What are rheumatological emergencies?

Rheumatological Emergencies

Rheumatological emergencies are life-threatening or organ-threatening conditions arising from systemic rheumatic diseases that require immediate recognition and aggressive intervention to prevent irreversible morbidity and mortality. 1, 2, 3

True Life-Threatening Emergencies

These conditions demand immediate critical care and immunosuppression:

Catastrophic Antiphospholipid Syndrome (cAPS)

• Presents with rapid-onset multiorgan thrombosis affecting three or more organ systems within days to weeks 4, 2
• Requires immediate anticoagulation combined with high-dose corticosteroids, plasma exchange, and intravenous immunoglobulin 4, 2
• Mortality approaches 50% without prompt intervention 2

Pulmonary-Renal Syndrome (Kidney-Lung Syndrome)

• Manifests as diffuse alveolar hemorrhage with rapidly progressive glomerulonephritis 4, 2
• Seen in systemic lupus erythematosus, microscopic polyangiitis, and Wegener's granulomatosis 2
• Requires emergent pulse methylprednisolone (1g IV daily for 3 days) plus cyclophosphamide or rituximab 2, 3
• Plasma exchange should be initiated within 24 hours for severe cases 2

Central Nervous System Vasculitis

• Presents with acute stroke, seizures, altered mental status, or acute psychosis in the context of known rheumatic disease 4, 2
• Demands immediate neuroimaging, lumbar puncture when safe, and urgent immunosuppression 2, 3
• Cerebral venous thrombosis requires anticoagulation even in the presence of hemorrhagic transformation 2

Macrophage Activation Syndrome (MAS)

• Characterized by unremitting fever, hepatosplenomegaly, cytopenias, hyperferritinemia (>10,000 ng/mL), elevated triglycerides, and hypofibrinogenemia 4, 3
• Most commonly complicates systemic juvenile idiopathic arthritis and adult-onset Still's disease 3
• Requires immediate high-dose corticosteroids, cyclosporine, and consideration of anakinra or etoposide for refractory cases 4, 3

Neonatal Lupus (Anti-Ro Syndrome)

• Presents with complete congenital heart block in fetuses or neonates born to mothers with anti-Ro/SSA antibodies 4
• Requires immediate pacemaker placement in symptomatic neonates 4
• Maternal treatment with fluorinated corticosteroids (dexamethasone) may prevent progression when detected in utero 4

Organ-Specific Emergencies Requiring Urgent Intervention

Renal Emergencies

• Scleroderma renal crisis: acute hypertensive emergency (BP >150/90 mmHg) with rapidly rising creatinine and microangiopathic hemolytic anemia 2, 3
• Start ACE inhibitors immediately (captopril 6.25-25 mg every 6-8 hours, titrate rapidly) even if creatinine is rising 2
• Rapidly progressive glomerulonephritis in ANCA-associated vasculitis: creatinine doubling within 3 months with active urinary sediment 2, 3
• Requires pulse steroids plus cyclophosphamide or rituximab within 24-48 hours 3

Respiratory Emergencies

• Diffuse alveolar hemorrhage: acute dyspnea, hemoptysis (may be absent initially), bilateral infiltrates, and dropping hemoglobin 2, 3
• Bronchoscopy shows progressively bloodier lavage returns 2
• Immediate pulse methylprednisolone plus cyclophosphamide or rituximab 2, 3
• Acute interstitial pneumonitis in polymyositis/dermatomyositis: rapidly progressive dyspnea over days to weeks 2, 3
• Requires mechanical ventilation support and aggressive immunosuppression 2

Cardiovascular Emergencies

• Cardiac tamponade from pericardial effusion: Beck's triad (hypotension, muffled heart sounds, jugular venous distension) with pulsus paradoxus >10 mmHg 2, 3
• Emergent pericardiocentesis followed by high-dose corticosteroids 2
• Acute myocarditis in systemic lupus erythematosus or polymyositis: new heart failure, arrhythmias, or troponin elevation 2, 3
• Requires ICU monitoring, pulse steroids, and heart failure management 3

Gastrointestinal Emergencies

• Mesenteric vasculitis in polyarteritis nodosa: acute abdominal pain, bloody diarrhea, peritoneal signs 2, 3
• CT angiography shows bowel wall thickening, mesenteric vessel irregularity 3
• Surgical consultation for perforation or infarction plus pulse steroids and cyclophosphamide 2, 3
• Acute pancreatitis in systemic lupus erythematosus or polyarteritis nodosa 2

Ophthalmologic Emergencies

• Temporal arteritis with visual symptoms: sudden vision loss, amaurosis fugax, diplopia in patient >50 years with ESR >50 mm/hr 2, 3
• Start prednisone 1 mg/kg (60-80 mg daily) immediately before temporal artery biopsy 3
• Biopsy should not delay treatment; remains positive for 2 weeks after steroid initiation 3
• Posterior uveitis in Behçet's disease: acute vision loss, floaters, eye pain 2, 3
• Requires immediate high-dose corticosteroids plus azathioprine or anti-TNF therapy 3

Neurological Emergencies

• Atlanto-axial subluxation in rheumatoid arthritis: acute neck pain with myelopathy (hyperreflexia, Babinski sign, sensory level) 2, 3
• Immediate cervical spine immobilization and neurosurgical consultation 2
• MRI to assess cord compression; surgical stabilization often required 3

Infectious Emergencies

• Septic arthritis: acute monoarticular swelling with fever in immunosuppressed patient 2, 3
• Emergent arthrocentesis showing WBC >50,000/μL with >90% neutrophils 3
• Start empiric antibiotics (vancomycin plus ceftriaxone) immediately after cultures, before awaiting results 3
• Urgent orthopedic drainage for hip, shoulder, or prosthetic joint involvement 3

Critical Diagnostic Approach

When a rheumatology patient presents acutely ill, immediately determine if this represents disease flare versus infection, as management is diametrically opposed. 5, 3

Infection vs. Flare Differentiation

• Fever with single organ involvement (especially monoarticular) favors infection 3
• Fever with multi-organ involvement and elevated inflammatory markers favors disease flare 3
• Presepsin >600 pg/mL strongly suggests bacterial infection over disease flare 5
• When uncertain, obtain cultures and start empiric antibiotics while holding immunosuppression escalation 5, 3

Laboratory Red Flags Requiring Immediate Action

• Creatinine rising >0.5 mg/dL in 24 hours with active urine sediment 3
• Platelet count <50,000/μL with new thrombosis (suggests cAPS) 2
• Ferritin >10,000 ng/mL with cytopenias (suggests MAS) 3
• Hemoglobin dropping >2 g/dL in 24 hours without bleeding source (suggests alveolar hemorrhage) 3

Common Pitfalls to Avoid

• Never delay corticosteroids in suspected temporal arteritis while awaiting biopsy; permanent blindness can occur within hours 3
• Never stop ACE inhibitors in scleroderma renal crisis despite rising creatinine; this is the only proven life-saving therapy 2
• Never assume acute monoarthritis is a gout flare in an immunosuppressed patient without arthrocentesis to exclude septic arthritis 3
• Never escalate immunosuppression for presumed disease flare without excluding infection first 5, 3
• Never discharge a rheumatology patient with new neurological signs without MRI to exclude CNS vasculitis or atlanto-axial subluxation 2, 3

Immediate Management Framework

All rheumatological emergencies require simultaneous critical care support and disease-specific intervention. 2, 3

First 60 Minutes

• Establish IV access, continuous monitoring, supplemental oxygen 3
• Obtain complete blood count, comprehensive metabolic panel, inflammatory markers, coagulation studies, blood cultures 3
• Perform targeted imaging based on presentation (CT chest for respiratory symptoms, CT abdomen for GI symptoms, MRI brain for neurological symptoms) 3
• Consult rheumatology and relevant subspecialty (nephrology, pulmonology, neurology) immediately 3

Empiric Immunosuppression When Infection Excluded

• Pulse methylprednisolone 1000 mg IV daily for 3 days for organ-threatening disease 2, 3
• Add cyclophosphamide 500-750 mg/m² IV or rituximab 1000 mg IV for vasculitis or severe lupus 3
• Plasma exchange for cAPS, pulmonary-renal syndrome, or thrombotic thrombocytopenic purpura 2