Granulomatosis with Polyangiitis (GPA) – Recognition and Management in the ED
Hosts:
Phoebe Draper, MD
Brian Gilberti, MD
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)