Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease.

Autor: Clifford LM; Department of General Medicine, Gosford Hospital, Gosford, NSW, Australia., Li J; Department of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, NSW, Australia.; Immunorheumatology Laboratory, New South Wales Health Pathology, Royal North Shore Hospital, Sydney, NSW, Australia.; University of Sydney, Sydney, NSW, Australia., Renaud CJ; Department of Anatomical Pathology, Royal North Shore Hospital, Sydney, NSW, Australia., Fernando SL; Department of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, NSW, Australia.; Immunorheumatology Laboratory, New South Wales Health Pathology, Royal North Shore Hospital, Sydney, NSW, Australia.; University of Sydney, Sydney, NSW, Australia.
Jazyk: angličtina
Zdroj: Case reports in rheumatology [Case Rep Rheumatol] 2019 Dec 31; Vol. 2019, pp. 2673947. Date of Electronic Publication: 2019 Dec 31 (Print Publication: 2019).
DOI: 10.1155/2019/2673947
Abstrakt: 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.
Competing Interests: The authors declare that they have no conflicts of interest.
(Copyright © 2019 Liam M. Clifford et al.)
Databáze: MEDLINE
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