Podcasts · Core EM - Emergency Medicine Podcast
Episode 211: Granulomatosis with Polyangiitis
· Episode 211
Episode Episode 211: Granulomatosis with Polyangiitis
Granulomatosis with Polyangiitis (GPA) – Recognition and Management in the ED
Hosts: Phoebe Draper, MD Brian Gilberti, MD
https://media.blubrry.com/coreem/content.blubrry.com/coreem/GPA.mp3
Download One Comment Tags: Rheumatology
Show Notes Background
• A vasculitis affecting small blood vessels causing inflammation and necrosis • Affects 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 failure
Red Flag Symptoms:
• Chronic sinus symptoms • Hemoptysis (especially bright red blood) • New pulmonary complaints • Renal dysfunction • Constitutional symptoms (fatigue, weight loss, fever)
Workup in the ED:
• CBC, CMP for anemia and AKI • Urinalysis 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/day • Unstable patients: High-dose IV steroids (methylprednisolone 1 g daily x3 days), consider plasma exchange, cyclophosphamide or rituximab initiation, ICU admission
Conditions that Mimic GPA:
• Goodpasture syndrome (anti-GBM antibodies) • TB, fungal infections • Lung malignancy • Other vasculitides (EGPA, MPA, lupus)
ANCA Testing Utility:
• C-ANCA/PR3-ANCA positive in 80-90% of GPA cases • P-ANCA/MPO-ANCA more common in MPA • Don’t delay treatment while awaiting results if suspicion is high
Outcomes:
• Without treatment: Fatal within a year (renal failure, respiratory complications) • With treatment: 5-year survival ~75-90%, but ~50% relapse rate • Long-term rheumatology follow-up is essential
Take-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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Links in the notes 4
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