What Is Systemic Sclerosis (Scleroderma)?
Systemic Sclerosis (SSc), also called Scleroderma, is a rare autoimmune connective tissue disease characterised by abnormal immune activation, blood vessel injury, and progressive fibrosis (scarring) of the skin and internal organs. The word “scleroderma” literally means “hard skin.”
While rare, SSc can be serious because it can damage the lungs, heart, kidneys, and gastrointestinal tract. India-specific data is limited, but SSc is seen regularly at tertiary rheumatology centres.
Types of Systemic Sclerosis
- Limited Cutaneous SSc (lcSSc) — skin involvement limited to hands, forearms, face, and feet. Often associated with CREST syndrome (Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasias). Slower progression, better prognosis.
- Diffuse Cutaneous SSc (dcSSc) — widespread skin involvement including the trunk. Higher risk of rapid organ damage. Requires more aggressive monitoring and treatment.
Symptoms of Systemic Sclerosis
Raynaud’s Phenomenon — The First Clue
Raynaud’s is often the first symptom, appearing years before other features. Fingers turn white (ischaemia), then blue (cyanosis), then red (reperfusion) on exposure to cold or stress. In India’s climate, Raynaud’s may seem less prominent, but cold air conditioning and winter months trigger it.
Skin Changes
Skin thickening starting in the fingers (sclerodactyly — tight, shiny skin over fingers), extending up the arms. Puffy, swollen hands in early stages. Salt-and-pepper skin changes. Calcium deposits under skin (calcinosis).
Lung Involvement — Interstitial Lung Disease (ILD)
ILD (lung fibrosis/scarring) is the leading cause of death in SSc. Symptoms: progressive breathlessness on exertion, dry cough. All SSc patients need baseline HRCT chest and pulmonary function tests (PFTs), and regular monitoring.
Pulmonary Arterial Hypertension (PAH)
Elevated blood pressure in the lung arteries — another serious complication. Symptoms: breathlessness, reduced exercise capacity. Annual echocardiography and 6-minute walk test screening is recommended.
GI Involvement
Reflux (GERD), difficulty swallowing (dysphagia), bloating, diarrhoea, constipation — from oesophageal and intestinal motility problems. Proton pump inhibitors are essential to prevent strictures.
Scleroderma Renal Crisis
A rare but life-threatening emergency — sudden onset high blood pressure and acute kidney injury. Requires immediate hospitalisation. ACE inhibitors are the treatment of choice.
Diagnosis
Diagnosis based on 2013 ACR/EULAR criteria. Key antibody tests:
- ANA — positive in >95%
- Anti-Scl-70 (anti-topoisomerase I) — associated with diffuse SSc and ILD risk
- Anti-centromere antibody (ACA) — associated with limited SSc and PAH risk
- Anti-RNA polymerase III — associated with rapid skin progression and renal crisis
Treatment of Systemic Sclerosis
No treatment reverses SSc, but organ-specific therapies prevent progression and manage symptoms:
- ILD: Mycophenolate mofetil (MMF) or nintedanib (anti-fibrotic, recently approved)
- PAH: Sildenafil, bosentan, riociguat
- Raynaud’s: Nifedipine, amlodipine, sildenafil; avoid cold, quit smoking
- Skin disease: Methotrexate for early diffuse skin involvement
- GERD: High-dose proton pump inhibitors, elevate bed head
- Scleroderma renal crisis: ACE inhibitors urgently
Living with Systemic Sclerosis in India
Keep hands warm (gloves, warm water). Use moisturisers for skin. Elevate the head of your bed to prevent nocturnal reflux. Exercise — swimming and gentle exercises maintain mobility. Regular monitoring at a specialist centre is essential. Carry a medical card noting your diagnosis for emergencies.
Written by Dr. Keerthivardhan, Rheumatologist & Immunologist, NIMS Hospital Hyderabad.