Synopsis
Recognise the evolving cutaneous and microvascular pattern of systemic sclerosis across skin tones, distinguish limited from diffuse disease, screen threatened organs promptly, and protect digits, movement and quality of life.
- Raynaud phenomenon and puffy fingers often precede fixed sclerodactyly; nailfold giant capillaries, haemorrhage and capillary loss strengthen suspicion of a systemic-sclerosis spectrum disorder.
- Limited cutaneous disease remains distal to elbows and knees apart from the face, while diffuse cutaneous disease extends proximally or onto the trunk and carries greater early renal, cardiac and interstitial-lung risk.
- Look for fingertip pits and ulcers, telangiectasia, calcinosis, reduced oral aperture, tendon friction rubs, salt-and-pepper pigment change and loss of normal skin folds.
- In brown or black skin, early inflammation may appear violaceous, grey or hyperpigmented; palpated oedema, tethering, sheen, reduced hair and sweat and functional restriction can be more reliable than redness.
- Document the modified Rodnan skin score serially, but never use skin extent alone to exclude interstitial lung disease, pulmonary hypertension, renal crisis, cardiac disease or gastrointestinal involvement.
- Anticentromere antibodies commonly accompany limited disease and later pulmonary arterial hypertension; anti-topoisomerase I supports ILD risk and anti-RNA-polymerase III signals rapid skin progression and renal-crisis vigilance.
- Baseline assessment includes blood pressure, urinalysis, renal tests, full blood count, ECG, pulmonary function with DLCO and high-resolution chest CT, followed by structured annual cardiopulmonary screening.
- First-line uncomplicated Raynaud care combines warmth, trigger and smoking avoidance with a dihydropyridine calcium-channel blocker; sildenafil or intravenous prostanoid belongs in specialist escalation for refractory or ischaemic disease.
- High-dose glucocorticoids increase renal-crisis risk and should be avoided where possible; skin-directed treatment must be coordinated with organ phenotype, rehabilitation and medicine monitoring.
Key red flags
New hypertension, falling renal function, haemolysis, persistent digital colour change with severe pain, rapidly progressive skin thickening, exertional breathlessness, syncope, chest pain, dysphagia with aspiration or marked weight loss requires urgent organ-directed assessment.
Fingertip pits, painful ulcers, splinter haemorrhage, delayed refill or gangrene demonstrate structural vasculopathy rather than uncomplicated vasospasm.
Investigation priorities
Classify distribution, quantify progression and identify threatened digits or fixed disability.
Management branches
Raynaud phenomenon, puffy fingers, sclerodactyly, fingertip injury or scleroderma-pattern capillaries raises systemic sclerosis.
- Map skin extent, ulcers, pits, telangiectasia, calcinosis, oral aperture and hand function and obtain neutral-light photographs with consent.
- Check nailfold capillaries, ANA and phenotype antibodies, blood pressure, FBC, renal function and urine and arrange prompt rheumatology review.
- Obtain baseline ECG, lung physiology with DLCO and HRCT, then assess cardiac, pulmonary-vascular, gastrointestinal and musculoskeletal involvement even if symptoms are mild.
Frequent cold- or stress-induced attacks occur without critical ischaemia.
- Keep core and hands warm, use layered gloves, stop smoking and avoid vibration, vasoconstricting medicines and abrupt temperature change while managing wounds gently.
- Offer a modified-release dihydropyridine calcium-channel blocker such as nifedipine, titrating against attack burden, blood pressure, headache and oedema.
- Review promptly if attacks become persistent, asymmetric or painful or if a pit or ulcer appears; refractory disease needs specialist sildenafil or prostanoid consideration.
Key medicines
Nifedipine modified releaseStart 30 mg by mouth once daily and titrate to 60 mg once daily if needed and tolerated, using the selected product's licensed administration instructions.Check sitting and standing blood pressure and review headache, flushing, ankle oedema, dizziness and interactions; do not allow gradual outpatient titration to delay admission for critical ischaemia.
Sildenafil specialist escalationA commonly used specialist regimen is 20 mg by mouth three times daily, adjusted to response, blood pressure, renal or hepatic function and interacting medicines.Never combine with nitrates or riociguat. Review hypotension, headache, flushing, visual symptoms and priapism and confirm whether pulmonary-hypertension prescribing follows a separate specialist protocol.