Sign up here!
image

Systemic sclerosis (Scleroderma)

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

LungsFibrosis, pulmonary hypertension
HeartMyocardial fibrosis → arrhythmias
KidneysAcute or chronic kidney injury, hypertension, acute renal hypertensive crises
GutReflux, 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?

Oops! This section is restricted to members.

Create an account or log in to continue reading.

image
Join our email list!
For free OSCE tips and updates