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Limited Granulomatosis With Polyangiitis Presenting With Pulmonary Embolism FREE TO VIEW

Henry Dinneen, DO; Alpeshkumar Bavishi, MBBS; Amee Patrawalla, MD
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Chest. 2013;144(4_MeetingAbstracts):964A. doi:10.1378/chest.1675113
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: There is an increased incidence of venous thromboembolism (VTE) in patients with granulomatosis with polyangiitis (GPA).(1) We describe a case of pulmonary embolism (PE) as an initial manifestation of limited GPA.

CASE PRESENTATION: A 50 year old male with past history of asthma presented with shortness of breath, productive cough, fever, hemoptysis and right pleuritic pain for two weeks. Physical exam was notable for mild respiratory distress and expiratory wheezing bilaterally. He was febrile with leukocytosis and normal eosinophil count. The erythrocyte sedimentation rate was 87 mm/hr. Chest radiography showed right upper lobe cavitary consolidation. A computed tomography scan of the chest revealed a filling defect in the left interlobar pulmonary artery and muti-focal regions of cavitary consolidation in the right upper lobe. Doppler ultrasound of the lower extremities was negative for deep venous thrombosis. Sputum samples were negative for acid fast bacilli. He was treated for pneumonia and asthma exacerbation without improvement. Flexible bronchoscopy revealed erythematous, friable mucosa throughout the bronchial tree. Video assisted thoracoscopic surgical lung biopsy revealed multiple recent and organizing arterial thromboemboli and chronic pleuritis. A C-ANCA titer was 1:160, and anti-PR3 antibody 58.1 U/ml. The patient was diagnosed with limited GPA and started on cyclophosphamide and prednisone with marked improvement. Anticoagulation was started on admission however was discontinued following persistent hemoptysis.

DISCUSSION: Limited GPA is typically isolated to the upper respiratory tract and lungs. Patients with GPA have a higher incidence of VTE, more so in systemic than in the limited form. The Wegener’s Clinical Occurrence of Thrombosis study found that 3/52 patients with limited GPA had a VTE prior to enrollment in the trial, and 3/52 patients experienced a new VTE during the trial, however the type of thrombotic events were not described.(1) In a study by Weidner and colleagues, 9 cases of VTE were found in patients with severe GPA, of whom 6 had a PE.(2) To our knowledge, this is the first reported case of PE as a presenting manifestation of limited GPA in the English language literature.

CONCLUSIONS: Patients with limited GPA are at increased risk of having VTE. Suspicion for PE as a presenting manifestation should remain high in the appropriate clinical context.

Reference #1: Merkel PA, Lo GH, Holbrook JT, Tibbs AK, Allen NB, Davis JC Jr, et al, and Wegener's Granulomatosis Etanercept Trial Research Group. Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study. Ann Intern Med 2005; 142: 620-6.

Reference #2: Weidner, S., Hafezi-rachti, S. and Rupprecht, H. D. (2006), Thromboembolic events as a complication of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis & Rheumatism, 55: 146-149.

DISCLOSURE: The following authors have nothing to disclose: Henry Dinneen, Alpeshkumar Bavishi, Amee Patrawalla

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