Scleroderma (Systemic Sclerosis)
About the disease & condition
Known Symptoms
- Scleroderma is a chronic, multisystem autoimmune disorder characterized by excessive collagen deposition, leading to fibrosis of the skin and internal organs, vascular abnormalities, and immune dysfunction.
- It is classified into two main forms: Limited cutaneous systemic sclerosis (lcSSc): Skin thickening confined to hands, face, and feet.
- Diffuse cutaneous systemic sclerosis (dcSSc): Widespread skin thickening, rapid progression, and higher risk of visceral organ involvement.
Known Causes
- Etiology: Genetic predisposition (e.g., HLA-DRB1, IRF5). Environmental triggers (e.g., silica exposure, solvents, viral infections).
- Pathophysiology: Vascular dysfunction: Endothelial injury causing Raynaud’s phenomenon, intimal hyperplasia, and thrombosis.
- Immune activation: Autoantibodies (e.g., anti-topoisomerase I [Scl-70], anti-centromere) and cytokine dysregulation (e.g., TGF-β, IL-6).
- Fibrosis: Fibroblast activation and collagen overproduction in skin and organs.
Care
- Prevention: Avoid cold exposure (Raynaud’s management), smoking cessation.
Early ACE inhibitor use in high-risk SRC patients. - Follow-up: Regular monitoring of skin, lung, cardiac, and renal function. Annual echocardiogram, PFTs, and serologic testing. Patient education on symptom recognition (e.g., SRC signs: hypertension, headache).
Note: Scleroderma requires multidisciplinary care (rheumatology, pulmonology, cardiology, nephrology). Prognosis varies by subtype; dcSSc has higher mortality due to visceral involvement. New therapies targeting fibrosis and vascular pathways are under investigation.
Relevant Specialties
Rheumatology
The Rheumatology & Clinical Immunology team at our diagnoses and treats disorders that arise when the immune system mistakenly attacks the body. These conditions can affect joints, muscles, skin and internal organs. Our specialists diagnose and combine clinical assessment with imaging, blood tests for autoantibodies and, where needed, tissue sampling, so treatment is based on clear evidence. Procdures are multidisciplinary: rheumatologists and clinical immunologists work closely with physiotherapists, pain specialists, surgeons, obstetricians and paediatric teams to create personalised plans for each patient. Treatment options range from conventional and biologic disease-modifying drugs to joint aspiration and targeted injections, alongside physiotherapy, bone-health management and supervised daycare infusions when required.
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