Granulomatosis with Polyangiitis (GPA) – Recognition and Management in the ED
Phoebe Draper, MD
https://media.blubrry.com/coreem/content.blubrry.com/coreem/GPA.mp3
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Tags: Rheumatology
Show Notes
Background
A vasculitis affecting small blood vessels causing inflammation and necrosisAffects upper respiratory tract (sinusitis, otitis media, saddle nose deformity), lungs (nodules, alveolar hemorrhage), and kidneys (rapidly progressive glomerulonephritis)Can lead to multi-organ failure, pulmonary hemorrhage, renal failureRed Flag Symptoms:
Chronic sinus symptomsHemoptysis (especially bright red blood)New pulmonary complaintsRenal dysfunctionConstitutional symptoms (fatigue, weight loss, fever)Workup in the ED:
CBC, CMP for anemia and AKIUrinalysis with microscopy (hematuria, RBC casts)Chest imaging (CXR or CT for nodules, cavitary lesions)ANCA testing (not immediately available but important diagnostically)Management:
Stable patients: Outpatient workup, urgent rheumatology consult, prednisone 1 mg/kg/dayUnstable patients: High-dose IV steroids (methylprednisolone 1 g daily x3 days), consider plasma exchange, cyclophosphamide or rituximab initiation, ICU admissionConditions that Mimic GPA:
Goodpasture syndrome (anti-GBM antibodies)TB, fungal infectionsLung malignancyOther vasculitides (EGPA, MPA, lupus)ANCA Testing Utility:
C-ANCA/PR3-ANCA positive in 80-90% of GPA casesP-ANCA/MPO-ANCA more common in MPADon’t delay treatment while awaiting results if suspicion is highOutcomes:
Without treatment: Fatal within a year (renal failure, respiratory complications)With treatment: 5-year survival ~75-90%, but ~50% relapse rateLong-term rheumatology follow-up is essentialTake-Home Points:
Always include vasculitis in the differential for unexplained respiratory, renal, or systemic symptoms.Recognize pulmonary-renal syndromes early.Initiate high-dose steroids immediately for unstable patients without waiting for ANCA results.GPA is rare but life-threatening – early recognition saves lives.
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