Systemic means affects multiple organs. Lupus is the Latin word for wolf meaning disease affecting skin where the skin lesions look like wolf bite. Erythematosus means reddening of the skin. Systemic Lupus Erythematosus or SLE, sometimes also called just lupus is a disease that’s systemic and affects a wide variety of organs, but notably often causes red lesions on the skin. Systemic Lupus Erythematosus(SLE) is a chronic, nonspecific autoimmune inflammatory disease that typically affects multiple organs and systems, including the skin, joints, muscles, lungs, heart, kidneys, and the CNS and circulatory system. Individuals with SLE are noted with the production of antibodies and inflammatory responses that are mistakenly directed at their own tissue.

1. BY : DR. ZEEL NAIK
B.PHARM , PHARM.D(PB)
MALIBA PHARMACY COLLEGE

6.  Systemic means affects multiple organ.
 Lupus is Latin word for wolf meaning disease affecting skin
where the skin lesions looks like wolf bite.
 Erythematosus means reddening of the skin.
 Systemic Lupus Erythematosus or SLE, sometimes also called
just lupus is a disease that’s systemic, and affects a wide variety
of organs, but notably often causes red lesions on the skin.
 Systemic Lupus Erythematosus(SLE) is a chronic, nonspecific
autoimmune inflammatory disease that typically affects multiple
organs and systems, including the skin, joints , muscles, lungs,
heart , kidneys , and the CNS and circulatory system.
 Individuals with SLE are noted with production of antibodies
and inflammatory responses that are mistakenly directed at their
own tissue.

7.  This abnormal reaction can occur in any organ system.
 SLE is characterized by periods of disease activity or flares
followed by disease remissions, particularly in early stages
of the disease.
 The diagnosis of SLE can be difficult particularly in the
early stages of the disease because signs and symptoms are
often nonspecific.
 Other autoimmune inflammatory diseases such as RA,
Sjogren syndrome, scleroderma, Raynauds phenomenon
and systemic vasculitis often present with similar signs and
symptoms and should be distinguished from SLE by careful
clinical examination and laboratory testing.

8.  For most people , SLE is a mild, chronic disease
affecting mainly the mucocutaneous tissues, joints,
muscles characterized by skin rashes, arthritis ,
myalgia , and fatigue.
 For others , SLE may be rapidly progressive affecting
internal organs and systems leading to early mortality
from kidney failure secondary to glomerulonephritis,
thromboembolic events from antiphospholipid
syndrome (APS) , or severe infections from
pancytopenias.
 In patients with long-standing SLE, coronary artery
disease has become the leading cause of death.

9.  Mortality rates in patients with SLE are 3-5 times
higher than the general population.
 Although clinical manifestations of SLE are
heterogeneous , SLE can be distinguished from a
number of related cutaneous lupus syndromes (drug-
induced lupus erythematosus , discoid lupus, sub-
acute cutaneous lupus).

10.  SLE is predominantly a disease of younger women.
 Rarely, the first onset of SLE develops before puberty and
after menopause.
 The disease affects mostly females in their childbearing
years between 15-55 years, approximately nine times more
than men in this age group.
 90 % cases of SLE are of women.
 The female to male ratio is approximately 2:1 in children
and adults over the age of 55 years.
 Drug – Induced Lupus (DIL) erythematosus , where the
male to female ratio is equal, is the most common form of
lupus affecting older individuals from drug exposure, and it
is self-limiting brief illness if the causative agent is
identified and discontinued.

11.  The worldwide prevalence rate of SLE is o.1-0.2 %.
 African-American women are 3 times more as likely to
get affected as European-Americans.
 SLE is more common in women of Asia and Hispanic
descent.
 The mortality rate of SLE is 10-15%.

12.  The origin of SLE is unknown.
 It occurs at all ages, but is more common in young women.
 There is a strong genetic component associated with
susceptibility and severity possibly triggered by immunological,
hormonal and environmental factors.
 The disease tends to occur within families but without a clear
pattern of inheritance.
 Genetic factors have been identified in SLE.
 Common genetic markers seen in SLE patients are the genes for
human leukocytes antigen (HLA)-B8, DR-2 , and DR-3.
 Less common are the genes for HLA-DQw1 and DMA*o401.
 There may be other markers for this disease including
complement deficiencies , allelic variants or polymorphisms of
the Fc portion of immunoglobin G (IgG)(Fc Receptors) genes.

13.  Most cases of SLE flares are sporadic without identifiable predisposing
factor, with the exception of UV-B light.
 Multiple environmental and yet unknown factors may trigger the
disease.
 Chemicals such as hydrazine containing hair dyes, foods containing L-
canavanine such as alfalfa sprouts and microorganisms have also been
implicated to cause SLE in genetically susceptible individuals.
 Hormone levels can influence disease activity and expression via their
immunomodulatory effects.
 A high-estrogen , low androgen state has been implicated in the
pathogenesis of SLE.
 In SLE patients, low plasma testosterone, raised plasma luteinizing
hormone (LH) values, low progesterone, low dihydroepiandrosterone
(DHEA) have been observed.
 Increasing endogenous estrogen concentration has also been shown to
influence disease activity and prognosis of SLE.

14.  The administration of large doses of exogenous
estrogens in the form of high-dose estrogen containing
oral contraceptives(OC) and hormone replacement
therapy (HRT) may exacerbate the disease in patients
with existing lupus.
 Patients with Klinefelter syndrome, a condition
characterized by hypergonadotrophic hypogonadism,
are prone to SLE.
 Hyperprolactinemia has been reported in patients
with both sexes.

18.  Like a ton of other autoimmune diseases, it is not completely
clear why SLE develops and like most autoimmune diseases it is
the result of both genetic and environmental factor.
 The pathogenesis of SLE is unknown.
 If a person has susceptibility gene(genes that make him/her
susceptible to getting lupus) and he is exposed to UV radiations
in sunlight, which we know is an environmental risk factor for
SLE.
 Due to UV rays, things like sunburn can occur in which cell’s
DNA can become so badly damaged, that the cell undergo
programmed cell death, or apoptosis and it dies.
 This produces all the little apoptotic bodies and exposes the
inside of the cell, including parts of the nucleus, like DNA,
histones and other proteins, to the rest of the body.

19.  Well those susceptibility genes specifically have an effect
on the person’s immune system such that their immune
cells are more likely to think that these are foreign invaders
or antigens and since they are from the nucleus, we call
them nuclear antigens and immune cells try to attack
them.
 Susceptibility genes also cause to have less effective
clearance, essentially they are not as good as getting rid of
the apoptotic bodies and so they end up having more
nuclear antigens floating around.
 This means that B cells swing by, see them and start the
production of antibodies against these pieces of nucleus
which are called antinuclear antibody (ANA) and they are
present in all cases of lupus.

20.  So these antinuclear antibodies bind to the nuclear
antigens, forming antigen-antibody complex.
 These complexes can get into the blood and drift away and
deposit or stick to the vessel wall in all sorts of different
organs and tissues such as the skin, kidneys, joints , heart ,
etc,
 Deposited complexes then initiate a local inflammatory
reaction, which causes damage through the activation of
the complement system, which, after a huge cascade of
enzyme activation, leaves cell membranes with channels
that let fluid and molecules go in and out all willingly ,
causing the cell to burst and die, usually though you would
want this to happen to foreign cell or an infected cell, not
the healthy cells.

21.  When tissues become damaged as a result of these immune
complexes, its known as a type III hypersensitivity reaction.
 UV radiation is not the only way to damage cells, it is therefore is
not the only trigger that is thought to be associated with SLE.
 Other potential triggers that can be associated with SLE include
cigarette smoking, viruses, bacteria, use of certain medications
like procainamide, hydralazine and isoniazid, as well as sex
hormones, particularly estrogen .
 Nuclear antigens are considered as foreign bodies so immune
system initiates an immune response , creates anti-nuclear
antibodies that bind to antigens and then float around and
deposit in various tissues, which causes inflammation.
 These deposits and inflammation seem to be the cause of most
of the symptoms of lupus, which cause type III hypersensitivity
reaction.

22.  Many patients, though, also develop antibodies targeting
other cells like RBC and WBC and molecules like various
phospholipids, which can mark them for phagocytosis and
destruction , leading to additional symptoms.
 This is considered a type II hypersensitivity reaction ,
although it is not fully understood.
 Overproduction of IL-10 seems to play a role in SLE,
involving imbalance of IL-10 and IL-12.
 Other cytokines such as TNF-α , interferon gamma, IL-1,
IL-2, IL-4 , IL- 6 , IL- 16, IL- 17 and IL- 18 may be also
implicated.

23.  The best characterized organ pathology is the kidney.
 Lupus nephritis is common with SLE because immune
complexes are often deposited in the basement membrane
of the glomeruli.
 Renal biopsies in the patients with SLE reveal mesangial
cell proliferation, inflammation, basement membrane
abnormalities and immune complex depositions with light
immunofluorescence microscopy.
 Arteriosclerosis is a complication of long standing SLE
associated with hypertension (BP > 140/90), corticosteroids
, and other drugs.

24.  SIGNS AND SYMPTOMS:
 Clinical features are diverse.
 The clinical presentation of SLE is variable, and the signs and
symptoms may be very subtle especially early in the course of the
disease leading to delay to a delay in diagnosis because
symptoms are not always initially associated with the disease.
 CONSTITUTIONAL.
 Symptoms may not always appear concurrently.
 In the early stages, fatigue and general malaise , fever in the
absence of infection, and weight loss are the usual complaints.
 Fatigue is often the earliest manifestation of SLE , clearly the
most common symptom in patients with SLE.

25.  MUSCLOSKELETAL.
 Arthritis , arthralgia , and myalgia are described in all patients of SLE.
 Intermittent symmetric arthritis and arthralgia can be confused with
RA in the course of the disease.
 Patients typically present with polyarticular joint involvement and
complain mild to moderate pain in affected joint.
 It is non-erosive and non-deforming , usually affecting the joints of
hands , wrist , knees, and feet.
 Other symptomatology include diffuse puffiness or tenderness, and a
feeling of warmth in the affected joints.
 Tenosynovitis and bursitis may also be present.
 Myopathy may also occur during period of active disease or secondary
to treatment (e.g. corticosteroids , hydroxychloroquine).
 In approximately 30% patients , muscle pain due to fibromyalgia has
been reported.

26.  CUTANEOUS.
 Three main types of rash are associated with SLE.
 They can be distinguished by their mucocutaneous
manifestations on sunlight exposed areas , such as acute
cutaneous lupus , discoid lupus , subacute cutaneous lupus.
 Mucosal manifestations of the mouth and nose may be present.
 Acute cutaneous lupus is triggered by exposure to UV light or is
associated with exacerbation of systemic disease..
 The classic “butterfly rash” occurs in approximately 60% of
patients, which consist of an erythematous skin rash over malar
area of the cheeks and the bridge of the nose sparing the
nasolabial folds , oftentimes raised and very inflammed.
 The rash persists for weeks and resolves without scarring.

28.  Approximately 30% of SLE patients develop discoid
lupus erythematosus (DLE) , characterized by circular
rimmed , raised patches with keratotic scaling of the
scalp , face , or neck.
 The lesions are usually associated with disfigurement
from depigmentation and scarring of the skin.
 Permanent hair loss may develop if rash is located on
the scalp.

29. DISCOID LUPUS ERYTHEMATOSUS

30.  A distinct subset, subacute cutaneous lupus erythematosus
(SCLE) , is associated with symmetric , superficial , non-
scarring , ring shaped skin lesions with resultant alopecia.
 The lesions are located on the shoulders, upper arms, chest
, back, and neck.
 DLE AND SCLE usually have no other systemic
involvement.
 Mucosal manifestations are frequent in SLE.
 They present as painful, recurrent ulcers in the mouth ,
nose and genital cavity.
 Vasculitic skin lesions such as purpura , subcutaneous
nodules , nail fold infarcts , ulcers , urticaria , panniculitis ,
and gangrene may also develop in patients with SLE, DLE,
SCLE.

31.  The diagnostic workup includes an assessment of the
clinical presentation , physical examination ,
appropriate diagnostic tests , and consideration of an
alternative diagnosis.
 Many autoimmune diseases have overlapping features
so the exact classification may be difficult.
 Features of SLE , RA , Polymyositis , and scleroderma
may overlap.
 Drug-induced lupus erythematosus (DILE) should
always be included on the differential list.

33.  THE AMERICAN COLLEGE OF RHEUMATOLOGY (ACR) first developed diagnostic
criteria for SLE in 1971 that are highly specific and sensitive that has permitted
classification of patients for purposes of research , epidemiology , and clinical trials.
 The classification is based on 11 criteria.
 A person shall be said to have SLE if 4 or more of the 11 criteria are present, serially or
simultaneously during any interval of observation.
 The ACR revised criteria to have overall specificity of 95% and sensitivity of 85% in the
diagnosis of SLE.
 More than 95% of patients with symptomatic SLE have a positive ANA test with a very
high concentration which supports the diagnosis of SLE but the test is not specific
because it may indicate chronic liver or renal disease , infection , malignancy, or other
autoimmune disease such as RA.
 A negative ANA test makes the diagnosis unlikely , but not impossible.
 Anti-double stranded DNA (dsDNA) and anti-smith (SM) antibodies are unique to
pateint with SLE.
 A high titer of anti-dsDNA antibodies is most helpful confirmatory test for SLE and
increases the likelihood of lupus nephritis.

34.  SKIN
1. Malar Rash/Butterfly Rash
2. Discoid Rash
3. Photosensitivity or subacute cutaneous Rash.
4. Ulcers : Oral/Mouth or Nose.
5. Serositis (inflammation of outer membrane of
an organ or tissue) : Pleuritis , Pericarditis.
6. Arthritis : more than 2 joints.
7. Renal Disorder : Abnormal amount of urine
protein, glomerulonephritis.

35. 8. Neurologic Disorder : Seizures and Psychosis.
9. Hematologic Diorder : Hemolytic Anemia ,
Leukopenia , thrombocytopenia , lymphopenia.
10. Antinuclear Antibody (ANA).
11. Immunological disorder
a. Anti-smith : targets ribonucleoprotein
b. Anti- dsDNA : targets double stranded DNA.
c. Anti-phospholipid : usually target protein
bound to phospholipid.
*ANTI-SMITH and ANTI – dsDNA are more specific
for lupus.

36.  There are 3 types of antiphospholipid antibodies –
 I) Anticardiolipin (Sometimes False Positive for syphilis)
 II) Lupus Anticoagulant also known as lupus antibody.
 III) Anti – Beta 2 Glycoprotein-1.
 Sometimes because of these , patients with lupus develop
an Antiphospholipid syndrome where the
antiphospholipid antibodies cause a hypercoagulable state,
meaning they are more prone to developing clots and have
complications like deep vein thrombosis , hepatic vein
thrombosis and stroke.
 These patients often end up needing lifelong
anticoagulation therapy.

41.  Ancillary diagnostic imaging tests include chest X-ray ,
x-rays of affected joints , echocardiography for
evaluation of pericardial , myocardial , and
endocardial involvement , chest computed
tomography (CT) to define parenchymal lung disease ,
brain magnetic resonance imaging (MRI) to evaluate
patients with CNS lupus.

42.  Pleural involvement (SLE pleuritis) is the most common
respiratory components in patients with SLE and has been found
at autopsy in 40% patients.
 Symptoms include pleural pain , cough , dyspnea , and fever.
 Alveolar hemorrhage , a common process in patients with SLE.
 Rarely , patient develops acute lupus pneumonitis..
 Finally , 25% of SLE patients characteristically develop weakness
of diaphragm and other respiratory muscles with unexplained
dyspnea ( worse in the supine position).
 This is known as shrinking lung syndrome.
 It is characterized by low lung volume and the inability to
generate a respiratory effort.

43.  Cardiac complications involving pericardium , valves ,
myocardium , and coronary arteries are frequently seen in SLE
patients.
 Valvular heart disease is usually asymptomatic at onset. Further
examination may reveal valve leaflet thickening with or without
nonbacterial vegetations (LIBMAN-SACKS Endocarditis) that
may be a risk factor for CHF or emboli formation.
 Increased incidence of accelerated atherosclerosis in SLE has
been sho0wn.
 Dyslipidemia , hypertension , hyperglycemia , tobacco smoke
and obesity are greater risk factors for lupus related cardiac
manifestations.
 Patients with SLE have an increased risk of myocardial infarction
compared with the general population.
 MI and CAD are very common in lupus mainly in women in the
age group of 35-44 years.

44.  CNS manifestations are often nonspecific and diverse and
may occur during the course of disease in 60% of patients
with idiopathic SLE.
 Depression is most common.
 In addition , anxiety , migraine headaches , memory loss ,
and seizures have been reported.
 Frank psychosis , acute confusional states , demyelinating
disorders , movement disorders , aspetic meningitis ,
encephalopathy , myelopathy , monomyopathy or
polymyopathy of cranial or peripheral nerves , autonomic
dysfunction , acute demyelinating polyneuropathy (
Guillian – Barre), mood disorders , and optic neuritis are
also seen.

45.  50 % of patients develop renal disease from lupus nephritis , a
form of Glomerulonephritis , which is a poor prognostic
indicator.
 Anti-dsDNA deposits in the kidney leads to complement
activation and chemotaxis of neutrophils resulting in local
inflammation.
 According t0 WHO classification, SLE nephritis is associated
with six histologically distinct categories.
 Class I and II lesions have an excellent prognosis.
 Class III and IV lesions are associated with proliferative disease
requiring aggressive immunosuppressive treatment.
 Class V and Vl indicates ESRD.
 Urine analysis shows presence of RBC, proteinuria and markedly
reduced creatinine clearance.
 Nephropathy is a marker for lupus activity.

46.  Normochromic , normocytic anemia is seen in 40 % of SLE
patients.
 Hemolytic anemia , leukopenia , lymphopenia , and
thrombocytopenia may be present in up to 85 % of SLE patients.
 APS , a potentially hypercoagulable disdorder may occur in the
presence of SLE.
 The antibodies responsible are lupus antibibodies ,
anticardiolipin antibodies and anti-B2 Glycoprotein 1.
 APS is characterized by recurrent venous or arterial thrombosis,
spontaneous abortions or recurrent fetal loss , and
thrombocytopenia from antiphospholipid antibodies.
 Neonatal lupus syndrome is seen in some neonates born to
mother with SLE.
 A condition characterized by congenital heart block , rash , or
thrombocytopenia in the infant.

47.  Vasculities affecting small blood vessels in the skin ,
CNS , and coronary branch are seen in SLE.
 Vasculitic skin lesions include petechiae , purpura ,
urticaria , nodules , atrophy of the finger pads.
 GI manifestations such as nausea , diarrhea , and
vague discomfort are commonly reported in SLE
patients and may occur in up to 45 % of patients.
 Acute pancreatitis may be severe.

48.  Clinicians should be aware of the psychosocial issues when
treating patients with SLE.
 Some of the issues include patient frustration during the
diagnostic process , to feeling of isolation and depression
and family stresses.
 SLE shortens life expectancy , creates significant morbidity
, and accounts for significant health care expenditures.
 Symptoms control using conditioning exercises , applying
heat to affected joints , weight loss if necessary , and
relaxation techniques may help patients cope.

49.  A treatment plan is developed by health care providers
based on the patient's age, sex, overall health status ,
symptoms , and lifestyle.
 The goals of therapy are to prevent flares , treat active
disease , and to minimize organ damage and
complications.
 Treatment is individualized according to the disease
activity and the organ affected.
 Avoid sulfa antibiotics , oral contraceptives , and estrogen
products because they may cause flares.
 Regular monitoring of patient by their physicians is
mandatory every 3-6 months.
 General lifestyle modification should be encouraged in all
patients with SLE.

50.  Consider the combination of NSAIDs with or without HCQ in patient
with mild lupus.
 HCQ may be effective in controlling joint manifestations of the disease.
 Patients with inadequate response to this combination should be
prescribed low-dose corticosteroids whereas high-dose corticosteroids
are recommended in acute flares.
 A lupus rash can be treated with topical corticosteroids.
 Hydrocortisone topical may be effective.
 Ointments are used for dry lesions , whereas lotions are used for scalp
lesions.
 In mild to moderate cases, oral prednisone ( 60 mg daily) is given
initially until resolution of signs of active disease.
 More intensive and induction therapy using immunosuppressant with
methylprednisolone (+) or (-) cyclophosphamide , azathioprine , or
mycophenolate mofetil is used in patients with major organ or system
involvement( e.g. lupus nephritis , CNS lupus , or APS).

51.  NON-SELECTIVE NSAIDS and SELECTIVE COX-2
Inhibitors
 Used for symptomatic relief of joint pain and serositis ,
and pain associated with pleuritis and pericarditis.
 Also used for fever and fatigue.
 Naproxen ( 500 mg twice daily) and Ibuprofen ( 600
mg 4 times a day) are some of the NSAIDS.
 The use of proton pump inhibitors with NSAIDs
reduces the incidence of GI and duodenal ulcers.
 Celecoxib can be used.

52.  Combination therapy with an NSAID and HCQ is used
in patient with mild lupus whose symptoms are fatigue
, arthralgia , arthritis and rash.
 In addition , HCQ may have added benefit to SLE
patients due to its antithrombotic and lipid lowering
properties and steroid sparing effects.
 DOSE : 6.5 mg/kg/day and restricted to less than 600
mg per day.
 In SLE , the usual oral HCQ dose is 400 mg
administered daily for 4-12 weeks, and then tapered by
50% to daily maintenance dose of 200 mg.

53.  The addition of a short course of low dose corticosteroid
(e.g. prednisone) should be considered in patients poorly
responsive to the combination of NSAIDs and HCQ.
 Cutaneous manifestations may be effectively controlled
with topical corticosteroid formulation.
 Low dose oral prednisone (10 mg/day) may be used for
mild disease activity and limited to a duration of 4-6 weeks.
 NSAIDS can be combined with prednisone.
 Prednisone can also be helpful for treating acute flares with
or without systemic symptoms in initial adult doses of 1
mg/kg/day, up to 60 mg per day.

54.  MTX, a dihydrofolate reductase inhibitor , is an
alternative agent to HCQ and may be used with or
without low dose corticosteroids in persistent arthritis
, rash or serositis.
 Weekly doses of 7.5 mg to 15 mg (maximum 25 mg) of
MTX is effective and may control joint and skin
manifestations.

55.  Judicious use of high dose oral corticosteroids ,
including large IV doses, particularly in severe disease
is warranted.
 More intensive immunosuppression may be necessary
in patients with major organ involvement and life
threatening complications ( e.g. severe flares of joint
symptoms , lupus nephritis , or CNS Lupus) who fail
conventional corticosteroid therapy.
 Combination therapy with high dose corticosteroid (
e.g. oral prednisone 40-60 mg/day or IV
methylprednidolone 0.5-1 g/day) and pulse IV
cyclophosphamide is recommended.

56.  In patients diagnosed with diffuse proliferative
glomerulonephritis (class IV) , the cytotoxic agents of
choice, cyclophosphamide in combo with
methylprednisolone have been shown to slow down
disease progression , decrease the frequency of relapse
events , and reduce the risk of ESRD.
 Monthly pulse IV cyclophosphamide (0.5-1.0 g/m2 of
BSA) in combination with pulse IV
methylprednisolone may effectively reduce kidney
scarring compared with corticosteroids alone.

57.  An induction phase consisting of pulse IV
methylprednisolone (0.5-1.0 g/day) for 3 days followed
by oral prednisolone (40-60 mg/day) for the first
month only plus IV cyclophosphamide (0.5-1.0 g /m2)
and continued for 6 months is given during active
disease in patients with biopsy proven WHO classes III
and IV lesions.
 Maintenance therapy is given for additional 18 months
with pulse IV cyclophosphamide every 3 months and
oral prednisone 0.5 mg/kg/day.

59.  Cyclophosphamide is an inhibitor of T-cell
proliferation and the transcription of IL-2.
 It tends to be toxic with many patients having one or
more side effects.
 Nausea and vomiting (60%), alopecia (30%) , dysuria
(30%) , hemorrhagic cystitis (15 %) , herpes zoster
(5%).
 Other ADRs include leukopenia , thrombocytopenia
and amenorrhea(no menses) in premenopausal
women.

60.  Immunosuppressive therapy is helpful reversing
neurologic problems.
 Treatment option include cyclophosphamide and
corticosteroids.
 Anticonvulsants ,antipsychotics , antidepressants ,
antianxiety medications , and antimigraine have all
been used in the management of CNS lupus.

61.  Patients with SLE may be prone to antibody mediated
destruction of peripheral blood cells resulting in
thrombocytopenia , neutropenia , lymphopenia or
hemolytic anemia.
 Treatment of COOMBS- POSITIVE hemolytic anemia with
an acute declining hematocrit and reticulocytosis require
treatment with high – dose steroids.
 When hematocrit rises , steroids can be tapered.
 IV immune globulin and danazol(class of medication
called androgenic hormones used in the treatment of
endometriosis , fibrocytic breast disease , angioedema )
have been used in some cases.

62.  Immunosuppression is only given in APS in the presence of
severe thrombocytopenia.
 Cytotoxic drugs such as vincristine, cyclosporine , danazole
can be given.
 Rituximab can be used in some cases.
 Prophylaxis heparin or low molecular weight (LMW)
heparin therapy is used during pregnancy in women with
antiphospholipid antibodies who have have a history of
thrombotic events or spontaneous abortions.
 Warfarin is Contraindicated in pregnancy due to its
teratogenecity.
 Life-long anticoagulation with warfarin has demonstrated
benefit in decreasing the incidence of recurrent thrombosis
in patients with persistent LA or aCL antibodies.

63.  Patient education and counseling is an important element of non-
pharmacologic management of SLE and minimizing complications
related to therapy.
 Should encourage general lifestyle measures to limit the onset and
severity of disease excerbations.
 Patients must be counseled about the avoidance of overexposure to
sunlight and the use of high-factor sunscreen protection.
 Ideal body weight should be achieved and maintained because obesity
stresses the musculoskeletal system.
 Vigorous activity and exercise should be avoided.
 Dietary and lifestyle modifications should include a good balance of
rest and moderate exercise, smoking cessation , appropriate nutritional
food intake diet , and adherence to a diet low in saturated fat.
 Weight- bearing exercises and oral calcium and Vitamin-D
supplementation are the 1st steps in the prevention of osteoporosis.

64.  The etiology of DIL is unknown.
 Drugs may induce the antigenicity of nucleoproteins leading to the production
of autoantibodies and the production of antinuclear antibodies.
 A number of drugs have been implicated in the induction of lupus like
syndrome known as DIL erythematosus with manifestation ranging from an
isolated positive ANA test result to a clinical lupus syndrome.
 The drugs most commonly implicated are hydralazine and procainamide.
 Other drugs include quinidine , phenytoin , primidone , INH, chlorpromazine
, penicillamine , practolol, propylthiouracil, methylthiouracil , and methyl
dopa.
 The gender ratio for DIL is equal.
 Clinical features include arthritis , arthralgias , myalgias , and fever.
 Pleuritis and pericarditis are prominent.
 A photosensitive skin rash , lymphadenopathy , splenomegaly , anemia ,
leucopenia are very less likely to occur.
 CNS and renal diseases are usually absent.

67.  Symptoms usually resolve in a few weeks when the
offending agent is removed , although the ANA test may
remain positive for several months after withdrawal of the
agent.
 NSAIDs may help to control symptoms of DIL associated
with arthritis , myalgia , fever and pleuritis.
 Corticosteroids and immunosuppressants are rarely used in
DIL.
 30 % cases of DIL in the world are due to procainamide
(antiarrythmic drug).
 21% of cases of DIL in the world are due to hydralazine
(used in hypertension and CHF).

68.  Patient should be informed that SLE is a lifelong disease.
 Current therapy can provide significant improvement , but not complete relief.
 Failure to follow a therapeutic plan can lead to unnecessary morbidity and
mortality.
 Patients need to know that prognosis has improved over the years and survival
in SLE patients is 90 %–95% at 2 years , 80 -90% at 5 years , 70-80 % at 10 years
, and 60-70% at 20 years.
 In lupus nephritis patients , the overall survival rate in the first 10 years is
approximately 50-70%.
 The 10 year survival rate has increased from 50 % in 1980 to 90% in current
time.
 The mortality rate has come down to 8-15 %.
 Deaths are attributed to active disease, infections , thrombosis, and
malignancy.
 Infections and active SLE is the leading cause of death in 1st 5 years , whereas
thrombosis was the leading cause of death in the 2nd 5 years of the disease.

69.  The presence of Lupus nephritis at time of diagnosis is
the strongest predictor of death.
 African- American and Hispanics generally have severe
disease and poor prognosis.
 Some the poor prognostic indicators include high
serum creatinine level , hypertension , nephritic
syndrome , anemia at the time of diagnosis .
 Fatigue is the most common complaint and an often
debilating symptom that may be controlled by energy
conservation.
 Photo protection by protecting clothing and high-
factor sunscreen use and avoidance of excess sun.

70.  Pain , lifestyle changes and emotional problems are
the most difficult to cope with.
 Steroid- sparing protocols , prophylaxis for infections ,
and prevention of osteoporosis are recommended.
 Regular check-ups, and preventative screening should
be done.
 Use of pulse dose of corticosteroids and
immunosuppressive agents have largely improved the
prognosis of SLE.