Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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Renal crisis or critical digital ischaemia
A new blood-pressure rise with headache, visual symptoms, acute kidney injury or microangiopathic haemolysis suggests scleroderma renal crisis; a persistently cold, pale or blue painful digit, progressive ulcer or gangrene indicates threatened tissue.
Action: Arrange same-day hospital assessment, urgent renal and blood testing and specialist treatment; renal crisis requires immediate ACE-inhibitor therapy under close monitoring, while critical digital ischaemia needs vascular assessment, analgesia and vasodilator rescue without waiting for routine review.
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.
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.
Digital vascular damage
Fingertip pits, painful ulcers, splinter haemorrhage, delayed refill or gangrene demonstrate structural vasculopathy rather than uncomplicated vasospasm.
Investigation priorities
01
Full skin and vascular examinationFirst step
Classify distribution, quantify progression and identify threatened digits or fixed disability.
Management branches
First diagnostic sequenceConnect early skin change to organ screening
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.
First-line Raynaud careReduce vasospasm before tissue is lost
Frequent cold- or stress-induced attacks occur without critical ischaemia.
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.
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.
National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.