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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