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MPO-ANCA Negative Microscopic Polyangiitis Presenting With Acute Respiratory Failure FREE TO VIEW

Ruth Haynes, MD; Inga Forde, MD; Paul Cohen, MD; Armand Wolff, MD
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Bridgeport Hospital/Yale New Haven Health, Stratford, CT

Chest. 2015;148(4_MeetingAbstracts):887A. doi:10.1378/chest.2227524
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SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Microscopic Polyangiitis (MPA) is an ANCA-associated systemic necrotizing vasculitis characterized by the absence of both immune deposits and granulomata. Although rapidly progressive glomerulonephritis is present in 90% of patients, alveolar hemorrhage is less common, reported in twelve to twenty-nine percent of cases 1.

CASE PRESENTATION: This is a case of a 42 year old female who presented with dyspnea, hemoptysis and fever in acute hypoxemic respiratory failure requiring mechanical ventilation. Imaging revealed multifocal dense consolidations throughout both lungs and mediastinal adenopathy. Laboratory data showed evidence of acute kidney injury and urinalysis demonstrated granular and white blood cell casts without red blood cells. Despite empiric broad spectrum antimicrobial therapy, there was no improvement and she underwent bronchoalveolar lavage confirming alveolar hemorrhage. Anti-proteinase 3, anti-myleoperoxidase and anti-glomerular basement membrane were negative. With her clinical deterioration, high dose systemic steroids were started and the patient underwent a thoracoscopy with wedge resection. There was improvement of her pulmonary and renal compromise shortly thereafter. Pathology revealed capillaritis without granulomata with negative anti-GBM immunofluorescence. Tissue microbiology was positive for group A Streptococcus.

DISCUSSION: MPA is a small vessel systemic necrotizing vasculitis in the absence of granulomata. Ten percent of patients are ANCA negative 2. Prompt implementation of systemic steroids has been shown to ameliorate pulmonary-renal syndrome caused by ANCA-negative MPA.

CONCLUSIONS: In summary, this is a case of MPO-ANCA antibody negative MPA confirmed by wedge biopsy with the pathognomonic features of capillaritis in the absence of granulomata.

Reference #1: Lauque, D., Cadranel, J. et al. Microscopic Polyangiitis with Alveolar Hemorrhage A Study of 29 Cases and Review of the Literature. Medicine July 2000- vol.79 issue 4 ppg 222-233

Reference #2: Falk RJ, Jeanette JC. ANCA small vessel vasculitis. J Am Soc Nephrol 1997; 8:314

DISCLOSURE: The following authors have nothing to disclose: Ruth Haynes, Inga Forde, Paul Cohen, Armand Wolff

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