Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease
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 polyang...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2019/2673947 |
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| author | Liam M. Clifford Jamma Li Christopher J. Renaud Suran L. Fernando |
| author_facet | Liam M. Clifford Jamma Li Christopher J. Renaud Suran L. Fernando |
| author_sort | Liam M. Clifford |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-05717c8e2fb249b0b0a6c080a03126a2 |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-05717c8e2fb249b0b0a6c080a03126a22025-02-03T01:11:49ZengWileyCase Reports in Rheumatology2090-68892090-68972019-01-01201910.1155/2019/26739472673947Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the DiseaseLiam M. Clifford0Jamma Li1Christopher J. Renaud2Suran L. Fernando3Department of General Medicine, Gosford Hospital, Gosford, NSW, AustraliaDepartment of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, NSW, AustraliaDepartment of Anatomical Pathology, Royal North Shore Hospital, Sydney, NSW, AustraliaDepartment of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, NSW, AustraliaBackground. 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.http://dx.doi.org/10.1155/2019/2673947 |
| spellingShingle | Liam M. Clifford Jamma Li Christopher J. Renaud Suran L. Fernando Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease Case Reports in Rheumatology |
| title | Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease |
| title_full | Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease |
| title_fullStr | Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease |
| title_full_unstemmed | Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease |
| title_short | Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease |
| title_sort | microscopic polyangiitis with pulmonary fibrosis an often recognized manifestation of the disease |
| url | http://dx.doi.org/10.1155/2019/2673947 |
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