Systemic sclerosis (Scleroderma)

Systemic sclerosis is a systemic autoimmune condition resulting in excessive collagen deposition by fibroblasts.


  • 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


  • 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 


  • 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


  • 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)


  • Immunosuppression options: methotrexate, mycophenolate mofetil, cyclophosphamide
  • Autologous stem cell transplantation in select cases
  • Prevention/management of organ-based disease
    • Raynaud’s: hand warmers, Ca2+ antagonists (nifedipine), IV prostacyclins (iloprost), phosphodiesterase-5 inhibitors
    • Skin disease: immunosuppression
    • GI: proton pump inhibitor, upright posture after meals, laxitives
    • Lung fibrosis: immunosuppression, oxygen therapy
    • Pulmonary hypertension: prostaglandins, endothelin antagonists, phosphodiesterase-5 inhibitors, oxygen therapy
    • Renal crises: low dose ACE inhibitor
    • Heart failure: immunosuppression, pacemaker, implantable cardioverter defibrillator, ACE inhibitor, carvedilol, diuretics

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.

Your email address will not be published. Required fields are marked *

Join our email list!
For free OSCE tips and updates