Episode 211: Granulomatosis with Polyangiitis

(https://coreem.net/podcast/episode-21...)






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



Hosts:

Phoebe Draper, MD

Brian Gilberti, MD





https://media.blubrry.com/coreem/cont...





Download (https://media.blubrry.com/coreem/cont...)


One Comment (https://coreem.net/podcast/episode-21...)





Tags: Rheumatology (https://coreem.net/tag/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.







Read More (https://coreem.net/podcast/episode-21...)