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, cardiopulmonary or ischaemic crisis
New severe hypertension or AKI, rapidly worsening breathlessness, hypoxia, syncope, chest pain, arrhythmia, myocarditis, critical digital ischaemia or sepsis requires same-day specialist assessment.
Action: Measure blood pressure and creatinine immediately, obtain ECG, troponin, chest imaging and organ-directed tests, start captopril promptly for suspected renal crisis, and involve rheumatology with renal, cardiopulmonary or vascular teams.
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.
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.
Pulmonary hypertension
Disproportionate breathlessness, falling DLCO, raised NT-proBNP, syncope or right-heart signs requires rapid specialist evaluation and right-heart catheterisation when indicated.
Investigation priorities
01
ANA and systemic-sclerosis-specific antibodiesFirst step
Support diagnosis and stratify organ surveillance within a compatible clinical phenotype.
Management branches
First-line confirmationDefine phenotype and baseline organs
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.
Preferred Raynaud sequenceWarm, vasodilate and protect tissue
Recurrent vasospasm causes pain or functional limitation without current critical ischaemia.
Key medicines
Nifedipine modified releaseStart 30 mg orally once daily and titrate, commonly to 60 mg once daily, according to Raynaud response, blood pressure and the selected modified-release product.
SildenafilA common specialist digital-vasculopathy regimen is 20 mg orally three times daily, titrated only under the relevant Raynaud, ulcer or pulmonary-hypertension protocol.
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.