Table of Contents
Systemic sclerosis is a systemic autoimmune condition resulting in excessive collagen deposition by fibroblasts.
Types
- Limited cutaneous systemic sclerosis: cutaneous sclerosis limited to below elbows
- Diffuse cutaneous systemic sclerosis: cutaneous sclerosis extends above elbows – more likely to get organ involvement
Clinical features
Symptoms
- Skin
- Thick, tight, itchy skin
- Usually affects the face, hands and feet but can progress to affect the whole body
- CREST features
- Calcinosis
- Raynaud’s
- Esophageal dysfunction (heartburn)
- Sclerodactyly (thickening and tightness of skin of fingers/toes)
- Telangiectasia
Signs
- Hands
- Look: sclerodactyly, tight waxy skin, ulceration, blanching, finger-pulp atrophy
- Feel: subcutaneous calcinosis, cool hands
- Move: prayer sign lost (fixed flexion deformity of fingers)
- Face
- Mask-like face
- Skin tethering
- Telangiectasia
- Microstomia
- Beaked nose
Fibrotic organ involvement
Lungs | Fibrosis, pulmonary hypertension |
Heart | Myocardial fibrosis → arrhythmias |
Kidneys | Acute or chronic kidney injury, hypertension, acute renal hypertensive crises |
Gut | Reflux, dysphagia, faecal incontinence |
Investigations
- Auto-antibodies
- Antinuclear (commonly positive but non-specific)
- Limited: anti-Centromere (CREST)
- Diffuse: anti-topoisomerase (anti-Scl-70)
- Assess extent of organ involvement (e.g. CXR/high-resolution CT, echocardiogram, ECG, U&Es, urinalysis, barium swallow)
- Hand X-rays (calcinosis)
Management
- No cure
- Symptomatic treatment
- Raynaud’s: hand warmers, Ca2+ antagonists, IV prostacyclins, phosphodiesterase-5 inhibitors
- GI: proton pump inhibitor
- Prevention of complications
- Lung fibrosis: immunosuppressants (e.g. cyclophosphamide, mycophenolate)
- Pulmonary hypertension: prostaglandins, endothelin antagonists, phosphodiesterase-5 inhibitors, supplemental oxygen
- Renal crises: low dose ACE inhibitor
Test yourself
What is colour sequence of Raynaud’s phenomenon?
Hands change colour in cold: White→Blue→Red
Remember as White Before Red!
What is the pathophysiology in Scleroderma renal crisis?
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