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

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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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