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Granulomatosis With Polyangiitis Twelve Years After Renal Anti-GBM Vasculitis FREE TO VIEW

Biana Manchik, BS; Fadi Aldaas, MD; Kavitha Gopalratnam, MD; Jeff Kwon, MD
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Bridgeport Hospital, Bridgeport, CT

Chest. 2015;148(4_MeetingAbstracts):879A. doi:10.1378/chest.2266867
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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: Anti-glomerular basement membrane (anti-GBM) disease occasionally overlaps with anti-nuclear cytoplasmic antibody (ANCA) positive vasculitis. We report a case of pulmonary granulomatosis with polyangiitis (GPA) presenting 12 years after anti-GBM renal disease.

CASE PRESENTATION: A 47-year-old gentleman with a past medical history of biopsy-proven renal anti-GBM vasculitis, complicated by end-stage renal disease, presented with substernal chest pain radiating to the left arm. He had no cough, fever, or hemoptysis. A chest computed tomography (CT) was performed and demonstrated several nodules in the right and left lung (Image 1). Twelve years ago he was diagnosed with renal anti-GBM and treated with plasmapheresis, mycophenolate mofetil and steroids. His treatment was eventually reduced to low doses of prednisone. Lab work showed a sedimentation rate of 124 mm/hr, and serum pANCA was detectable at 1:80. Serum anti-GBM and PR3 antibodies were negative. Complete cardiac and infectious workup was negative. The patient underwent a surgical lung biopsy, which demonstrated “dirty necrosis” with giant cells consistent with necrotizing GPA (Image 2). He was subsequently started on rituximab therapy.

DISCUSSION: Concurrent presentation of GPA and anti-GBM disease has been well described in previous published case reports. Our case is unique as it presents a patient with pulmonary GPA vasculitis occurring 12 years after renal anti-GBM. The longest interval between GPA and anti-GBM disease that has been previously reported is less than a month. It is uncertain why our patient’s case of GPA was so temporally separate in relation to his anti-GBM disease. We speculate that the patient’s use of low dose prednisone resulted in a low grade case of GPA that had gone unrecognized over years. The incidental nature of the discovery of lung nodules on CT and lack of classic symptoms of vasculitis support this possibility.

CONCLUSIONS: Overlap of vasculitis syndromes occur on rare occasions and may present separately over a prolonged time period. In patients with a previous history of anti-GBM disease spanning months or years, clinicians should consider possible GPA in the appropriate clinical setting.

Reference #1: Gmurczyk, A. A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies. Scientific World Journal. 2010; 10:1078-83.

Reference #2: Clyne, S. Concurrent and discrete clinicopathological presentations of Wegener granulomatosis and anti-glomerular basement membrane disease. Am J Kidney Dis. 2009; 54(6):1116-20.

Reference #3: O'Donoghue, DJ. Sequential development of systemic vasculitis with anti-neutrophil cytoplasmic antibodies complicating anti-glomerular basement membrane disease. Clin Nephrol. 1989; 32(6):251-5.

DISCLOSURE: The following authors have nothing to disclose: Biana Manchik, Fadi Aldaas, Kavitha Gopalratnam, Jeff Kwon

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