ICU · renal-metabolic
Rheumatological Emergencies in the ICU
Also known as Rheumatological emergencies · Scleroderma renal crisis · Systemic sclerosis renal crisis · Neuropsychiatric lupus · Lupus cerebritis · Antisynthetase syndrome · Inflammatory myositis ICU · Rheumatoid vasculitis · Connective tissue disease in ICU · Autoimmune emergency ICU
Rheumatological emergencies in the ICU — life-threatening presentations of systemic autoimmune diseases requiring critical care: (1) scleroderma renal crisis (SRC — sudden malignant hypertension + AKI + microangiopathic haemolysis in diffuse cutaneous systemic sclerosis — ACE inhibitor is LIFE-SAVING), (2) SLE emergencies (neuropsychiatric lupus — seizures, psychosis, transverse myelitis; lupus pneumonitis; lupus nephritis with RPGN; catastrophic antiphospholipid syndrome), (3) inflammatory myopathy with interstitial lung disease (antisynthetase syndrome — anti-Jo-1, anti-MDA5 — rapidly progressive ILD), (4) rheumatoid vasculitis and atlantoaxial subluxation, (5) adult-onset Still disease with MAS. Management: ACE inhibition, pulse methylprednisolone with cyclophosphamide, triple immunosuppression for anti-MDA5 RP-ILD, difficult-airway anticipation in RA, and PAH-targeted therapy in systemic sclerosis.
Checked against its sources on 2 Sept 2026
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- Scleroderma renal crisis: sudden malignant hypertension + AKI + microangiopathic haemolysis in systemic sclerosis = START AND UPTITRATE AN ACE INHIBITOR IMMEDIATELY — this is the ONE intervention that changes outcome
- Anti-MDA5 dermatomyositis: rapidly progressive ILD (within days-weeks) with high mortality — high-dose steroids + calcineurin inhibitor ± cyclophosphamide early — check anti-MDA5 and ferritin in ANY patient with rapidly progressive ILD + Gottron papules/mechanic's hands
- Neuropsychiatric lupus: seizures, psychosis, transverse myelitis, or cognitive dysfunction in SLE = pulse methylprednisolone + cyclophosphamide — distinguish from steroid-induced neuropsychiatric effects (dose-related, recover when the steroid dose falls)
- Atlantoaxial subluxation in RA: neck pain + neurological symptoms + RA = flexion-extension X-ray/MRI BEFORE intubation — in-line stabilisation during laryngoscopy — anterior subluxation can compress spinal cord during neck extension for intubation
- Cricoarytenoid arthritis in RA: hoarseness + stridor + RA = difficult airway — fiberoptic intubation preferred — cricothyroidotomy may be anatomically distorted
- Systemic sclerosis + refractory hypoxaemia: screen for pulmonary arterial hypertension with annual echo (SSc accounts for most CTD-associated PAH) — right heart catheter for confirmation
- DO NOT withhold ACE inhibitor in scleroderma renal crisis even if creatinine rises — with ACE inhibitors 1-year survival is 76% versus 15% without, and over half of patients still on dialysis after 3 months who continue the ACE inhibitor come off dialysis — a rising creatinine is NOT a reason to stop
Meet the patient
A 47-year-old woman with diffuse cutaneous systemic sclerosis, started on prednisone 30 mg daily two weeks ago for progressive interstitial lung disease, arrives on your unit with a thunderclap headache and blurred vision. BP 196 over 118, fundi show flame haemorrhages, creatinine has jumped from a baseline of 78 to 186, and the blood film is full of schistocytes. The registrar reaches for amlodipine.[1]
That reach is moment exam question lives or dies. The two questions that decide her next 24 hours are the two that decide every rheumatology emergency: is this a disease-specific crisis with a disease-specific drug? (yes — scleroderma renal crisis, and the drug is an ACE inhibitor), and will you persist with that drug when the creatinine climbs? (you will — because renal function recovers in most survivors who persist, and stopping is how patients stay on dialysis). Hold those two questions and the whole topic slots into place.[2][3]
[7]References33ShowHide
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