Core EM - Emergency Medicine Podcast

Episode 211: Granulomatosis with Polyangiitis

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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
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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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