Synopsis
Recognise systemic sclerosis from vascular, skin and organ features, define limited or diffuse phenotype and autoantibody risk, screen lungs, heart and kidneys proactively, and treat each complication before irreversible fibrosis develops.
- Suspect systemic sclerosis when Raynaud phenomenon accompanies puffy fingers, sclerodactyly, fingertip pits or ulcers, telangiectasia, abnormal nailfold capillaries or characteristic internal-organ disease.
- Classify skin involvement as limited or diffuse, but do not use the skin label as a substitute for organ screening; severe PAH, ILD, gut or cardiac disease can occur in either group.
- Request ANA with systemic-sclerosis-specific antibodies, perform nailfold capillaroscopy where available and document a modified Rodnan skin score, joints, tendons, ulcers and function.
- At diagnosis obtain HRCT chest and pulmonary function tests including DLCO to detect ILD; follow lung trajectory rather than waiting for advanced dyspnoea.
- Screen for pulmonary hypertension at least annually using symptoms, examination, PFT or DLCO, NT-proBNP and echocardiography, applying DETECT or local right-heart-catheter criteria in eligible patients.
- Check blood pressure and creatinine regularly and during new symptoms or glucocorticoid exposure; anti-RNA-polymerase-III and rapidly progressive diffuse skin disease increase renal-crisis risk.
- For Raynaud phenomenon use warmth, smoking cessation and a dihydropyridine calcium-channel blocker; escalate recurrent ulcers or critical ischaemia to sildenafil, intravenous iloprost and specialist vascular care.
- Treat reflux early, commonly with a proton-pump inhibitor plus positioning and meal measures; investigate dysphagia, weight loss, anaemia, aspiration or altered bowel habit rather than repeatedly increasing acid suppression.
- Mycophenolate is widely used for progressive skin or ILD disease, while nintedanib slows FVC decline in eligible SSc-ILD; neither reverses established scar.
- Avoid high-dose glucocorticoid when possible because renal-crisis risk rises; if unavoidable, use the minimum exposure with close home blood-pressure and renal monitoring.
Key red flags
A new headache, visual symptoms, encephalopathy, hypertension, oliguria or creatinine rise may indicate scleroderma renal crisis and requires immediate ACE-inhibitor treatment.
Rest pain, a cold pale or blue digit, ulcer progression or necrosis requires urgent vascular assessment and intravenous vasodilator consideration.
New exertional syncope, rapidly falling exercise tolerance, chest pain or right-heart signs may indicate pulmonary hypertension or myocardial disease.
Acute hypoxia, new diffuse opacity or a sudden lung-function fall requires urgent assessment for infection, ILD acceleration, aspiration, embolism or heart failure.
Dysphagia with aspiration, inability to maintain intake, obstruction symptoms or gastrointestinal bleeding needs urgent gastroenterology and nutritional review.
Disproportionate breathlessness, falling DLCO, raised NT-proBNP, syncope or right-heart signs requires rapid specialist evaluation and right-heart catheterisation when indicated.
Persistent rest pain, pallor, cyanosis, ulcer deepening or gangrene is an emergency distinct from an uncomplicated brief Raynaud attack.
Investigation priorities
Support diagnosis and stratify organ surveillance within a compatible clinical phenotype.
Management branches
Raynaud, puffy or sclerotic fingers and capillary or antibody features suggest systemic sclerosis.
- Document skin distribution and score, nailfolds, ulcers, pulses, joints, strength, reflux, bowel, respiratory and cardiac symptoms, pressure and weight.
- Request antibodies, FBC, renal and liver profile, urinalysis, ECG, baseline HRCT and PFT with DLCO; obtain echocardiography and NT-proBNP for cardiopulmonary screening.
- Refer to a systemic-sclerosis service, communicating antibody and organ-risk phenotype and arranging early lung, renal, vascular and gastrointestinal prevention.
Recurrent vasospasm causes pain or functional limitation without current critical ischaemia.
- Use whole-body warming, layered gloves, smoking cessation, trigger and medicine review, skin care and prompt treatment of cracks or infection.
- Start and titrate a dihydropyridine calcium-channel blocker such as nifedipine while monitoring pressure, oedema, headache and reflux.
- For persistent severe attacks or ulcers, add sildenafil and involve the specialist team for intravenous iloprost, wound care and vascular imaging when indicated.