arterial and ve-
nous-occlusive insufficiency because of the lupus nephritis [24].
Several studies revealed that an increased prevalence of renal in-
volvement and progressive renal damage among males with SLE
compared to females [25]. Furthermore, sexual dysfunction is re-
latively more common among patients with renal dysfunction and
chronic kidney disease [26].
3.2. Psychological Factors
The psychological consequences of chronic disease also play a cri-
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Volume 5 Issue 11-2021 Review Article
tical role in the development of sexual dysfunction and therefore
should not be neglected. Patients with SLE often have a higher
level of depression and anxiety, a lower quality of life, and more
negative attitudes towards their body image. The SLE patients are
often encumbered with disfigurement and disabilities caused by
skin rashes, hair loss, and joint deformities [27].
Boomsma et al., [28] reported that 14% of SLE patients had sui-
cidal ideation, and half of them described that their disease had a
great impact on their daily lifestyles.
Pinto et al. [29] showed that when factors such as a higher dose of
steroids and disease activity were disregarded, it becomes apparent
that depression, anxiety, and marital satisfaction might impair the
sexual function of SLE patients.
All these combined effects of psychological factors might impair
patients’ sexual desire and function.
3.3. Drugs Factors
Glucocorticoid agents used in the treatment of SLE might also ne-
gatively impair sexual function and sexual satisfaction. High doses
of glucocorticoids can reduce testosterone levels, which plays a
crucial role in sexual dysfunction [30]. Glucocorticoids are known
to exert an inhibitory effect on sex hormones, or cause Cushing’s
Syndrome and other side effects during treatment. The glucocor-
ticoids can also induce hypothalamic-pituitary-adrenal (HPA) axis
dysregulation and result in persistently low sexual desire [31].
The use of Non-steroidal anti-inflammatory drugs (NSAID) can
increased risk of erectile dysfunction [32]. Cyclophosphamide can
interfere with DNA repair mechanisms and lead to gonadal toxi-
city. This cytotoxic alkylating agent can also lead to decreased sex
drive or erectile dysfunction [33].
In summary, most studies suggested that SLE is significantly asso-
ciated with an increased risk of sexual dysfunction in both female
and male patients. However, all conclusions need to be carefully
evaluated. In fact, the literature quality of included studies was
inadequate and the sample size of the available studies was insuf-
ficient small and limited. Substantial heterogeneity was also found
in the published studies. Further studies of a larger population of
SLE patients are required to further evaluate the mechanism by
which SLE affects sexual function.
4. Systemic Sclerosis
Systemic Sclerosis (SSc) is a chronic multisystemic disease cha-
racterized by abnormal fibrotic processes, inflammation and mi-
crovascular damage. The disease is most frequently seen at ages
between 30 and 50 years and the female/male ratio is 8/1.
The vascular alterations and immunological activation lead to
progressive and widespread fibrosis of multiple organ systems in-
cluding the skin, the lungs , the gastrointestinal tract and the kid-
neys. Changes associated with SSc such as skin tightening, muscle
weakness, joint pain, deformity and decreased physical function
can have a negative impact on sexuality and sexual functioning.
Sexual dysfunction and lower health quality are common problems
in female patients with SSc [34,35]. In the littérature, the number
of female patients with SSc reporting sexual dysfunction is higher
than those reported in the general population and also higher than
those reported in studies on other chronic diseases [36,37,38].
A study assessed the prevalence of sexual dysfunction in 163 pa-
tients with rheumatic diseases including SLE; rheumatoid arthri-
tis; systemic sclerosis; antiphospholipid antibody syndrome; and
fibromyalgia. The mean age was 40.4 years. The prevalence of
sexual dysfunction was 18.4%, but 24.2% of the patients reported
no sexual activity over the past 4 weeks. Patients with fibromyal-
gia and systemic sclerosis had the highest sexual dysfunction in-
dex (33%). Excluding patients with no sexual activity, the sexual
dysfunction rate reaches 24.2% [39].
A systematic review found that women with diffuse or limited SSc
experienced levels of sexual impairment similar to or higher than
women with breast cancer, HIV and gynaecological cancer [36].
Levis et al. [40] investigated sexual dysfunction and related clini-
cal symptoms in an evaluation of both sexually active and inactive
patients. The FSFI was applied to 165 sexually active SSc pa-
tients. The presence of Reynaud’s phenomenon and digital ulcers
and evaluation of the modified Rodnan skin score, gastro-intesti-
nal and pulmonary symptoms, and pain were investigated. Sexual
dysfunction was established in 102/165 (61.8%). This dysfunction
was higher in patients of advanced age with a high Rodnan skin
score and marked pulmonary symptoms.
A recent study included 30 sexually active female patients with
SSc and 30 healthy control subjects. Sexual dysfunction was
found in 26/30 (86.6%) of the SSc patients and in 6/30 (20%) of
the control group (P = 0.0001). Significant differences were deter-
mined between the groups with respect to sexual desire, arousal,
lubrication, orgasm, sexual satisfaction, and pain. There was no
significant relationship between the subgroups of SSc patients, du-
ration of disease, lung involvement, and FSFI scores. The SF-36
scores in the patient group were significantly lower than those in
the control group [34].
Impaired sexual functioning in women was probably less studied
due to the complexity and multifactorial nature of female sexual
response. A little bit more attention was paid to erectile dysfunc-
tion. Major problems that cause sexual dysfunction for male and
female patients with SSc are pain, limited physical ability, nega-
tive body image, fatigue and depression.
The exercise capacity of those patients is significantly reduced.
Symptoms such as dyspnea, fatigue, and cough in these patients
can also affect sexual activity in both sexes [41].
Raynaud’s phenomenon can affect the tongue and nipples, sclero-
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Volume 5 Issue 11-2021 Review Article
sis of the fingers and digital ulcers may interfere with touch and
foreplay.
Finally, fatigue, which is a common symptom of SSc, can decrease
quality of life by diminishing the ability to engage in meaningful
personal and social activities and has important implications for
employment, compliance with medical treatments and the use of
healthcare services [42].
4.1. Sexual functioning in women with systemic sclerosis
Bhaduria et al. [43] showed that more than half of systemic scle-
rosis patients reported a decrease in the number and intensity of
orgasms, compared to <20% of control subjects. Skin tightness,
reffux-heartburn, and muscle weakness adversely affected sexual
relations more in systemic sclerosis than in control subjects.
In female patients, the most common physical problems associated
with female sexual dysfunction include vaginal dryness, vaginal
stenosis, dyspareunia, Raynaud’s phenomenon, fatigue, joint pain,
joint contractures, muscle weakness and changes in breast skin.
All this factors lead to diminished libido, decreased vaginal lubri-
cation, and satisfaction. In addition, changes in the skin in these
patients, especially in the face, can affect self-confidence in wo-
men, which can also lead to sexual dysfunction [41,43].
When the skin become stiffer around the vaginal introitus, penetra-
tion becomes painful, and changes in the vaginal mucosae causing
lubrication disorder contribute to this painful penetration [3,41].
Raynaud’s phenomenon, which affect fingers , toes , tongue and
nipples, is another reason, why the cuddling, foreplay, and oral
sex could become uncomfortable and unpleasant.
In our study, the major reasons for decreased sexual activity in
married women with SSc were fatigue, altered body image, and
pain [44].
Physical problems, emotional problems and partnership difficul-
ties arising from disease relatedness contribute to a less active and
often less enjoyable sex life. The pleasure of intercourse can beco-
me diminished by pain of joint movement, or difficulty in finding
positions that do not cause discomfort.
The management of impaired sexuality in women with SSc was
less studied but there are some general recommendations for wo-
men that may help to continue enjoying an active, fulfilling sexual
life a medical provider, physical therapist, occupationaltherapist,
psychotherapist, and sex therapist [45].
4.2. Sexual functioning in male with systemic sclerosis
The prevalence of erectile dysfunction in men with SSc has been
reported to be approximately 12%–81% in different studies [3,46].
The exact pathogenesis of this erectile dysfunction in SSc is not
clearly defined.
Underlying vasculopathic, microangiopathic and fibrotic changes,
and neuropathic/disautonomic factors are currently thought to be
responsible [3,47]. It was proven that damage of the penile caver-
nous arteries occurs in almost all SSc patients regardless of clinical
symptoms. They are characterized by the presence of hyperechoic
spots, suggesting fibrotic changes and low peak systolic velocities
that are signs for vascular alterations [48]. Other risk factors of
erectile dysfunction such as smoking, hypertension, diabetes, and
steroid use have been investigated. It was found that only self-re-
ported history of nerve damage and diabetes are significant for pre-
dicting the likelihood of erectile dysfunction in SSc. Some studies
have reported that total testosterone and prolactin levels in blood
are correlated with erectile dysfunction in men with SSc [47].
Some treatment options have been used to cause the relaxation
of smooth muscle cells and temporarily increases arterial blood
flow in the penis. Phosphodiesterase-5 (PDE-5) inhibitors are re-
commended as a first-line option for pharmacotherapy. The most
commonly used are sildenafil, tadalafil, and vardenafil. Proeitti
et al. [48], found that once-daily tadalafil improves both vascular
measures of cavernous arteries and so erectile function.
It is also recommended to eliminate general cardiovascular risk
factors including lifestyle, psychological, or drug-related factors.
In summary, sexual dysfunction is a common problem in both
men and women with systemic sclerosis. Sexual dysfunction in
the female patient is considerably more complex and it has been
less studied. Erectile dysfunction is the dominant issue in males,
and seems to be tightly related to vascular dysfunction and fibrotic
changes. Further research regarding sexual dysfunction in patients
with systemic sclerosis is strongly needed.
5. Sjogren’s Syndrome
Sjogren syndrome (SS) is a chronic systemic autoimmune disease
of unknown origin characterized by sicca symptoms of the mouth
and eyes (xerostomia and xerophtalmia), together with a variety
of extraglandular symptoms (disabling fatigue, interstitial lung di-
sease, neurologic involvement, and arthritis). Primary SS (pSS) is
the second most common systemic autoimmune disease that usual-
ly affects women more than man.
While dryness of mucosal surfaces is the main characteristic fea-
ture of this disease, other mucosal surfaces can also be involved
such as nasal and vaginal mucosa. Women with pSS often expe-
rience vaginal dryness and dyspareunia which can play a major
role in sexual dysfunction and had the greatest impact on quality
of life [49]. Kissing can be diffcult and unpleasant due to the dry
mouth [3]. Many studies showed also that patients with pSS have
more depressive symptoms than healthy controls and that depres-
sion and fatigue is associated with higher ESSPRI scores [50] and
so an altered quality of life. Van Nimwegen et al. reported depres-
sion as being the most important predictor of sexual dysfunction
inpatients with SS [51].
Previous studies have largely focused on vaginal symptoms and
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Volume 5 Issue 11-2021 Review Article
dyspareunia, but only a few studies highlighted sexual dysfunction
and distress in women with SS. Maddali Bongi et al. [52] found
that 68% of the patients reported alterations in their sexual abi-
lity because of the symptoms of pSS, especially vulvar or vaginal
dryness, dyspareunia and reduced sexual drive.
In a recent study, Van Nimwegenet al [51] demonstrated that wo-
men with pSS have significantly more sexual dysfunction in the
domains of desire, arousal, orgasm, lubrication and pain compared
with healthy controls.
Sexual function has been measured by self administered question-
naire, namely the Female Sexual Function Index (FSFI), in three
included studies [51,53,54], and was compared between pSS pa-
tients (102 patients) and healthy controls (99 participants). A ran-
dom-effects model was used in all domains, and the pooled results
displayed significant difference between pSS patients and healthy
controls. The SMD of the FSFI scores of pSS patients were lower
than that of controls on each domain of sexual function: desire ,
arousal , lubrication , orgasm , satisfaction , pain and total FSFI.
Recently, Isik H et al [55] found that patients with SS had lower
sexual function scores compared to age- matched controls and
80.4% of them were sexually dissatisfied according to previously
determined cut-off values (FSFI score of <26). All domains of
sexual function, including desire, arousal, orgasm, lubrication,
pain, and satisfactionwere affected in these women. The authors
demonstrated that women who used lubricants experienced that
sexual satisfaction was improved with lubricant use, so it can be
advised to SS patientsby their physician even if they are premeno-
pausal [55]. The same study found that physical function scores in
life quality tests positively correlated with sexual function scores.
If a patient has a bet-ter physical function and less physical restric-
tion, she tends to have more satisfaction.
Two studies [51,53] found that sexual dysfunction does not corre-
late with the measurement assessed by physicians using the vali-
dated EULAR Sjogren’s Syndrome Disease Activity Index (ESS-
DAI), however it is more associated with the self-reported symp-
toms of the syndrome as rated by the EULAR Sjogren’s Syndrome
Patients’ Reported
Index (ESSPRI).
For sicca symptoms of pSS, patients can be offered local symp-
tomatic treatment of vaginal dryness with lubricants, topical oes-
trogens and moisturizers. Jozkowski et al [56] concluded that lu-
bricant use recommendations from health professionals and sex
educators could be helpful. If necessary, patients can be referred to
a gynaecologist or sexologist.
Treatment with biologic drugs (rituximab and abatacept) have a
beneficial effect on disease activity, fatigue and quality of life and
thus may also improve sexual function.
In summary, women with pSS experience significantly more
sexual dysfunction and depression than healthy controls. Sexual
function in pSS could be influenced by age, increased vaginal
dryness pain and fatigue, as well as psychological consequences
of the disease that can strongly alter the quality of patients life.
6. Antiphospholipid Syndrome
Antiphospholipid syndrome (APS) is a rare systemic autoimmune
disorder characterized by the occurence of venous or arterial
thrombosis and/or recurrent fetal loss, in addition to serum anti-
phospholipid antibodies (aPL), mainly lupus anticoagulant and an-
ticardiolipin antibodies (aCL). To date, few studies have evaluated
erectile function in subjects with primary APS [57].
Gallinaro et al [57] compared 11 APS male patients with 22
healthy. Erectile dysfunction was frequently observed in APS
versus controls (45.5 vs. 4.5%, p¼0.0096), especially moderate/
severe erectile dysfunction (p¼0.0081). The total International In-
dex of Erectile Functions (IIEF) score (49.6 vs. 67.1, p¼0.019),
erectile function (19.6 vs. 28.1, p¼0.005) and intercourse satisfac-
tion (7.8 vs. 11.9, p¼0.009) were lower in patients than in controls.
This alteration was linked to arterial events and longer disease du-
ration. Sadetski et al [58] demonstrated that almost all primary an-
tiphospholipid syndrome patients have psychological alterations.
In this study, sexual limitations was observed in 20 % of patients.
Erectile dysfunction in APS patients can be multifactorial. It is
reasonable to speculate that a thrombotic mechanism and the im-
pairment of the penile vascular system may be implicated in the
pathophysiology of this erection dysfunction. Other factors can be
associated with erectile dysfunction such as age, disease activity
and drug use [16].Some case reports showed thrombosis of penile
vessels leading to erectile dysfunction [59].
Rabelo-Júnior CN et al [60] observed normal testicular function
in a small group of primary antiphospholipid syndrome (PAPS)
patients, in spite of morphofunctional penile abnormalities. They
concluded that this alteration may lead to an issue about fertility
or fear of impaired sexual function (impotence) in male patients
with APS.
Another cross-sectional study was conducted by Rabelo-Júnior CN
et al [61] in ten SLE-APS male patients and 20 healthy controls.
The authors identified that intravenous cyclophosphamid was the
major factor for severe and potentially permanent damage to the
tests in SLE-APS patients. They observed for the first time an as-
sociation of reduced penile size with erectile dysfunction and pre-
vious arterial thrombosis in these patients.
In summary, male patients with APS may have erectile dysfunc-
tion and low intercourse Satisfaction. This sexual dysfunction is
probably associated with arterial events and duration of the disease
and may cause psychological problems with a consequent reduc-
tion of health-related quality of life.
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7. Behçet Disease
Behçet’s disease (BD) is an inflammatory disorder of unknown
etiology which is characterized by by recurrent aphthous stomati-
tis, genital ulceration and can be accompanied by dermatological,
ocular, neurological, intestinal, urogenital and cardiopulmonary
symptoms
Sexual dysfunction in male and female patients with BD has been
repoted in many studies [62-68] and it was associated with depres-
sion, arthritis and neurological and vascular involvement.
In fact, patients suffering from chronic recurrent and painful di-
sease such as BD may have negative feelings, distress and dissa-
tisfaction in all aspects of their life.
In male patients with BD, the prevalence of erectile dysfunction
varied from 43.9 to 90% among patients [66,69]. It has been re-
ported that erectile dysfunction according to the International In-
dex of Erectile Functions (IIEF) was significantly higher in pa-
tients with BD than control group. IIEF scores were negatively
correlated with BD Index in the patient group [63].
Koçak et al. Concluded that depressive mood correlates with the
sexual status of BD patients, and this may be because of the de-
pressive effect of BD as a chronic disease [62].
Yıldız et al. reported that the five parameters of IIEF score were
significantly lower in patients with BD (p < 0.001). The authors
found that the Hospital Anxiety and Depression Scale in patients
with BD were significantly higher than healthy control subjects (p
< 0.001). Sexual dysfunction was found to be associated with age,
duration of disease, psychological status and quality of life in this
suty [66].
Female sexual dysfunction is also common in the previous studies
and significantly associated with depression rather than to active
organic manifestations, such as genital ulcers [67].
Yetkin et al. [64] investigated sexual dysfunction and depression
in a group of premenopausal women with mucocutaneous BD;
The sutudy included two groups based on the presence or absence
of genital ulcerations and compared with a group of age-matched
healthy volunteers. The authors found that both depression and
sexual dysfunction were more common among BD patients than
in controls. In particular, depression and sexual fysfunction were
found in the 32% and 56% of the patient group, respectively (vs
14.8% and 41% of the control group, respectively). The pain do-
main of FSFI (Female Sexual Function Index) was significantly
higher in BD group than in control group.
The origin of sexual dysfunction in this vasculitis is multifactorial,
being related to different factors such as mucocutaneous involve-
ment, the high frequency of psychiatric involvement [67,71,72]
and to the side effects of various treatments used for treating the
disease.
In their study, Hiz et al. [63] suggested that sexual dysfunction
is an association with psychological status and history of arthritis
whereas Erdogru et al. [73] suggested that it was associated with
neurological involvement in patients with BD.
Vascular pathologies of BD can lead to sexual dysfunction [64] in
BD; moreover, mixed neurological and vasculogenic pathologies
can lead to sexual dysfunction in Neuro-Behçet’ disease.
In a recent study, Hayriye et al [68] have included prospectively
a total of 25 patients with neurological involvement, 22 patients
with Behçet’s disease (BD) without neurologic involvement and
19 persons in control group. A semi-structured interview guided
by a Arizona sexual experience scale (ASEX) questionnaire, Beck
Depression Inventory and Beck Anxiety Inventory was applied to
all of the patients. The sexual dysfunction was significantly hi-
gher in patients with neurological involvement (47.9%) compared
to BD patients without neurological involvement (35.4%) and
control group (16.7%) (p = 0.001). The authors showed that neu-
rological involvement in BD had a negative effect on sexuality in
all but especially on arousal and vaginal lubrication/penil erection
function based on ASEX questionnaire. This higher prevalence
of sexual dysfunction in neuro-Behçet’diseas may be a result of
derangement of parasympathetic pathways, limbic or other brain
areas, yet, the exact locations of the derangement are not known.
Moreover, male sexuality was more affected by BD than female
sexuality.
In another hand, it seems relatively frequent that patients with BD
develop a neurobehavioural syndrome, characterized by eupho-
ria, bipolar disorders and paranoid attitudes, loss of insight/disin-
hibition, and indifference to their disease, defined as ‘neuropsy-
cho-BD’ [67,70] and this may have a great impact on the quality
of life of these patients.
Aksu et al reported that the cause of erectile dysfunction in their
two patients with BD without neurological involvement was se-
vere venous leak shown by penile Doppler ultrasound and caver-
nosography [65].
Considering also the hormonal aspects of BD, Yetkin et al. showed
that BD patients have lower levels of total testosterone and
DHEASO4; which may allow to speculate on the presence of low
androgen levels that may also contribute to sexual dysfunction in
these patients [64].
Finally, treatment with glucocorticoids may also have side effects
with great impact on sexual
function, due to change in body image, as well as leading to de-
pression and psychosis. Furthermore, several drugs used to treat a
frequent comorbid condition may also have an impact on sexual
function. Tricyclic antidepressants and serotonin reuptake inhibi-
tors may lead to a significant decrease of libido [67].
In summary, such a systemic and complex disease like BD is at
http://www.acmcasereport.com/ 6
Volume 5 Issue 11-2021 Review Article
high risk of developing sexual dysfunction for many different or-
ganic and psychological reasons. Therefore, management strate-
gies including psychotherapeutic interventions and behavioral
based sexual therapies might help improve the quality of life in
patients with BD.
8. Conclusion
Sexuality is an often neglected area of quality of life in patients
with chronic systemic and auto-immune diseases. Manifestations
and symptoms of disease can impair sexual functioning, but this
can be much improved by adequate intervention and counseling.
Sexual dyfunction is multifactorial and it is influenced by physi-
cal as well as psychological consequences of the diseases, such
as pain, fatigue, stiffness, functional impairment, depression, an-
xiety, negative body image, reduced libido, hormonal imbalance
and side effects from treatments.
In order to improve the sexual health and quality of life of pa-
tients with systemic and auto-immune diseases, the management
of patients of both genders should include regular family planning
and sexual counselling. It is mendatory to implement for active
interventions that aimed to treat or prevent sexual dysfunction
among those patients. In addition, the presence of a psychologist
or trained health professionals could be helpful in association with
specific lifestyle clinical practice guidelines for patients. A multi-
disciplinary approach is essential in order to offer preventive mea-
sures for these patients.
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http://www.acmcasereport.com/ 9
Volume 5 Issue 11-2021 Review Article

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Sexual Function and Dysfunction among Patients with Systemic and Auto-Immune Diseases

  • 1. Annals of Clinical and Medical Case Reports Review Article Volume 5 ISSN 2639-8109 Sexual Function and Dysfunction among Patients with Systemic and Auto-Immune Diseases Frikha F1* , Masmoudi J2 , Salah RB1 , Ghribi M1 and Bahloul Z1 1 Department of Internal Medicine Hedi Chaker Hospital 3029 Sfax-Tunisia. Medical University School of Sfax, Tunisia 2 Department of Psychiatry A. Hedi Chaker Hospital 3029 Sfax-Tunisia. Medical University School of Sfax, Tunisia *Corresponding author: Faten Frikha, Department of Internal Medicine Hedi Chaker University Hospital, Medical University School of Sfax, Tunisia, E-mail: fetenfrikha@yahoo.fr Received: 21 Jan 2021 Accepted: 18 Feb 2021 Published: 22 Feb 2021 Copyright: ©2021 Frikha F et al., This is an open access article dis- tributed under the terms of the Creative Commons Attri- bution License, which permits unrestricted use, distribu- tion, and build upon your work non-commercially. Citation: Frikha F. Sexual Function and Dysfunction among Patients with Systemic and Auto-Immune Diseases. Ann Clin Med Case Rep. 2021; V5(11): 1-9 Keywords: Sexuality; Sexual dysfunction; Systemic Diseases; Auto-immune diseases 1. Summary Systemic autoimmune diseases affect various organs and they can determine sexual dysfunction in females and males patients par- ticularly with rheumatoid arthritis (RA), systemic lupus erythe- matosus (SLE), systemic sclerosis (SSc), Sjögren syndrome (SS), antiphospholipid syndrome, Behçet’s disease and other vasculitis. Sexual dysfunction among patients with rheumatic diseases is multifactorial due to disease-related factors (chronic disease as- pects, disease activity) as well as therapy. There is little data regarding the impact of these diseases and treatments, mainly with large population, on sexual function. In rheumatoid arthritis patients, sexual dysfunction is principally associated with pain and depression. In patients with SLE, Sjög- ren’s syndrome and systemic sclerosis, vaginal discomfort or pain during intercourse could be the principal factors contributing to sexual dysfunction. Sexual dysfunction is probably associated with psychological status and neurological involvement in patients with BD. This systematic review synthesizes the current literature concer- ning sexual function and dysfunction in patients with systemic and auto-immune diseases. Our attention was directed specifically on the sexual function and dysfunction of patients affected by SLE, systemic Sclerosis, primary Sjogren syndrome, antiphospholipid syndrome and Behçet’s disease. 2. Introduction Sexuality is a complex aspect of the human being’s life and is clo- sely associated to the quality of life (QoL). Sexuality has been cited as an important part of the whole person, and sexual expres- sion has been described as a crucial part of personal’s self-identity [1]. Normal sexual functioning is important for healthy and all in- dividuals and consists of sexual activity with transition through the phases from arousal to relaxation with no problems, and with a feeling of pleasure, and satisfaction [2,3]. Sexual dysfunction refers to a term that encompasses problems that affect any phase of sexual responses. Sexual dysfunction in females mainly includes desire, arousal, orgasmic, as well as sexual pain disorders whereas males encounter erectile dysfunc- tion, diminished libido, and abnormal ejaculation [4,5]. Systemic autoimmune diseases affect various organs and they can determine sexual dysfunction in patients particularly with rheuma- toid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren syndrome (SS), Behçet’s disease (BD) and other vasculitis. Sexual dysfunction among patients with rheumatic or systemic au- to-immune diseases is multifactorial due to disease-related factors (chronic disease aspects, disease activity) and drugs. Many factors including pain, stiffness, fatigue, functional impairment, negative body image, reduced libido, hormonal imbalance, depression and anxiety, contribute to reduce an individual’s sexual interest and lead to a less active and enjoyable sex life [6]. Because of sexuality may be a taboo subject to many people, sexual health is rarely addressed by health professionals during routine consultations and is rarely proactively reported by patients. In other hand, sexual dysfunction can cause frustration and distress http://www.acmcasereport.com/ 1
  • 2. and can impact both a couple’s relationship and sexual satisfaction. In routine clinical practice, the focus is typically on fertility, pre- gnancy or contraception, whereas sexual function is not addressed. There is little data regarding the impact of these diseases and treat- ments, mainly with large population, on sexual function. In rheumatoid arthritis, sexual dysfunction is principally asso- ciated with pain and depression. In patients with SLE, Sjögren’s syndrome and systemic sclerosis, vaginal discomfort or pain during intercourse could be the principal factors contributing to sexual dysfunction. Moreover, the disturbances of sexual function may also be attributed to psychological and cultural factors [6,7]. To date, little is known about the prevalence and the characteristics of sexual dysfunction among patients with systemic and auto-im- mune diseases suche as SLE, SSc, SS, antiphospholipid syndrome and Behçet’s disease. Thus, this paper aimed to investigate the im- pact of those diseases on patients’s sexual function by performing a systematic review of studies available in the literature. 3. Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a connective-tissue disor- der, chronic inflammatory autoimmune disease of unknown etio- logy that can affect multiple organs (skin rash, arthritis, serositis, nephritis, pneumonitis, cardiac disease, hematological problems and neurological involvement) [8,9]. Various studies have revealed that SLE can exert detrimental in- fluences on different aspects of patients’ life, leading to an impair- ment of psychological health (depression and low self-esteem), health-related quality of life, and sexual function [10,11,12] in both males and females. Sexual function is of particular significance for SLE patients be- cause it occurs predominantly in women, especially young wo- men, at a ratio of 9 :1 (women : men) and plays a vital role in quality of life [13]. The impact of SLE on sexual functioning has been studied in previous studies and more evidence suggests that an important part of QoL, sexual function is affected by SLE. The proportion of SLE patients reporting sexual dysfunction across studies ranged between 15% to 85.9% [10,13-16]. Shen et al. Found that the frequency of self-reported impaired sexual function among patients with SLE was significantly higher than healthy individuals (64.10% vs. 35.7%, p<0.001) [13]. Curry et al. [17] found that patients with SLE, when compared with controls, had a significantly higher rate of abstention, a lower frequency of sexual activity among the sexually active, diminished vaginal lubrication, poorer general sexual adjustment, and more depression. In this study, sexual dysfunction in f SLE patients may be associated with age, relationship status, weight concerns, pre- morbid sexual adjustment, and depression In a review, the author reported that sexual dysfunction in women with SLE is apparently most associated to vaginal discomfort or pain during intercourse [3]. In a cross-sectional study, Morales et al [18] found that women with SLE had a lower desire, lubrication, and orgasm. The results of a recent meta-analysis showed that female SLE patients have a lower total FSFI score, compared with healthy controls, which means that SLE had some influence on women’s sexual function [8]. Male patients with SLE might be also more vulnerable to sexual dysfunction [10]. In fact, an increased prevalence of impo- tence has been suggested [19]. However, other studies did not find a negative relationship between SLE and sexual dysfunction. Tseng et al. [20] reported that female patients with SLE were not more inclined to experience impaired sexual function than the control population (52.5% vs. 47.1%, p¼0.206). SLE touches on all aspects of life including sexuality and repro- duction. The pathogenesis of sexual problems in SLE patients is still unclear. The reasons for inhibited sexual functioning and inef- ficient reproduction are multifactorial and comprises of disease-re- lated factors as well as medication [6]. Previous studies have found that sexual function is significantly related to depression in female SLE patients [21] and depression affects about 40% of SLE patients [22]. Recent studies have shown that impairment of the normal structure and function of microglia, caused by autoimmune diseases, can result in depression and associated impairments in neuroplasticity and neurogenesis [23]. 3.1. Disease-Related Factors In SLE patients, two main disease-related factors account for the sexual dysfunction : diminished sexual drive and difficulties in performing sexual intercourse [10]. The impairment of motion, fatigue, stiffness and pain of knee or hip joints also limited sexual activity [10]. It is recognized that the androgenic status could be related with sexual function including sexual desire and satisfaction. Female SLE patients often had a higher level of estrogen but a lower level of testosterone and dehydroepiandrosterone. In addition, vaginal dryness and dyspareunia associated with secondary Sjogren’s syn- drome are frequently found in women with SLE [6,10]. In males, erectile dysfunction can result from arterial and ve- nous-occlusive insufficiency because of the lupus nephritis [24]. Several studies revealed that an increased prevalence of renal in- volvement and progressive renal damage among males with SLE compared to females [25]. Furthermore, sexual dysfunction is re- latively more common among patients with renal dysfunction and chronic kidney disease [26]. 3.2. Psychological Factors The psychological consequences of chronic disease also play a cri- http://www.acmcasereport.com/ 2 Volume 5 Issue 11-2021 Review Article
  • 3. tical role in the development of sexual dysfunction and therefore should not be neglected. Patients with SLE often have a higher level of depression and anxiety, a lower quality of life, and more negative attitudes towards their body image. The SLE patients are often encumbered with disfigurement and disabilities caused by skin rashes, hair loss, and joint deformities [27]. Boomsma et al., [28] reported that 14% of SLE patients had sui- cidal ideation, and half of them described that their disease had a great impact on their daily lifestyles. Pinto et al. [29] showed that when factors such as a higher dose of steroids and disease activity were disregarded, it becomes apparent that depression, anxiety, and marital satisfaction might impair the sexual function of SLE patients. All these combined effects of psychological factors might impair patients’ sexual desire and function. 3.3. Drugs Factors Glucocorticoid agents used in the treatment of SLE might also ne- gatively impair sexual function and sexual satisfaction. High doses of glucocorticoids can reduce testosterone levels, which plays a crucial role in sexual dysfunction [30]. Glucocorticoids are known to exert an inhibitory effect on sex hormones, or cause Cushing’s Syndrome and other side effects during treatment. The glucocor- ticoids can also induce hypothalamic-pituitary-adrenal (HPA) axis dysregulation and result in persistently low sexual desire [31]. The use of Non-steroidal anti-inflammatory drugs (NSAID) can increased risk of erectile dysfunction [32]. Cyclophosphamide can interfere with DNA repair mechanisms and lead to gonadal toxi- city. This cytotoxic alkylating agent can also lead to decreased sex drive or erectile dysfunction [33]. In summary, most studies suggested that SLE is significantly asso- ciated with an increased risk of sexual dysfunction in both female and male patients. However, all conclusions need to be carefully evaluated. In fact, the literature quality of included studies was inadequate and the sample size of the available studies was insuf- ficient small and limited. Substantial heterogeneity was also found in the published studies. Further studies of a larger population of SLE patients are required to further evaluate the mechanism by which SLE affects sexual function. 4. Systemic Sclerosis Systemic Sclerosis (SSc) is a chronic multisystemic disease cha- racterized by abnormal fibrotic processes, inflammation and mi- crovascular damage. The disease is most frequently seen at ages between 30 and 50 years and the female/male ratio is 8/1. The vascular alterations and immunological activation lead to progressive and widespread fibrosis of multiple organ systems in- cluding the skin, the lungs , the gastrointestinal tract and the kid- neys. Changes associated with SSc such as skin tightening, muscle weakness, joint pain, deformity and decreased physical function can have a negative impact on sexuality and sexual functioning. Sexual dysfunction and lower health quality are common problems in female patients with SSc [34,35]. In the littérature, the number of female patients with SSc reporting sexual dysfunction is higher than those reported in the general population and also higher than those reported in studies on other chronic diseases [36,37,38]. A study assessed the prevalence of sexual dysfunction in 163 pa- tients with rheumatic diseases including SLE; rheumatoid arthri- tis; systemic sclerosis; antiphospholipid antibody syndrome; and fibromyalgia. The mean age was 40.4 years. The prevalence of sexual dysfunction was 18.4%, but 24.2% of the patients reported no sexual activity over the past 4 weeks. Patients with fibromyal- gia and systemic sclerosis had the highest sexual dysfunction in- dex (33%). Excluding patients with no sexual activity, the sexual dysfunction rate reaches 24.2% [39]. A systematic review found that women with diffuse or limited SSc experienced levels of sexual impairment similar to or higher than women with breast cancer, HIV and gynaecological cancer [36]. Levis et al. [40] investigated sexual dysfunction and related clini- cal symptoms in an evaluation of both sexually active and inactive patients. The FSFI was applied to 165 sexually active SSc pa- tients. The presence of Reynaud’s phenomenon and digital ulcers and evaluation of the modified Rodnan skin score, gastro-intesti- nal and pulmonary symptoms, and pain were investigated. Sexual dysfunction was established in 102/165 (61.8%). This dysfunction was higher in patients of advanced age with a high Rodnan skin score and marked pulmonary symptoms. A recent study included 30 sexually active female patients with SSc and 30 healthy control subjects. Sexual dysfunction was found in 26/30 (86.6%) of the SSc patients and in 6/30 (20%) of the control group (P = 0.0001). Significant differences were deter- mined between the groups with respect to sexual desire, arousal, lubrication, orgasm, sexual satisfaction, and pain. There was no significant relationship between the subgroups of SSc patients, du- ration of disease, lung involvement, and FSFI scores. The SF-36 scores in the patient group were significantly lower than those in the control group [34]. Impaired sexual functioning in women was probably less studied due to the complexity and multifactorial nature of female sexual response. A little bit more attention was paid to erectile dysfunc- tion. Major problems that cause sexual dysfunction for male and female patients with SSc are pain, limited physical ability, nega- tive body image, fatigue and depression. The exercise capacity of those patients is significantly reduced. Symptoms such as dyspnea, fatigue, and cough in these patients can also affect sexual activity in both sexes [41]. Raynaud’s phenomenon can affect the tongue and nipples, sclero- http://www.acmcasereport.com/ 3 Volume 5 Issue 11-2021 Review Article
  • 4. sis of the fingers and digital ulcers may interfere with touch and foreplay. Finally, fatigue, which is a common symptom of SSc, can decrease quality of life by diminishing the ability to engage in meaningful personal and social activities and has important implications for employment, compliance with medical treatments and the use of healthcare services [42]. 4.1. Sexual functioning in women with systemic sclerosis Bhaduria et al. [43] showed that more than half of systemic scle- rosis patients reported a decrease in the number and intensity of orgasms, compared to <20% of control subjects. Skin tightness, reffux-heartburn, and muscle weakness adversely affected sexual relations more in systemic sclerosis than in control subjects. In female patients, the most common physical problems associated with female sexual dysfunction include vaginal dryness, vaginal stenosis, dyspareunia, Raynaud’s phenomenon, fatigue, joint pain, joint contractures, muscle weakness and changes in breast skin. All this factors lead to diminished libido, decreased vaginal lubri- cation, and satisfaction. In addition, changes in the skin in these patients, especially in the face, can affect self-confidence in wo- men, which can also lead to sexual dysfunction [41,43]. When the skin become stiffer around the vaginal introitus, penetra- tion becomes painful, and changes in the vaginal mucosae causing lubrication disorder contribute to this painful penetration [3,41]. Raynaud’s phenomenon, which affect fingers , toes , tongue and nipples, is another reason, why the cuddling, foreplay, and oral sex could become uncomfortable and unpleasant. In our study, the major reasons for decreased sexual activity in married women with SSc were fatigue, altered body image, and pain [44]. Physical problems, emotional problems and partnership difficul- ties arising from disease relatedness contribute to a less active and often less enjoyable sex life. The pleasure of intercourse can beco- me diminished by pain of joint movement, or difficulty in finding positions that do not cause discomfort. The management of impaired sexuality in women with SSc was less studied but there are some general recommendations for wo- men that may help to continue enjoying an active, fulfilling sexual life a medical provider, physical therapist, occupationaltherapist, psychotherapist, and sex therapist [45]. 4.2. Sexual functioning in male with systemic sclerosis The prevalence of erectile dysfunction in men with SSc has been reported to be approximately 12%–81% in different studies [3,46]. The exact pathogenesis of this erectile dysfunction in SSc is not clearly defined. Underlying vasculopathic, microangiopathic and fibrotic changes, and neuropathic/disautonomic factors are currently thought to be responsible [3,47]. It was proven that damage of the penile caver- nous arteries occurs in almost all SSc patients regardless of clinical symptoms. They are characterized by the presence of hyperechoic spots, suggesting fibrotic changes and low peak systolic velocities that are signs for vascular alterations [48]. Other risk factors of erectile dysfunction such as smoking, hypertension, diabetes, and steroid use have been investigated. It was found that only self-re- ported history of nerve damage and diabetes are significant for pre- dicting the likelihood of erectile dysfunction in SSc. Some studies have reported that total testosterone and prolactin levels in blood are correlated with erectile dysfunction in men with SSc [47]. Some treatment options have been used to cause the relaxation of smooth muscle cells and temporarily increases arterial blood flow in the penis. Phosphodiesterase-5 (PDE-5) inhibitors are re- commended as a first-line option for pharmacotherapy. The most commonly used are sildenafil, tadalafil, and vardenafil. Proeitti et al. [48], found that once-daily tadalafil improves both vascular measures of cavernous arteries and so erectile function. It is also recommended to eliminate general cardiovascular risk factors including lifestyle, psychological, or drug-related factors. In summary, sexual dysfunction is a common problem in both men and women with systemic sclerosis. Sexual dysfunction in the female patient is considerably more complex and it has been less studied. Erectile dysfunction is the dominant issue in males, and seems to be tightly related to vascular dysfunction and fibrotic changes. Further research regarding sexual dysfunction in patients with systemic sclerosis is strongly needed. 5. Sjogren’s Syndrome Sjogren syndrome (SS) is a chronic systemic autoimmune disease of unknown origin characterized by sicca symptoms of the mouth and eyes (xerostomia and xerophtalmia), together with a variety of extraglandular symptoms (disabling fatigue, interstitial lung di- sease, neurologic involvement, and arthritis). Primary SS (pSS) is the second most common systemic autoimmune disease that usual- ly affects women more than man. While dryness of mucosal surfaces is the main characteristic fea- ture of this disease, other mucosal surfaces can also be involved such as nasal and vaginal mucosa. Women with pSS often expe- rience vaginal dryness and dyspareunia which can play a major role in sexual dysfunction and had the greatest impact on quality of life [49]. Kissing can be diffcult and unpleasant due to the dry mouth [3]. Many studies showed also that patients with pSS have more depressive symptoms than healthy controls and that depres- sion and fatigue is associated with higher ESSPRI scores [50] and so an altered quality of life. Van Nimwegen et al. reported depres- sion as being the most important predictor of sexual dysfunction inpatients with SS [51]. Previous studies have largely focused on vaginal symptoms and http://www.acmcasereport.com/ 4 Volume 5 Issue 11-2021 Review Article
  • 5. dyspareunia, but only a few studies highlighted sexual dysfunction and distress in women with SS. Maddali Bongi et al. [52] found that 68% of the patients reported alterations in their sexual abi- lity because of the symptoms of pSS, especially vulvar or vaginal dryness, dyspareunia and reduced sexual drive. In a recent study, Van Nimwegenet al [51] demonstrated that wo- men with pSS have significantly more sexual dysfunction in the domains of desire, arousal, orgasm, lubrication and pain compared with healthy controls. Sexual function has been measured by self administered question- naire, namely the Female Sexual Function Index (FSFI), in three included studies [51,53,54], and was compared between pSS pa- tients (102 patients) and healthy controls (99 participants). A ran- dom-effects model was used in all domains, and the pooled results displayed significant difference between pSS patients and healthy controls. The SMD of the FSFI scores of pSS patients were lower than that of controls on each domain of sexual function: desire , arousal , lubrication , orgasm , satisfaction , pain and total FSFI. Recently, Isik H et al [55] found that patients with SS had lower sexual function scores compared to age- matched controls and 80.4% of them were sexually dissatisfied according to previously determined cut-off values (FSFI score of <26). All domains of sexual function, including desire, arousal, orgasm, lubrication, pain, and satisfactionwere affected in these women. The authors demonstrated that women who used lubricants experienced that sexual satisfaction was improved with lubricant use, so it can be advised to SS patientsby their physician even if they are premeno- pausal [55]. The same study found that physical function scores in life quality tests positively correlated with sexual function scores. If a patient has a bet-ter physical function and less physical restric- tion, she tends to have more satisfaction. Two studies [51,53] found that sexual dysfunction does not corre- late with the measurement assessed by physicians using the vali- dated EULAR Sjogren’s Syndrome Disease Activity Index (ESS- DAI), however it is more associated with the self-reported symp- toms of the syndrome as rated by the EULAR Sjogren’s Syndrome Patients’ Reported Index (ESSPRI). For sicca symptoms of pSS, patients can be offered local symp- tomatic treatment of vaginal dryness with lubricants, topical oes- trogens and moisturizers. Jozkowski et al [56] concluded that lu- bricant use recommendations from health professionals and sex educators could be helpful. If necessary, patients can be referred to a gynaecologist or sexologist. Treatment with biologic drugs (rituximab and abatacept) have a beneficial effect on disease activity, fatigue and quality of life and thus may also improve sexual function. In summary, women with pSS experience significantly more sexual dysfunction and depression than healthy controls. Sexual function in pSS could be influenced by age, increased vaginal dryness pain and fatigue, as well as psychological consequences of the disease that can strongly alter the quality of patients life. 6. Antiphospholipid Syndrome Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder characterized by the occurence of venous or arterial thrombosis and/or recurrent fetal loss, in addition to serum anti- phospholipid antibodies (aPL), mainly lupus anticoagulant and an- ticardiolipin antibodies (aCL). To date, few studies have evaluated erectile function in subjects with primary APS [57]. Gallinaro et al [57] compared 11 APS male patients with 22 healthy. Erectile dysfunction was frequently observed in APS versus controls (45.5 vs. 4.5%, p¼0.0096), especially moderate/ severe erectile dysfunction (p¼0.0081). The total International In- dex of Erectile Functions (IIEF) score (49.6 vs. 67.1, p¼0.019), erectile function (19.6 vs. 28.1, p¼0.005) and intercourse satisfac- tion (7.8 vs. 11.9, p¼0.009) were lower in patients than in controls. This alteration was linked to arterial events and longer disease du- ration. Sadetski et al [58] demonstrated that almost all primary an- tiphospholipid syndrome patients have psychological alterations. In this study, sexual limitations was observed in 20 % of patients. Erectile dysfunction in APS patients can be multifactorial. It is reasonable to speculate that a thrombotic mechanism and the im- pairment of the penile vascular system may be implicated in the pathophysiology of this erection dysfunction. Other factors can be associated with erectile dysfunction such as age, disease activity and drug use [16].Some case reports showed thrombosis of penile vessels leading to erectile dysfunction [59]. Rabelo-Júnior CN et al [60] observed normal testicular function in a small group of primary antiphospholipid syndrome (PAPS) patients, in spite of morphofunctional penile abnormalities. They concluded that this alteration may lead to an issue about fertility or fear of impaired sexual function (impotence) in male patients with APS. Another cross-sectional study was conducted by Rabelo-Júnior CN et al [61] in ten SLE-APS male patients and 20 healthy controls. The authors identified that intravenous cyclophosphamid was the major factor for severe and potentially permanent damage to the tests in SLE-APS patients. They observed for the first time an as- sociation of reduced penile size with erectile dysfunction and pre- vious arterial thrombosis in these patients. In summary, male patients with APS may have erectile dysfunc- tion and low intercourse Satisfaction. This sexual dysfunction is probably associated with arterial events and duration of the disease and may cause psychological problems with a consequent reduc- tion of health-related quality of life. http://www.acmcasereport.com/ 5 Volume 5 Issue 11-2021 Review Article
  • 6. 7. Behçet Disease Behçet’s disease (BD) is an inflammatory disorder of unknown etiology which is characterized by by recurrent aphthous stomati- tis, genital ulceration and can be accompanied by dermatological, ocular, neurological, intestinal, urogenital and cardiopulmonary symptoms Sexual dysfunction in male and female patients with BD has been repoted in many studies [62-68] and it was associated with depres- sion, arthritis and neurological and vascular involvement. In fact, patients suffering from chronic recurrent and painful di- sease such as BD may have negative feelings, distress and dissa- tisfaction in all aspects of their life. In male patients with BD, the prevalence of erectile dysfunction varied from 43.9 to 90% among patients [66,69]. It has been re- ported that erectile dysfunction according to the International In- dex of Erectile Functions (IIEF) was significantly higher in pa- tients with BD than control group. IIEF scores were negatively correlated with BD Index in the patient group [63]. Koçak et al. Concluded that depressive mood correlates with the sexual status of BD patients, and this may be because of the de- pressive effect of BD as a chronic disease [62]. Yıldız et al. reported that the five parameters of IIEF score were significantly lower in patients with BD (p < 0.001). The authors found that the Hospital Anxiety and Depression Scale in patients with BD were significantly higher than healthy control subjects (p < 0.001). Sexual dysfunction was found to be associated with age, duration of disease, psychological status and quality of life in this suty [66]. Female sexual dysfunction is also common in the previous studies and significantly associated with depression rather than to active organic manifestations, such as genital ulcers [67]. Yetkin et al. [64] investigated sexual dysfunction and depression in a group of premenopausal women with mucocutaneous BD; The sutudy included two groups based on the presence or absence of genital ulcerations and compared with a group of age-matched healthy volunteers. The authors found that both depression and sexual dysfunction were more common among BD patients than in controls. In particular, depression and sexual fysfunction were found in the 32% and 56% of the patient group, respectively (vs 14.8% and 41% of the control group, respectively). The pain do- main of FSFI (Female Sexual Function Index) was significantly higher in BD group than in control group. The origin of sexual dysfunction in this vasculitis is multifactorial, being related to different factors such as mucocutaneous involve- ment, the high frequency of psychiatric involvement [67,71,72] and to the side effects of various treatments used for treating the disease. In their study, Hiz et al. [63] suggested that sexual dysfunction is an association with psychological status and history of arthritis whereas Erdogru et al. [73] suggested that it was associated with neurological involvement in patients with BD. Vascular pathologies of BD can lead to sexual dysfunction [64] in BD; moreover, mixed neurological and vasculogenic pathologies can lead to sexual dysfunction in Neuro-Behçet’ disease. In a recent study, Hayriye et al [68] have included prospectively a total of 25 patients with neurological involvement, 22 patients with Behçet’s disease (BD) without neurologic involvement and 19 persons in control group. A semi-structured interview guided by a Arizona sexual experience scale (ASEX) questionnaire, Beck Depression Inventory and Beck Anxiety Inventory was applied to all of the patients. The sexual dysfunction was significantly hi- gher in patients with neurological involvement (47.9%) compared to BD patients without neurological involvement (35.4%) and control group (16.7%) (p = 0.001). The authors showed that neu- rological involvement in BD had a negative effect on sexuality in all but especially on arousal and vaginal lubrication/penil erection function based on ASEX questionnaire. This higher prevalence of sexual dysfunction in neuro-Behçet’diseas may be a result of derangement of parasympathetic pathways, limbic or other brain areas, yet, the exact locations of the derangement are not known. Moreover, male sexuality was more affected by BD than female sexuality. In another hand, it seems relatively frequent that patients with BD develop a neurobehavioural syndrome, characterized by eupho- ria, bipolar disorders and paranoid attitudes, loss of insight/disin- hibition, and indifference to their disease, defined as ‘neuropsy- cho-BD’ [67,70] and this may have a great impact on the quality of life of these patients. Aksu et al reported that the cause of erectile dysfunction in their two patients with BD without neurological involvement was se- vere venous leak shown by penile Doppler ultrasound and caver- nosography [65]. Considering also the hormonal aspects of BD, Yetkin et al. showed that BD patients have lower levels of total testosterone and DHEASO4; which may allow to speculate on the presence of low androgen levels that may also contribute to sexual dysfunction in these patients [64]. Finally, treatment with glucocorticoids may also have side effects with great impact on sexual function, due to change in body image, as well as leading to de- pression and psychosis. Furthermore, several drugs used to treat a frequent comorbid condition may also have an impact on sexual function. Tricyclic antidepressants and serotonin reuptake inhibi- tors may lead to a significant decrease of libido [67]. In summary, such a systemic and complex disease like BD is at http://www.acmcasereport.com/ 6 Volume 5 Issue 11-2021 Review Article
  • 7. high risk of developing sexual dysfunction for many different or- ganic and psychological reasons. Therefore, management strate- gies including psychotherapeutic interventions and behavioral based sexual therapies might help improve the quality of life in patients with BD. 8. Conclusion Sexuality is an often neglected area of quality of life in patients with chronic systemic and auto-immune diseases. Manifestations and symptoms of disease can impair sexual functioning, but this can be much improved by adequate intervention and counseling. Sexual dyfunction is multifactorial and it is influenced by physi- cal as well as psychological consequences of the diseases, such as pain, fatigue, stiffness, functional impairment, depression, an- xiety, negative body image, reduced libido, hormonal imbalance and side effects from treatments. In order to improve the sexual health and quality of life of pa- tients with systemic and auto-immune diseases, the management of patients of both genders should include regular family planning and sexual counselling. It is mendatory to implement for active interventions that aimed to treat or prevent sexual dysfunction among those patients. In addition, the presence of a psychologist or trained health professionals could be helpful in association with specific lifestyle clinical practice guidelines for patients. A multi- disciplinary approach is essential in order to offer preventive mea- sures for these patients. References 1. Hill J, H Bird, R Thorpe. Effects of rheumatoid arthritis on sexual activity and relationships. Rheumatology. 2003; 42: 280-6. 2. Prady J, Vale A, Hill J. Body image and sexuality. In: Hill J (ed) Rheumatology nursing: a creative approach. Churchill Livingstone, Edinburgh. 1998; 109-124. 3. Tristano AG. The impact of rheumatic diseases on sexual function. 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