Systemic
sclerosis
Presented By,
Anjumi Naoshin
Roll -59, TA07
Systemic sclerosis
An autoimmune disorder of connective tissue results in
fibrosis affecting skin, internal organs and vasculature.
Peak age of onset is in fourth
& fifth decades
Prevalence is 10 to 20 per
10000
4:1 female to male ratio
Diagnostic criteria
According to the American College of Rheumatology
(ACR):
Systemic sclerosis is diagnosed when a patient has 1 major
and 2 minor criteria:
Major features include centrally located skin sclerosis
that affects the arms, face, and/or neck.
Minor features include sclerodactyly, erosions, atrophy
of the fingertips, and bilateral lung fibrosis.
Types of systemic sclerosis
(1) Diffuse (2)Limited
systemic sclerosis systemic sclerosis
Poor Good
prognosis prognosis
5 year survival CREST
of approx. Syndrome
70%
Clinical Features
Skin involvement
Non pitting edema of fingers
Shiny and taut skin, sclerodactyly,
flexion contractures of fingers
Thinning of lip and radial
furrowing
Raynaud’s phenomenon
Universal features
Involvement of small
vessels of extremities
leading to ischaemia to
infarction & necrosis
Musculoskeletal involvement
Arthralgia
Flexor tenosynovitis
Muscle weakness
Wasting
Gastrointestinal involvement
Reflux with erosive oesophagitis
Dysphagia
Odynophagia
Recurrent occult upper GI bleeding
Small intestinal bacterial overgrowth
Constipation
Pulmonary and Renal involvement
Pulmonary hypertension
Interstitial lung disease
Hypertensive renal crisis
Investigations
Complete blood count with ESR
Renal function test
Liver function test
Urinalysis
ANA (positive in 95%)
Antibodies to topoisomerase 1 ( Scl70) ( positive in 30 %
in dcSScl)
Investigations
Anticetromere antibodies ( positive in60% in limited
SScl)
Chest X- Ray
Echocardiography
Lung function test
High resolution CT scan
Right heart catheterization
Barium swallow
Management
Goal of treatment
To ameliorate symptoms
To combat the effects of the disease on target organs
Severe diffuse systemic sclerosis
Autologous stem cell transplantation following
myeloablative therapy following cyclophosphamide
Raynaud’s phenomenon
Avoidance of cold exposure
Use of thermal insulating gloves
Pharmacotherapy- calcium channel blockers, losartan,
fluoxetine, sildenafil, tadalafil, intravenous prostacyclin,
bosentan
Regular use of Fucidin-hydrocortisone cream ( over
digital ulcer)
Management of organ involvement
Oesophageal reflux – proton pump inhibitors, anti-
reflux agents
Small intestinal bacterial overgrowth- Rotating courses
of antibiotics
Pseudo-obstruction- Metoclopramide or domperidone
Hypertension- ACE inhibitor
Management of organ involvement
Joint involvement- Analgesic or NSAID
Progressive pulmonary hypertension- Bosentan, Heart-
lung transplantation
Interstitial lung disease-Cyclophosphamide, MMF
Tyrosine kinase inhibitor- Nintedanib
