What is Scleroderma?
Systemic sclerosis is characterised by three processes acting together: small-vessel damage, immune activation, and excessive collagen deposition causing fibrosis of skin and internal organs.
It ranges from limited cutaneous disease, where skin change stays distal to elbows and knees, to diffuse disease with rapid widespread skin thickening and early internal organ involvement.
Outcomes are now driven almost entirely by two complications: interstitial lung disease and pulmonary arterial hypertension. Both are silent early, which is why annual screening rather than symptom-based testing is the standard of care.
IllustrativeSymptoms of Scleroderma
Early signs
- Raynaud's phenomenon — fingers turning white, then blue, then red with cold or stress
- Puffy, swollen fingers that later become tight and shiny
- Difficulty making a fist or opening the mouth fully
- Acid reflux, difficulty swallowing solids, early satiety
- Small dilated blood vessels on face and hands (telangiectasia)
If left untreated
- Painful digital ulcers or pitting scars at the fingertips
- Progressive breathlessness and dry cough (interstitial lung disease)
- Breathlessness on exertion with swollen ankles (pulmonary hypertension)
- Calcium deposits under the skin (calcinosis)
- Sudden severe hypertension with kidney failure (scleroderma renal crisis)
Causes & risk factors
- Autoimmune-driven fibroblast activation and excessive collagen production
- Endothelial injury and microvascular obliteration
- Specific autoantibodies: anti-centromere, anti-Scl-70 (topoisomerase I), anti-RNA polymerase III
- Occupational silica, solvent or vinyl chloride exposure in a minority
- Female sex, with onset typically between 30 and 50
How Scleroderma is diagnosed
No single test diagnoses a rheumatic disease. The diagnosis comes from combining the pattern of symptoms, the examination findings, and only the investigations that answer a specific question.
Nailfold capillaroscopy
A simple, non-invasive look at nailfold capillaries. Giant loops, haemorrhages and capillary dropout distinguish secondary Raynaud's from harmless primary Raynaud's — often years before other features appear.
Scleroderma-specific antibodies
Anti-centromere suggests limited disease with higher pulmonary hypertension risk; anti-Scl-70 suggests diffuse disease with lung fibrosis; anti-RNA polymerase III flags renal crisis and malignancy risk.
High-resolution CT chest and lung function
HRCT plus spirometry and DLCO at baseline and annually. Interstitial lung disease is detected on imaging long before the patient reports breathlessness.
Echocardiography and NT-proBNP
Annual screening for pulmonary arterial hypertension, with right heart catheterisation to confirm when suspected.
Skin score and GI assessment
Modified Rodnan skin score to track progression objectively; oesophageal manometry or endoscopy where reflux is severe.
Blood pressure self-monitoring
Patients are taught to check blood pressure at home, because scleroderma renal crisis presents as an abrupt hypertensive emergency.
Treatment options
Organ-based treatment strategy
There is no single 'scleroderma drug'. Each organ system is assessed and treated on its own track, which is why coordinated specialist follow-up matters so much.
Raynaud's and digital ulcer therapy
Calcium channel blockers, PDE-5 inhibitors such as sildenafil, and bosentan for recurrent ulcers, plus rigorous cold protection.
Interstitial lung disease
Mycophenolate mofetil, nintedanib or tocilizumab depending on pattern and progression rate, with cyclophosphamide in selected rapidly progressive cases.
Pulmonary arterial hypertension
Managed jointly with cardiology or pulmonology using targeted vasodilator therapy — early detection substantially improves survival.
Gastrointestinal management
Proton pump inhibitors, prokinetics, small frequent meals, head-of-bed elevation and rotating antibiotics for bacterial overgrowth.
ACE inhibitors for renal crisis
Started immediately and continued lifelong at crisis onset. Notably, prophylactic ACE inhibitors are not used, and high-dose steroids are avoided because they precipitate renal crisis.
Living with Scleroderma
- Keep the whole body warm, not just the hands — core warmth prevents peripheral vasospasm
- Stop smoking absolutely; nicotine directly constricts the very vessels already compromised
- Moisturise skin daily and protect fingertips from minor trauma
- Eat smaller, more frequent meals and stay upright for two hours afterwards
- Check blood pressure at home weekly and report any sustained rise immediately
Seek urgent medical attention if you have
- A sudden rise in blood pressure with headache or reduced urine output — renal crisis is an emergency
- Increasing breathlessness or a new dry cough
- A blackened fingertip or a new painful digital ulcer
- Chest pain, palpitations or fainting
- Rapidly spreading skin tightening over weeks
Do not wait for a scheduled appointment for any of the above. Call the clinic on +91 70571 19999 or attend the nearest emergency department.
Frequently asked questions about Scleroderma
Does scleroderma always affect internal organs?
My fingers go white in the cold. Do I have scleroderma?
Is there any treatment that reverses skin tightening?
Get an expert opinion on Scleroderma in Pune
Consultations at the Ravet clinic in Pimpri-Chinchwad. Bring your previous reports — a properly reviewed history usually saves repeating tests.
Part of a wider group
Autoimmune & Connective Tissue DiseaseMulti-system disease where the complications that matter are silent early.Related conditions
Raynaud's Phenomenon
Usually harmless — but sometimes the first sign of autoimmune disease years in advance.
Lupus (SLE)
A multi-system autoimmune disease where early kidney screening changes the entire prognosis.
Sjögren's Syndrome
Dry eyes and dry mouth that deserve investigation rather than eye drops alone.
Myositis
Weakness — not pain — is the defining symptom, and it needs urgent investigation.