Scleroderma

Scleroderma
Dr. DOHA RASHEEDY ALY
Assistant Professor
Department of Geriatric and Gerontology
Ain Shams University
10/23/2017 Dr Doha Rasheedy

Etiology
• The cause is unknown, but it probably involves an
environmental trigger in a genetically predisposed person.
• Genome-wide scanning has identified susceptibility loci on
three different non-HLA chromosomes: fibrillin on
chromosome 15, SPARC (secreted protein acidic and rich in
cysteine) on chromosome 5 and topoisomerase 1 on
chromosome 20.
• Environmental factors are important in isolated cases that
result from exposure to
– silica dust,
– vinyl chloride,
– bleomycin
– hypoxy resins
– trichloroethylene.

Systemic sclerosis (SSc) is the result of an
interplay among four pathogenetic processes:
– Immune activation (autoimmunity)
– Inflammation
– Vascular injury and obliteration
– Fibroblast activation with synthesis of increased
extracellular matrix components.
Pathophysiology

• autoimmunity with vascular reactivity precedes development of
SSc, as patients usually have autoantibodies and RP prior to the
development of other organ involvement.
• Autoantibodies against components of the extracellular matrix
found in 50% of SSc patients can activate fibroblasts, induce
collagen production, and prevent collagen degradation, resulting
in tissue fibrosis.
• Endothelial injury appears to occur early in the course of SSc, but
the trigger for the damage has not been identified. Injury results
in endothelial cells becoming activated, producing adhesion
molecules and inflammatory cytokines, which can lead to
endothelial damage, resulting in tissue hypoxemia.
• Hypoxemia stimulates production of angiogenesis factors like
vascular endothelial growth factor (VEGF), tumor (or
transforming) growth factor (TGF) β and endothelin-1 (ET-1),
which results in vasoconstriction without angiogenesis in SSc
patients.

1. Early in the disease there is
– Skin infiltration by T lymphocytes
– Abnormal fibroblast activation
2. That leads to increased production of
extracellular matrix in the dermis, primarily type
I collagen.
3. This results in symmetrical thickening, tightening
and induration of the skin
4. In addition to skin changes there is arterial and
arteriolar narrowing due to intimal proliferation
and vessel wall inflammation.
5. Endothelial injury causes release of
vasoconstrictors and platelet activation,
resulting in further ischaemia.

Epidemiology
• Prevalence rate in US is estimated at
28/100,000.
• Women: men= 5:1
• The most common age of onset is between
30 and 50 years.
• Having a first degree relative with disease
confers a relative risk of about 13 but rates
between monozygotic and dizygotic twins
are similar

scleroderma
Localized
Only skin
affection
morphea
Linear
scleroderma
scleroderma
en coup de
sabre
Systemic
(skin+ organ)
Localizes
SSC
Diffuse
SSC
SS sine
scleroderma (n
skin affection)

Scleroderma is divided into two main forms:
localized and systemic.
• Localized scleroderma only involves the skin with, at
most, some atrophy of the subcutaneous tissue and
may affect bone underlying the lesions, no internal
organ affection.
• It includes:
– Morphea (patches of thickened skin)
– Linear scleroderma (a line of thickened skin on one or more
extremities)
– scleroderma en coup de sabre (linear disease affecting only
one side of the face and scalp).

Morphea

Morphea
• Hardening and thickening of the
skin.
• Discoloration of the skin of affected
area, skin looks lighter or darker than
the surrounding skin area.
• There occur patches which are oval
shaped and change their colors
slowly develop a white centre.
• There is loss of hair and sweat glands
in the affected area.

Linear scleroderma
a line of thickened skin on one or more extremities)

Scleroderma en coup de sabre
• linear disease affecting
only one side of the
face and scalp

Forms of Systemic Sclerosis
• Limited Scleroderma
• Skin thickening is distal to elbows and knees, not involving trunk
• Can involve perioral skin thickening (pursing of lips)
• Less organ involvement
• Seen in CREST syndrome (calcinosis, Raynaud’s phenomenon
[RP], esophageal dysmotility, sclerodactyly, and telangiectasias
• Isolated pulmonary hypertension , isolated biliary cirrhosis can
occur
• Diffuse Scleroderma
• Skin thickening proximal to elbows and knees, involving the trunk
• More likely to have organ involvement
• Pulmonary fibrosis and Renal Crisis are more common.
• Systemic sclerosis sine scleroderma:
– characterized by visceral and immunological manifestations of
SSc in the absence of clinically detectable skin involvement.

• Diffuse systemic sclerosis (dcSSc) involves skin of
extremities, face and trunk while limited systemic
sclerosis (lcSSc) involves only distal extremities
and face with no involvement of trunk.
• Face can be involved in both forms.
• In diffuse variety systemic complications like
interstitial lung disease and renal crisis are more
common whereas in limited variety pulmonary
arterial hypertension is more common.
• The initial complaint of limited cutaneous
scleroderma is Raynaud’s phenomenon, whereas
patients with diffuse cutaneous scleroderma
often present with generalized hand swelling,
skin thickening or arthralgias with or without
Raynaud’s phenomenon. They may initially
complain of tight, puffy fingers that occurs in the
morning but then lasts all day10/23/2017 Dr Doha Rasheedy

Systemic sclerosis sine scleroderma
Its diagnosis should be considered if he or she has all of
following:
1) Raynaud's phenomenon,
2) Positive ANA
3) Any one of following- distal oesophageal hypomotility,
small bowel hypomotility, pulmonary interstitial fibrosis,
pulmonary artery hypertension, cardiac involvement
typical of scleroderma or scleroderma renal crisis and
4) no other defined connective tissue or other disease as a
cause of 1), 2), or 3
• Most frequently associated serum autoantibody
associated with it is anticentromere antibody

CREST Syndrome
ACR and Mayo Foundation

Organs Involved
• Skin
• Gastrointestinal
• Pulmonary
• Musculoskeletal
• Renal
• Cardiac

Skin Manifestations
• Raynaud's phenomenon
• Skin thickening
• Telangectasia
• Calcinosis
• Dyspigmentation
• Ulceration

Raynaud's phenomenon
• RP is almost universally present in SSc.
• It is usually the first manifestation of SSc and may
precede the development of other features by months
to years.
• Patients report episodic, bilateral color changes
precipitated by the cold or by emotional stress.
• Digits, nose, and ears turn white when vasospasm
occurs as a result of sympathetic hyperactivation. As
oxygen supply is depleted, they turn blue and, upon
rewarming, they become red as reperfusion occurs.
• In contrast to primary Raynaud syndrome, which
typically presents during adolescence and does not lead
to ischemic complications, secondary Raynaud
phenomenon occurs later and often is complicated by
ischemic tissue damage.

Raynoud’s phenomenon

Other Skin changes
• Often the first complaint specific for scleroderma is swelling or
“puffiness” of the fingers or hands corresponding to the edematous
phase.
• Patients may also complain of pruritus, dry skin, tightening and
decreased flexibility, and skin ulcerations.
• Affected skin is thickened and leathery or hide-bound(attached to
underlying tissue)
• Sclerodactyly refers to such skin changes affecting the fingers.
Fingertips can appear tapered from ischemic loss of the digital pulp.
• A tight “purse-lip” appearance associated with decreased oral
aperture is attributed to increased fibrotic activity within the perioral
skin.
• loss of forehead wrinkles
• Telangiectasias may be appreciated over the face, oropharyngeal
mucosa and hands.
• Hyper- and hypopigmentary changes may lead to a “salt-and-pepper”
appearance of the skin.
• Subcutaneous calcinosis can also be seen.

Skin changes
occur in three phases: (edematous, induration, atrophic)
• the oedematous stage, with oedema usually non-
pitting and painless.
• This usually progresses to a thickened, tight indurative
stage over months to years, with the skin becoming
shiny and adherent to the underlying subcutis. Dermal
thickening and epidermal thinning lead to loss of
dermal appendages, with hair loss and absent
sweating.
• there is an atrophic stage in which the dermis softens
and thins but remains firmly bound down to the
subcutaneous fat.

Face affection
1. Elongated face
2. Tight shiny skin
3. Loss of skin wrinkes, nasolabial folds
4. Loss of facial expression
5. pinched nose
6. Purse lip, deepening of perioral facial
folds (radial furrowing)
7. Lip thinning and retraction
8. Small oral aperture: (microstomia).
9. telangiectasia10/23/2017 Dr Doha Rasheedy

Indurated phase
• firm, thickened bound
to underlying soft tissue
• Dyspigmented: salt and
pepper appearance,
May become hyper- or
hypo-pigmented
• Creases disappear
• Hair loss
• Decreased sweating

Dyspigmentation
• Hyper- and hypopigmentary
changes may lead to a “salt-and-
pepper” appearance of the skin.
• Pigment loss spares perifollicluar
areas – salt and pepper appearance
of skin –scalp ,upperback ,chest

Telangectasia

Sclerodactly
• Sclerodactyly means thickening of the skin of the digits of
the hands and feet.
• Three phases of skin changes are seen in scleroderma: the
edematous phase, indurative phase, and atrophic phase.
• Patients with early scleroderma present with puffy edema in
the fingers and may report morning stiffness or arthralgias.
The edematous phase is usually short (ie, months, but
occasionally years).
• In the indurative phase, the skin becomes thickened.
Patients may report pruritus. The skin appears shiny and
tight. Skin creases are lost. Erythema may be present. In
limited scleroderma, this process continues slowly for many
years.

Puffy hands of early scleroderma

Acrosclerosis (flexion contractures
secondary to skin tightening)

• Tight shiny skin
• Digital ulcer
• Limited movement
• Loss of transverse
creases on dorsum of
fingers
• Acro-osteolysis
terminal phalanx
resorption

Digital pitting scars

Calcinosis
• calcific deposits are found predominantly in the extremities, around joints, and
around bony prominences. Osteonecrosis has also been reported. Deposits
typically are found in the flexor surfaces of the hands and the extensor
surfaces of the forearms and knees.
• calcinosis at areas of pressure such as the finger pads, olecranon bursa,
extensor surface of forearm, buttocks and around the patella.

Calcinosis on x-ray
Gupta E., et al. Malaysian Family
Physician. 2008;3(3):xx-xx ACR

Digital ulcers
• Skin ulceration on pressure
points
• Localised areas of infarction
and Pulp atrophy at the
fingertips.
• Skin ulcers are of two forms:
digital tip ulcers secondary to
ischaemia, and ulceration
over bony prominences
where the skin is contracted
and tight and susceptible to
trauma

Late:Flexion contractures of arms and Painful flexed claw
like hands
• Severe scleroderma with deformity of hands as a result of sclerodactyly leading
to severe flexion contractures
• DD:
1. Generalised nodal OA
2. RH A10/23/2017 Dr Doha Rasheedy

Nailfold capillary abnormalities
• Three-quarters of scleroderma
patients have abnormalities of
their nailfold capillaries,
Capillaroscopy
with dilated capillary loops but without
dropout.

Gastrointestinal(GI) complaints
• are common, with involvement anywhere along the alimentary
tract.
Gastroesophageal reflux is the most common complaint.
Patients may also note:
• Dry mouth
• Dysphagia
• Dyspepsia
• Nausea
• Early satiety
• cramping abdominal pain
• diarrhea
• weight loss
• Intestinal pseudoobstruction may occur secondary to
hypomotility.

• The gastrointestinal (GI) tract is the second most
commonly affected organ, with involvement in 75%–
90% of cases.
• The hallmark effect is smooth muscle fibrosis, leading
to dysmotility,
• with 80% of patients developing oesophageal
dysfunction. Impaired gastric emptying and decreased
lower oesophageal sphincter tone allow reflux with
heartburn and dysphagia. In the longer term, Barrett’s
oesophagus and strictures may develop.
• Dysmotility of the intestinal tract may result in
pseudo-obstruction and small-bowel bacterial
overgrowth with vitamin B12 and folate deficiency,
and malabsorption. Symptoms include bloating and
abdominal pain, diarrhea/constipation, steatorrhea,
and wasting.

• Anemia may be a sign of vitamin deficiency resulting from
bacterial overgrowth or chronic blood loss resulting from
gastric antral vascular ectasia syndrome, or “watermelon
stomach.” characteristic endoscopic appearance of
longitudinal rows of sacculated and ectatic mucosal
vessels in the antrum of the stomach; they resemble the
stripes on a watermelon. Endoscopic laser coagulation and
obliteration of vascular ectasia decrease the risk of
rebleeding
• Chronic constipation is common and may result in
intestinal impaction.
• A diagnosis of intestinal pseudo-obstruction often is made
at the time of laparotomy, although nonsurgical
treatments—including bowel rest, antibiotics, and
judicious use of promotility agents—often are effective.
Smooth muscle dysfunction in the anus causes fecal
incontinence and is a common problem.10/23/2017 Dr Doha Rasheedy

• Film from an
esophagram shows a
dilated esophagus with
a persistent air-fluid
level, indicating delayed
emptying

• Recurrent occult upper GI
bleeding may indicate a
'watermelon stomach'
(antral vascular ectasia),
which occurs in up to 20%
of patients
• Hidebound sign (crowding
of valvulae conniventes).
• = stack of coin sign
• = accordion sign

pulmonary involvement
• Pulmonary involvement occurs in more than 70% of cases and is the most
common cause of SSc-related death.
• patients may complain of
• dyspnea on exertion
• nonproductive cough.
1. interstitial lung disease
2. Pulmonary hypertension
3. NO pleural effusion (DD with Rh & SLE).
• Patients with both limited and diffuse forms of SSc are at risk for PAH, The
only way to directly measure pulmonary artery pressures and evaluate
response to treatment is right heart catheterization.
• The five-year cumulative survival is 10% in those with PAH compared with
80% in those without.
• PAH can develop early , particularly in limited cutaneous SSc, and can
occur without pulmonary fibrosis. In diffuse cutaneous SSc,development
of pulmonary fibrosis may lead to secondary PAH.

• Less frequently –aspiration pneumonitis ,
pulmonary hemorrhage , obliterative
bronchiolitis , pleural involvement , restrictive
ventilatory defect due to chest wall fibrosis ,
spontaneous pneumothorax.

• The diagnosis of ILD often is made with screening
high-resolution CT (HRCT) scans of the chest that
demonstrate reticular opacification of the lung base
or ground-glass opacification. More advanced
• ILD is associated with radiographic findings of
honeycombing, traction bronchiectasis, and
bilateral subpleural fibrosis most prominent in the
lower lung zones.
• A restrictive pattern (reduced forced expiratory
volume in 1 second, forced vital capacity [FVC], and
total lung capacity) is most characteristic on
screening pulmonary function tests

• Ground glass opacification.
• Reticular opacities &
interlobular septal
thickening.

Cardiac affection
1. Arrhythmias from conduction system fibrosis,
2. pericardial rubs from pericarditis,
3. signs of congestive heart failure from
myocardial fibrosis can be seen with cardiac
involvement.
Chest pain may occur due to esophagitis,
pleurisy, pericarditis, costochondritis, coronary
vasospasms, and fibrosis of the chest wall.

Musculoskeletal complaints
• Nonspecific.
• Patients often experience generalized arthralgias with
stiffness of the joints under the fibrotic skin.
• Tendons
– Friction rubs
– Contractures
• Bone changes:
Acro-osteolysis (resorption of terminal phalanges).
Joint space narrowing.
Erosions.
• CARPAL TUNNEL SYNDROME –may be a presenting
feature.

Kidney involvement
• Signs of kidney involvement, proteinurea and mild
hypertension, are common. The degree of severity
depends on whether it is acute or chronic.
• Slow Progression. The typical course of scleroderma in
the kidney is a slow progression that may produce
some damage but does not usually require dialysis.
• Renal Crisis: The most serious. It occurs in about 20%
of patients with diffuse scleroderma, usually early in
the course of the disease. This syndrome includes a life
threatening condition called malignant hypertension, a
sudden increase in blood pressure that can cause
rapidly progressive kidney failure.

Scleroderma renal crisis
• is a major complication in patients with systemic sclerosis (SSc).
• SRC is clinically characterized by:
1. new onset, often symptomatic hypertension with blood
pressure >140/90 mmHg or a >30 mmHg rise in blood pressure
from baseline
2. rising serum creatinine levels and/or oligoanuria.
3. Microangiopathic haemolytic anaemia (MAHA) occurs in up to
50% of patients and is characterized by proteinuria, haematuria,
and circulating fragmented red blood cells
4. Left ventricular insufficiency and hypertensive encephalopathy
are typical clinical features.
5. Anti-RNA-polymerase III antibodies are present in one third of
patients who develop SRC.
Roughly 10% of patients who have SSc with SRC have normotensive
SRC, with blood pressures that are elevated from baseline but in the
normotensive range

• The pathogenesis of SRC is attributed to endothelial cell
injury that leads to intimal thickening in small renal
arteries as well as platelet aggregation. Luminal narrowing
decreases renal perfusion, which further activates the
renin-angiotensin system. Malignant hypertension and
more renal damage ensue.
• D.D – TTP commonly misdiagnosed
• Renal biopsy is not necessary if SRC presents with classical
features. However, it can help to define prognosis and
guide treatment in atypical forms.
• SRC is more likely to occur in patients with dcSSc than in
those with lcSSc, especially in patients with rapidly
progressive dcSSc within the first 3–5 years of SSc onset

• predictive factors for SRC include:
1. the presence of anti-RNA polymerase III
antibodies
2. tendon friction rubs
3. Synovitis
4. Rapid progression of skin manifestation
5. Glucocorticoid treatment (>7.5 mg daily)
exerts a dose-dependent effect on the risk of
SRC development

• Prompt recognition of SRC and initiation of ACEi therapy offer the best
outcome
• ACEi must be continued even if there is a deterioration in renal function.
The goal of the treatment is to obtain control of blood pressure as soon as
possible. The use of prophylactic ACEi remains a matter of debate since
some SSc patients developed SRC while taking these agents
• Angiotensin receptor blockers offer a theoretical benefit, but—in contrast
to ACEi—clinical experience with these agents has been variable
• Add (low doses of prostacyclin or endothelin receptor blockers)
• Additional antihypertensive therapy is mandatory with combinations of
calcium blockers, nitrates, or other vasodilators agent
• Since relative hypovolemia is frequent is SRC, there are concerns about the
use of diuretics or labetalol.
• Plasma exchanges or immunosuppressive drugs exert no proven beneficial
effect in the treatment of SRC.
• Corticosteroids are contra-indicated in SRC.
• Requirement for dialysis is needed in up to half of the patients. It may be
temporarily, with 50% of the patients being weaned from dialysis within
2 years after the onset of SRC
• The final decision of transplantation should not be made before 2 years
after the onset of SRC.

The American College of Rheumatology classification criteria
for SSc
Diagnosis: 1 major or 2 minor criteria
MAJOR criterion :
PROXIMAL scleroderma –characterized by SYMMETRICAL
thickening,tightening and induration of the skin of the fingers and the
skin proximal to the MCP /MTP joints. These changes may affect the
entire extremity,face,neck,trunk.
MINOR
1.SCLERODACTYLY – thickening,induration,tightening of the skin
limited only to fingers.
2.DIGITAL PITTING SCARS/LOSS OF SUBSTANCE FROM THE FINGER
PAD –due to ischemia.
3.BIBASILAR PULMONARY FIBROSIS- b/l reticular pattern of linear or
lineonodular densities in basilar portions of the lung on CXR.diffuse
mottling/honey comb lung not attributable to primary pulmonary
disease

Differential Diagnosis
1- Skin thickening
• do not have RP or digital ulcerations
1. Nephrogenic systemic fibrosis (NSF)
2. Scleredema, Scleromyxedema, POEMS
syndrome
3. Eosinophilic fasciitis
4. Other conditions

• Nephrogenic systemic fibrosis (NSF), affecting
mostly dialysis patients, tends to affect lower
extremities more than upper extremities and
generally spares the hands.
• Eosinophilic fasciitis can have a rapid onset of skin
thickening with early development of flexion
contractures due to fascial thickening. Skin tends
to be more puckered appearing, but a deep biopsy
will demonstrate eosinophilic infiltration,
separating it from SSc. Eosinophilia is common,
and eosinophilic infiltrates are usually seen on
biopsy.

Three syndromes with paraproteinemia have
scleroderma-like skin changes:
• Scleredema, complication of long-standing
diabetes or paraproteinemia, causes thickening of
the skin in the neck, shoulder girdle, proximal
upper extremities, and back, and is characterized
by mucin deposition on skin biopsy.
• Scleromyxedema, which can involve the hands,
but involved skin tends to be more folded and
pendulous rather than tight and thickened.
• POEMS syndrome (polyneuropathy, organomegaly,
endocrinopathy, monoclonal gammopathy, and
scleroderma-like skin changes).

• Other conditions with scleroderma-like skin
thickening include:
1. diabetic digital sclerosis
2. chronic graft-versus-host disease,
3. vinyl chloride exposure, bleomycin toxicity,
4. vibratory injury,
5. complex regional pain syndrome/
6. reflex sympathetic dystrophy,
7. amyloidosis,
8. porphyria cutanea tarda,
9. and carcinoid syndrome.

Other DD
• Diseases with similar organ involvement
include primary pulmonary hypertension, ILD,
primary biliary cirrhosis, intestinal
hypomotility, and collagenous colitis.
• Diseases that may present in a similar fashion
to scleroderma include systemic lupus
erythematosus (SLE), mixed connective tissue
disease (MCTD), rheumatoid arthritis (RA),
and inflammatory myopathies.

Skin Thickening in Systemic
Sclerosis
Assessed by Modified Rodnan Skin Score
– Validated measure of skin thickening used in clinical trials
– Correlates with internal organ involvement and survival in
diffuse patients
Thickening assessed in 17 body areas: face/neck, anterior
chest, abdomen and bilateral fingers, dorsal hands, forearms,
upper arms, feet, lower legs, and thighs
– Each area scored and summed for total score (0-51):
0=normal
1=mild thickening
2=moderate thickening, unable to move
3=hidebound, unable to pinch

Laboratories
• 95% of patients with scleroderma will have a positive ANA,
with the anticentromere staining pattern of the ANA being
specific (but not sensitive) for limited scleroderma.
• Anti–Scl-70 (anti–topoisomerase-I) antibodies are specific
(but not sensitive) for diffuse scleroderma, and are
associated with risk for ILD.
• Less commonly assayed antibodies associated with
scleroderma include anti- RNA polymerase I, II, and III and
U3-ribonucleoprotein (RNP). RNP is associated with risk for
pulmonary hypertension.
• anti- RNA polymerase III associated with increased risk of
renal crisis
• Anti–U1-RNP antibodies are found in MCTD, and anti–PM-
Scl antibodies may be present in overlap syndromes.

Imaging
• Transthoracic echocardiography can be used
to evaluate for pulmonary hypertension or
pericardial effusion.
• Patients with ILD will have interstitial
infiltrates on high-resolution CT and a
restrictive pattern on pulmonary function
testing.

Diagnostic Procedures
• Reflux, dysphagia, and odynophagia are frequent
complaints related to esophageal dysmotility,
which can be evaluated with barium esophagram
or esophageal manometry.
• Patients with elevated pulmonary pressures on
echocardiography or those with dyspnea
unexplained by other causes should undergo a
right heart catheterization, as echocardiography
can under- or over-estimate pulmonary pressures
in about 10% of patients

Management
• There is no treatment for the underlying
disease process and, hence, treatment is
targeted at specific organ complications
and/or patient symptoms.

Skin involvement
• No effective antifibrotic therapy has been
discovered to date. D-Pencillamine,
methotrexate, Mycophenolate mofetil ,
Minocycline can be used
• Autologous hematopoietic stem cell
transplant for severe dSSc has shown promise
in reversing cutaneous disease, improving
quality of life, and maintaining internal organ
function. Randomized trials are in progress

RP
1. Nonpharmacologic measures for treating RP include
smoking cessation and avoiding cold exposure.
2. Diltiazem and dihydropyridine calcium-channel
blockers (CCBs) like amlodipine, nifedipine, and
felodipine have been shown to be effective in RP.
3. Other medications that have been shown to have
some efficacy in RP include the angiotensin II
inhibitors, α-antagonists, selective serotonin
reuptake inhibitors, nitrates, phosphodiesterase
inhibitors such as sildenafil, and, in severe disease,
prostacyclins. All of these agents are more effective
in primary RP than in RP associated with
scleroderma.

Digital ulcers:
• Ulcers are extremely painful and, because they result
from ischemia, are difficult to heal.
1. Analgesics and local wound care can be helpful.
2. Case series have demonstrated the benefit of
sildenafil and tadalafil in facilitating healing and
preventing new ulcer formation.
3. Bosentan has been shown to reduce the
development of new ulcers but without any effect
on existing ulcers.
4. Iloprost has been shown to help heal ulcers and
prevent new ones
5. Sympathectomies, sympathetic blocks, and intra-
arterial injections of vasodilators have been
reported to help but responses have been
inconsistent.10/23/2017 Dr Doha Rasheedy

ILD
• Cyclophosphamide has been used for SSc ILD
for many years but randomized, controlled
trials have demonstrated only modest benefit
in forced vital capacity, fibrosis, and dyspnea
• A small randomized controlled study
demonstrated improvement in skin and ILD
with rituximab therapy

GI
• Reflux symptoms can be controlled with proton pump inhibitors, but it
may be necessary to use up to three times the usual dose. Because
esophageal disease may be asymptomatic, acid suppression therapy
should be started in all patients; in theory, such therapy may prevent
stricture formation, aspiration pneumonitis, and Barrett esophagus.
However, the precise role of acid suppression therapy in halting the
progression of esophageal or pulmonary disease has not been
established
• Esophageal strictures are treated with dilatation when necessary.
• Gastric antral venous ectasia is the most common cause of GI bleeding
in scleroderma and can be treated with endoscopic laser
photocoagulation.
• Prokinetic agents like metoclopramide can be for aperistaltic symptoms.
• Intestinal pseudo-obstruction can be managed with octreotide.
• Patients who develop symptoms of small bowel bacterial overgrowth
can be managed with alternating doses of ciprofloxacin and10/23/2017 Dr Doha Rasheedy

• Corticosteroids have been shown to be associated with SRC.
Therefore, if corticosteroids are to be used to manage an
inflammatory arthritis or SSc/myositis overlap syndrome,
they should be given at the lowest possible dose, and
patients should be instructed to monitor their blood
pressure.
Other Non-Pharmacologic Therapies
• Avoiding excess bathing and using proper moisturizing
creams can aid in skin care.
• Aggressive occupational and physical therapy may be
helpful early in the course of disease to minimize
contractures
• Patients with scleroderma are at increased risk for
depression and despair. Support groups may be beneficial.

Surgical Management
• Post-operative healing can be difficult in SSc
patients. However, digital ulcers can become
infected and may need debridement.
Amputations may be necessary for deeper
infections

Scleroderma

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