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Systemic sclerosis (scleroderma)
Systemic sclerosis almost always begins with Raynaud phenomenon and shows skin thickening; the antibodies predict complications — anti-centromere for the limited form (pulmonary hypertension), anti-Scl-70 for the diffuse form (interstitial lung disease), and anti-RNA polymerase III for renal crisis — which is treated with an ACEi even as the creatinine rises.
Why it matters in the exam
The question presents a woman with Raynaud, puffy fingers and later thickened skin, heartburn and difficulty swallowing. The candidate must separate the limited form — skin distal to the elbows and knees and on the face, calcinosis, telangiectasia, anti-centromere and, late on, pulmonary hypertension — from the diffuse form — proximal and truncal skin, rapid progression, anti-Scl-70 and early interstitial lung disease. Skin thickening of the fingers extending above the MCP joints is enough for classification. Nailfold capillaroscopy separates primary from secondary Raynaud.
The emergency that is tested is scleroderma renal crisis: new severe hypertension, AKI and sometimes microangiopathic haemolytic anaemia, mainly in the diffuse form with anti-RNA polymerase III and after high-dose steroids (prednisone above 15 mg). Treatment is an ACEi (captopril) at once, even when creatinine keeps rising. Yearly follow-up includes an echocardiogram and lung function with DLCO, and HRCT at diagnosis. Treatments: Raynaud — a calcium-channel blocker (nifedipine), and for ulcers a PDE5 inhibitor or iloprost; lung disease — mycophenolate, nintedanib or tocilizumab; reflux — a PPI.
Key points
- Raynaud and skin thickening; above the MCP joints is enough for classification.
- Anti-centromere — limited and pulmonary hypertension; anti-Scl-70 — diffuse and lung disease.
- Anti-RNA polymerase III and steroids above 15 mg — renal crisis.
- Renal crisis: captopril at once, even with a creatinine rise.
- Yearly follow-up: echocardiogram and lung function with DLCO.
Summary table
| Form / complication | Clue | Antibody / test | Treatment |
|---|---|---|---|
| Limited | Distal and facial skin, calcinosis, telangiectasia | Anti-centromere | Follow up for pulmonary hypertension |
| Diffuse | Proximal and truncal skin, rapid progression | Anti-Scl-70 | Early screening for lung disease |
| Renal crisis | New hypertension, AKI, microangiopathic haemolysis | Anti-RNA polymerase III; steroids above 15 mg | Captopril at once, even as creatinine rises |
| Interstitial lung disease | Breathlessness, low DLCO | HRCT | Mycophenolate, nintedanib, tocilizumab |
| Raynaud and ulcers | White-blue-red fingers | Capillaroscopy | Nifedipine; PDE5 inhibitor or iloprost for ulcers |
| Yearly follow-up | Pulmonary hypertension and lung disease | Echocardiogram, lung function with DLCO | PPI for reflux |
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Sources
- ACR/EULAR Classification Criteria for Systemic Sclerosis, 2013 — Skin thickening, antibodies and organ involvement
- EULAR recommendations for the treatment of systemic sclerosis, 2023 — Raynaud, digital ulcers, ILD, PAH and renal crisis
- Harrison's Principles of Internal Medicine, 21st edition (2022) — Systemic Sclerosis (Scleroderma) and Related Disorders
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-02. The text may contain errors; when in doubt, check the source.