What is Scleroderma?
Scleroderma, formally known as Systemic Sclerosis (SSc), is a rare and complex autoimmune connective tissue disease characterised by three core pathological processes: immune system activation (autoimmunity), widespread fibrosis (excessive collagen deposition hardening the skin and organs), and vasculopathy (structural damage to small blood vessels). This triad distinguishes it from all other rheumatic diseases and makes it one of the most challenging conditions to manage.
Scleroderma is classified into two main subtypes: Limited Cutaneous SSc (lcSSc), where skin hardening is limited to the face, hands, and forearms, and Diffuse Cutaneous SSc (dcSSc), where skin involvement is more widespread (above the elbows and knees, and the trunk). Diffuse SSc carries a higher risk of severe internal organ involvement.
Tightened, shiny skin of the hands with loss of skin folds — characteristic changes of systemic sclerosis.
Recognising the Symptoms
Scleroderma has a broad and varied presentation. The most important symptoms to recognize include:
- Raynaud's Phenomenon: The earliest and almost universal manifestation of SSc. Episodes of skin colour change in the fingers in response to cold or stress — classically triphasic: white (blanching due to vasospasm) → blue (cyanosis) → red (hyperaemic reperfusion). Digital ulcers can develop in severe Raynaud's.
- Skin Hardening (Sclerodactyly): Gradual tightening and hardening of the skin, starting in the fingers, causing immobility, contractures, and a characteristic beaked nose and reduced mouth opening (microstomia).
- GERD and Dysmotility: The gastrointestinal tract is the most commonly affected internal organ. Heartburn, difficulty swallowing (dysphagia), bloating, diarrhoea, and malabsorption from intestinal dysmotility are common.
- Interstitial Lung Disease (ILD): Pulmonary fibrosis is the leading cause of death in SSc. Characterised by progressive breathlessness and dry cough. High-resolution CT of the chest is essential for diagnosis.
- Pulmonary Arterial Hypertension (PAH): High blood pressure in the lung arteries — another major cause of mortality in SSc, particularly in lcSSc. Causes progressive breathlessness and right heart failure.
- Scleroderma Renal Crisis: A rare but life-threatening complication of dcSSc — sudden onset of severe hypertension with rapid renal failure. Requires immediate ACE inhibitor treatment.
Raynaud's phenomenon — episodic colour changes in the fingers — is almost universally the first symptom of Scleroderma.
🫁 Lung Monitoring is Critical
All patients with Scleroderma require annual High-Resolution CT (HRCT) of the chest and echocardiography to detect ILD and PAH early, when treatment is most effective. These are not optional tests — they are life-saving screenings that every SSc patient must receive.
Targeted Treatment Approach
Scleroderma treatment targets each of its three pathological pillars — fibrosis, autoimmunity, and vasculopathy — with specific agents:
- For Raynaud's and Vasculopathy: Calcium channel blockers (Nifedipine), PDE5 inhibitors (Sildenafil), endothelin receptor antagonists (Bosentan for digital ulcers).
- For ILD (Fibrosis): Mycophenolate Mofetil or Cyclophosphamide (immunosuppression), and the anti-fibrotic agent Nintedanib (specifically approved for SSc-ILD).
- For PAH: A combination of endothelin receptor antagonists (Ambrisentan, Macitentan), PDE5 inhibitors (Sildenafil, Tadalafil), and prostacyclin analogues, used according to PAH risk stratification.
- For GI Disease: Proton pump inhibitors, prokinetics, rotational antibiotics for bacterial overgrowth.
- For Scleroderma Renal Crisis: ACE inhibitors (Captopril, Enalapril) — a critical emergency treatment.
Dr. Prateek's Approach to Scleroderma
Scleroderma demands a Rheumatologist who understands its multi-system nature and who can coordinate care across pulmonology, cardiology, gastroenterology, and nephrology. Dr. Prateek Deo provides centralised, comprehensive SSc care, functioning as the integrating physician for the complex multi-specialist team these patients require.
Annual Organ Screening
We implement systematic annual HRCT chest and echocardiogram screening for ILD and PAH — the two leading causes of SSc mortality.
Targeted Anti-Fibrotic Therapy
We prescribe disease-modifying agents like Mycophenolate and Nintedanib that directly target the fibrotic process in the lungs, slowing disease progression.
Vascular Protection
Aggressive Raynaud's management with vasodilators to protect digital circulation and prevent painful ulcers and digital infarctions.
Patient Education
We educate patients on warning signs of renal crisis (sudden severe headache, elevated BP) to ensure immediate medical attention can be sought — saving kidneys and lives.