Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels
<i>Background:</i> Antifibrotic agents have been approved for the treatment of idiopathic pulmonary fibrosis (IPF). However, the efficacy of these drugs in the treatment of familial IPF (FIPF) has not been previously reported. <i>Case presentation:</i> We report the case of a...
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doaj-3e75d9515cda4ed3a90fb8b4f1349d9b2020-11-25T01:13:05ZengMDPI AGMedicina1010-660X2019-05-0155516110.3390/medicina55050161medicina55050161Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin LevelsYasuhiko Koga0Yoshimasa Hachisu1Hiroaki Tsurumaki2Masakiyo Yatomi3Kyoichi Kaira4Shoichiro Ohta5Junya Ono6Kenji Izuhara7Kunio Dobashi8Takeshi Hisada9Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, JapanDepartment of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, JapanDepartment of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, JapanDepartment of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, JapanDepartment of Respiratory Medicine, Comprehensive Cancer Center, International Medical Center, Saitama Medical University, Saitama 350-0495, JapanDivision of Medical Biochemistry, Department of Biomolecular Sciences, Saga Medical School, 5-1-1, Nabeshima, Saga 849-8501, JapanShino-Test Corporation, 2-29-14 Oonodai Minami-ku, Sagamihara, Kanagawa 252-0331, JapanDivision of Medical Biochemistry, Department of Biomolecular Sciences, Saga Medical School, 5-1-1, Nabeshima, Saga 849-8501, JapanJobu Hospital for Respiratory Diseases, 586-1 Taguchi-machi, Maebashi, Gunma 371-0048, JapanGunma University Graduate School of Health Sciences, 3-39-22 sho-wa machi Maebashi, Gunma 371-8514, Japan<i>Background:</i> Antifibrotic agents have been approved for the treatment of idiopathic pulmonary fibrosis (IPF). However, the efficacy of these drugs in the treatment of familial IPF (FIPF) has not been previously reported. <i>Case presentation:</i> We report the case of a 77-year-old man with FIPF, successfully treated with pirfenidone. His uncle died due to IPF, and his niece was diagnosed with the disease. He had worsening dyspnea two months prior to admission to our hospital. Upon admission, he had desaturation when exercising and broad interstitial pneumonia. Administration of pirfenidone improved his dyspnea, desaturation, and the reticular shadow on his chest radiograph. Increased fibrotic marker levels KL-6 and SP-D were also normalized in six months; treatment had no effect on his serum periostin level. Pirfenidone has been effective for over two years. <i>Conclusion:</i> Antifibrotic agents such as pirfenidone may be useful for the management of FIPF, as well as cases of sporadic IPF.https://www.mdpi.com/1010-660X/55/5/161familialidiopathic interstitial pneumoniapirfenidoneidiopathic pulmonary fibrosisperiostin |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Yasuhiko Koga Yoshimasa Hachisu Hiroaki Tsurumaki Masakiyo Yatomi Kyoichi Kaira Shoichiro Ohta Junya Ono Kenji Izuhara Kunio Dobashi Takeshi Hisada |
spellingShingle |
Yasuhiko Koga Yoshimasa Hachisu Hiroaki Tsurumaki Masakiyo Yatomi Kyoichi Kaira Shoichiro Ohta Junya Ono Kenji Izuhara Kunio Dobashi Takeshi Hisada Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels Medicina familial idiopathic interstitial pneumonia pirfenidone idiopathic pulmonary fibrosis periostin |
author_facet |
Yasuhiko Koga Yoshimasa Hachisu Hiroaki Tsurumaki Masakiyo Yatomi Kyoichi Kaira Shoichiro Ohta Junya Ono Kenji Izuhara Kunio Dobashi Takeshi Hisada |
author_sort |
Yasuhiko Koga |
title |
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels |
title_short |
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels |
title_full |
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels |
title_fullStr |
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels |
title_full_unstemmed |
Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels |
title_sort |
pirfenidone improves familial idiopathic pulmonary fibrosis without affecting serum periostin levels |
publisher |
MDPI AG |
series |
Medicina |
issn |
1010-660X |
publishDate |
2019-05-01 |
description |
<i>Background:</i> Antifibrotic agents have been approved for the treatment of idiopathic pulmonary fibrosis (IPF). However, the efficacy of these drugs in the treatment of familial IPF (FIPF) has not been previously reported. <i>Case presentation:</i> We report the case of a 77-year-old man with FIPF, successfully treated with pirfenidone. His uncle died due to IPF, and his niece was diagnosed with the disease. He had worsening dyspnea two months prior to admission to our hospital. Upon admission, he had desaturation when exercising and broad interstitial pneumonia. Administration of pirfenidone improved his dyspnea, desaturation, and the reticular shadow on his chest radiograph. Increased fibrotic marker levels KL-6 and SP-D were also normalized in six months; treatment had no effect on his serum periostin level. Pirfenidone has been effective for over two years. <i>Conclusion:</i> Antifibrotic agents such as pirfenidone may be useful for the management of FIPF, as well as cases of sporadic IPF. |
topic |
familial idiopathic interstitial pneumonia pirfenidone idiopathic pulmonary fibrosis periostin |
url |
https://www.mdpi.com/1010-660X/55/5/161 |
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