Systemic Sclerosis (SSc), commonly known as Scleroderma, is a rare, chronic, multisystem autoimmune connective tissue disease characterised by three defining pathological processes: immune dysregulation and autoantibody production, widespread vasculopathy (especially Raynaud's phenomenon), and progressive fibrosis of the skin and visceral organs. The hallmark is thickening and hardening of the skin due to excessive collagen deposition by activated fibroblasts. SSc is classified into two major subtypes — diffuse and limited — based on the extent of skin involvement, which guides prognosis and risk of internal organ complications. It carries the highest mortality of any systemic autoimmune rheumatic disease.
Clinical Presentation
Currently Licensed States:
Pericarditis, accelerated atherosclerosis, increased MI risk
Pleuritis, pneumonitis, pulmonary hypertension
Episcleritis, scleritis, keratoconjunctivitis sicca
Anaemia of chronic disease, Felty syndrome
Laboratory & Imaging
HLA-DR2/DR3, complement deficiencies (C1q, C4), IRF5, STAT4 gene polymorphisms increase risk.
UV light, infections (EBV), hormonal changes, and drugs (procainamide, hydralazine) can precipitate disease.
B cells produce ANA, anti-dsDNA, and other autoantibodies. T helper cells and type I interferons amplify the response.
Autoantibody-antigen complexes deposit in tissues — kidneys, skin, vessels — activating complement and causing inflammation.
Persistent inflammation leads to fibrosis and functional impairment of target organs if disease remains uncontrolled.
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Rheumatology & Autoimmune Specialist
Providing expert-level autoimmune care with over 30 years of academic medicine experience. Serving Colorado in-person and licensed telemedicine states.
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St. Charbel Rheumatology is located at 3601 S. Pennsylvania St. Suite B, Englewood, CO 80113. Office hours are Monday–Thursday, 8:30 AM – 4:30 PM MT, with limited availability on Fridays. This website is for informational purposes only and does not constitute medical advice. Please consult with a qualified healthcare provider for medical advice, diagnosis, or treatment.