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Title: Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease
Authors: Clifford, Liam ;Li, J.;Renaud, C.J.;Fernando, S.L.
Affliation: Central Coast Local Health District
Gosford Hospital
Issue Date: Dec-2019
Source: 2019:2673947
Journal title: Case reports in rheumatology
Department: Medicine
Abstract: Background: Microscopic polyangiitis (MPA) can manifest with atypical features such as pulmonary fibrosis and chronic obstructive pulmonary disease (COPD), which are atypical and unusual features of small vessel vasculitis. Case Presentation: This paper presents two patients with microscopic polyangiitis and respiratory symptoms attributable to atypical pulmonary manifestations. Pulmonary fibrosis was present in both cases, with COPD also present in one patient. Management involved methylprednisone, prednisone, and cyclophosphamide. The second patient also received azathioprine. Both patients responded well to immunosuppressive treatment; however, pulmonary fibrosis and COPD were refractory to immunosuppression. Conclusion: Pulmonary manifestations including pulmonary fibrosis, emphysema, and bronchiectasis are observed in MPA. Evaluation of MPA in unexplained cases should be performed to avoid delays in diagnosis and management. Patients who present with MPA with pulmonary manifestations may respond to treatment, but their pulmonary features demonstrate a refractory nature to such management.
DOI: 10.1155/2019/2673947
ISSN: 2090-6897
Publicaton type: Journal Article
Keywords: Drug Therapy
Study or Trial: Case Series and Case Reports
Appears in Collections:Health Service Research

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