Episode 211: Granulomatosis with Polyangiitis

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Jul 1, 2025

Phoebe Draper, an emergency medicine resident, dives into granulomatosis with polyangiitis (GPA), a serious vasculitis affecting small blood vessels. She highlights red flag symptoms like chronic sinus issues and hemoptysis that emergency physicians should recognize. The conversation covers diagnostic essentials, including CBC and ANCA testing, and urgent management strategies for unstable patients. Draper stresses the significance of timely intervention to avoid severe complications, making GPA a critical topic for emergency care.

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ANECDOTE

Patient Case Introduction

A 23-year-old woman presented with two weeks of shortness of breath, bloody cough, and leg swelling.
She looked very sick with pallor, tachycardia, and leg edema, raising concern for serious disease.

INSIGHT

GPA Pathophysiology and Impact

GPA is a vasculitis causing inflammation and necrosis of small vessels affecting upper respiratory tract, lungs, and kidneys.
It can lead to potentially fatal multi-organ failure if missed.

ADVICE

Red Flags to Recognize GPA

Suspect GPA in patients with chronic sinus symptoms, hemoptysis, new pulmonary complaints, or renal dysfunction.
Also consider constitutional symptoms like fatigue, weight loss, and fever for subtle presentations.

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Granulomatosis with Polyangiitis (GPA) – Recognition and Management in the ED

Hosts:
Phoebe Draper, MD
Brian Gilberti, MD

Download Leave a 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.