SHM Converge Scientific Abstract ePoster Gallery-Gathering Granules of Evidence for a Diagnosis

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This case describes a rare presentation of granulomatosis with polyangiitis (GPA) in a 22-year-old woman who initially presented with syncope, weight loss, anorexia, chest pain, fever, fatigue, and chills. She had recently moved from Missouri to Georgia and reported exposure to farm chickens and cats. On arrival to the community hospital, she was thin and ill-appearing, with low-normal blood pressure, mild tachycardia, and a BMI of 15.<br /><br />Initial workup showed anemia (Hgb 9.7), elevated high-sensitivity troponin and D-dimer, but normal metabolic profile, transthoracic echocardiogram, lower extremity Dopplers, and CT head. CTA chest revealed a subcarinal mass measuring 4.5 cm with mass effect on the right main pulmonary artery and associated lymphadenopathy. She underwent EBUS/VATS biopsy of the mass and nodes. Pathology demonstrated necrotizing granulomatous inflammation, with negative bacterial, fungal, and AFB cultures, and negative cytology/flow cytometry.<br /><br />She was later readmitted to a large academic center for worsening fatigue, fever, chest pain, and moderate pericardial effusion. A repeat EBUS biopsy was performed, and she transiently improved after dexamethasone. Because histoplasmosis was suspected, itraconazole and prednisone were started. However, she then developed a painful blanching erythematous rash with plaques, especially on the palms and soles.<br /><br />Further evaluation showed elevated 24-hour urine protein, hematuria with dysmorphic red blood cells, C-ANCA positivity at 1:320, strongly positive proteinase-3 (640), and rheumatoid factor >200, consistent with glomerulonephritis and vasculitis. Given the necrotizing granulomatous inflammation, positive serologies, renal involvement, and rash, a diagnosis of GPA was made. She improved after high-dose IV methylprednisolone and her first rituximab dose, and was discharged on hospital day 9. The authors note this as a highly unusual case of GPA initially presenting as a subcarinal mass.

Asset Subtitle

Babar Junaidi

Meta Tag

Author List Babar Junaidi, David Krakow, Giancarlo Licitra, William Britt

Category Clinical Vignettes

Concept Granulomatosis with Polyangiitis

Concept Necrotizing Granulomatous Inflammation

Concept Proteinase 3 ANCA

Concept PR3

Concept Mediastinal Mass

Distinguished Non-Finalist

Presenter Organization Emory University Hospital

Presenting Author Babar Junaidi

Track Adult

Keywords

granulomatosis with polyangiitis

GPA

subcarinal mass

necrotizing granulomatous inflammation

C-ANCA positivity

proteinase-3

glomerulonephritis

rituximab

pericardial effusion

vasculitis

Granulomatosis with Polyangiitis

Necrotizing Granulomatous Inflammation

Proteinase 3 ANCA

PR3

Mediastinal Mass