Systemic Sclerosis / Scleroderma - Musculoskeletal

The hand concentrates many features of the disease. Sclerodactyly - tight, shiny, tethered skin - gradually fixes the fingers in flexion contractures, producing a stiff, claw-like hand with poor function. The vasculopathy gives near-universal Raynaud's with digital ulcers and pitting scars at the fingertips, and in severe cases digital ischaemia/gangrene. Calcinosis deposits chalky subcutaneous calcium (often in the fingers in limited disease), which can ulcerate and become infected, and acro-osteolysis resorbs the distal phalangeal tufts. There may be arthralgia/arthritis, joint erosions, distal ulna resorption, and a high prevalence of erosive osteoarthritis; tendon friction rubs signal diffuse disease and a worse prognosis. Hand radiographs are abnormal in roughly two-thirds of patients, increasingly so with longer disease duration.

Diagnosis is clinical (ACR/EULAR criteria) supported by autoantibodies and nailfold capillaroscopy (a scleroderma pattern - giant capillaries and dropout). Key associations:

• Limited cutaneous (CREST): anti-centromere; calcinosis; pulmonary arterial hypertension risk.
• Diffuse cutaneous: anti-Scl70 (topoisomerase) or anti-RNA polymerase III; interstitial lung disease and scleroderma renal crisis.
• On hand radiographs, acro-osteolysis associates with anti-Scl70/diffuse disease and ILD; calcinosis with anti-centromere and PAH/GI involvement; flexion contracture with higher skin score and GI disease. Patients need systemic surveillance (lung function/HRCT, echocardiography for PAH, renal/BP monitoring), which is a rheumatology-led responsibility but important to know.

• Medical (rheumatology-led): disease-modifying immunosuppression for diffuse/active disease; vasodilator therapy for Raynaud's/digital ulcers (calcium-channel blockers, PDE5 inhibitors, IV iloprost, bosentan to prevent new ulcers); organ-directed treatment (e.g. ACE inhibitors for renal crisis).
• Hand therapy: stretching, splinting, paraffin baths and occupational therapy to maintain mobility and manage contractures; skin care and meticulous wound care for ulcers.
• Surgery (selective, cautious): excision of symptomatic/ulcerating calcinosis, debridement or amputation of infected/gangrenous digits, digital sympathectomy for refractory ischaemia, and occasionally contracture correction or joint fusion - all undertaken with awareness of poor wound healing, vasculopathy and infection risk; optimise perfusion, avoid tourniquets where possible, and counsel about delayed healing and recurrence (calcinosis recurs).