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      Accumulation of periostin in acute exacerbation of familial idiopathic pulmonary fibrosis

      case-report

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          Abstract

          Periostin, an extracellular matrix molecule, is associated with idiopathic pulmonary fibrosis (IPF). It is known that the frequency of familial IPF (FIPF) ranges from 0.5% to 2.2% among IPF cases. However, the relationship between periostin and FIPF has not been previously described. We report the first case of periostin accumulation in the lungs of a patient with an acute exacerbation of FIPF. A 72-year-old woman, diagnosed with FIPF, had been followed up for 5 years. The patient experienced increased dyspnea within a 1-month period and was referred to our hospital. The patient was hypoxic, and chest computed tomography showed rapidly expanding bilateral reticular shadows. Despite pulse-steroid and intravenous-cyclophosphamide therapy, the patient died 25 days after admission. On admission, serum periostin levels were not significantly elevated, while serum fibrotic marker levels were elevated. Immunohistochemical analysis of the lungs on autopsy showed marked accumulation of periostin in the active fibrotic lesions, whereas intact and burned-out areas did not show significant expression of periostin. This case might provide insight into the role of periostin in acute exacerbation of IPF.

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          Author and article information

          Journal
          J Thorac Dis
          J Thorac Dis
          JTD
          Journal of Thoracic Disease
          AME Publishing Company
          2072-1439
          2077-6624
          July 2018
          July 2018
          : 10
          : 7
          : E587-E591
          Affiliations
          [1 ]Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 Showa-machi, Maebashi, Gunma, Japan;
          [2 ]Division of Medical Biochemistry, Department of Biomolecular Sciences, Saga Medical School, 5-1-1 Nabeshima, Saga, Japan;
          [3 ]Department of Human Pathology, Gunma University Graduate School of Medicine, 3-39-15 Maebashi, Gunma, Japan;
          [4 ]Department of Oncology Clinical Development, Gunma University Graduate School of Medicine, 3-39-15 Maebashi, Gunma, Japan;
          [5 ]Gunma University Graduate School of Health Sciences, 3-39-15 Showa-machi, Maebashi, Gunma Japan
          Author notes
          Correspondence to: Yasuhiko Koga. Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 Showa-machi, Maebashi, Gunma 371-8511, Japan. Email: ykoga@ 123456gunma-u.ac.jp .
          Article
          PMC6105951 PMC6105951 6105951 jtd-10-07-E587
          10.21037/jtd.2018.06.96
          6105951
          30174940
          b95930a7-bb8f-4239-b526-3ac60e9e2a6c
          2018 Journal of Thoracic Disease. All rights reserved.
          History
          : 10 February 2018
          : 10 June 2018
          Categories
          Case Report

          Familial idiopathic pulmonary fibrosis (FIPF),acute exacerbation (AE),idiopathic interstitial pneumonia,periostin

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