Scleroderma: Understanding Causes, Symptoms & Treatment

Scleroderma (systemic sclerosis) is a chronic autoimmune disease characterized by fibrosis (collagen overproduction), vascular dysfunction, and autoimmunity affecting skin and internal organs.

Subtypes

• Limited cutaneous (lcSSc): Skin thickening distal to elbows/knees + face; CREST syndrome (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia). Slower progression.
• Diffuse cutaneous (dcSSc): Proximal skin involvement (trunk, upper arms/thighs); rapid progression; high risk of interstitial lung disease (ILD), renal crisis, cardiac, GI involvement.

Key Manifestations

• Raynaud's phenomenon (often first sign)
• Skin thickening, tightening, contractures
• ILD (dyspnea, cough) – leading cause of death
• Pulmonary arterial hypertension
• Renal crisis (acute hypertension, renal failure)
• GI dysmotility (reflux, bloating, constipation)

Management

• Immunosuppressants (mycophenolate, cyclophosphamide, tocilizumab, nintedanib for ILD)
• Vasodilators for Raynaud's/PAH
• ACE inhibitors for renal crisis prophylaxis/treatment
• Physical/occupational therapy, skin care, reflux management

Multidisciplinary care (rheumatology, pulmonology, cardiology, nephrology, dermatology) is essential.