{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T03:39:00Z","timestamp":1760240340286,"version":"build-2065373602"},"reference-count":22,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2019,5,17]],"date-time":"2019-05-17T00:00:00Z","timestamp":1558051200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background: 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. Case presentation: 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. Conclusion: Antifibrotic agents such as pirfenidone may be useful for the management of FIPF, as well as cases of sporadic IPF.<\/jats:p>","DOI":"10.3390\/medicina55050161","type":"journal-article","created":{"date-parts":[[2019,5,17]],"date-time":"2019-05-17T11:06:46Z","timestamp":1558091206000},"page":"161","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels"],"prefix":"10.3390","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4974-9026","authenticated-orcid":false,"given":"Yasuhiko","family":"Koga","sequence":"first","affiliation":[{"name":"Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshimasa","family":"Hachisu","sequence":"additional","affiliation":[{"name":"Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0837-9329","authenticated-orcid":false,"given":"Hiroaki","family":"Tsurumaki","sequence":"additional","affiliation":[{"name":"Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masakiyo","family":"Yatomi","sequence":"additional","affiliation":[{"name":"Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 sho-wa machi Maebashi, Gunma 371-8511, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyoichi","family":"Kaira","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Comprehensive Cancer Center, International Medical Center, Saitama Medical University, Saitama 350-0495, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4487-3036","authenticated-orcid":false,"given":"Shoichiro","family":"Ohta","sequence":"additional","affiliation":[{"name":"Division of Medical Biochemistry, Department of Biomolecular Sciences, Saga Medical School, 5-1-1, Nabeshima, Saga 849-8501, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junya","family":"Ono","sequence":"additional","affiliation":[{"name":"Shino-Test Corporation, 2-29-14 Oonodai Minami-ku, Sagamihara, Kanagawa 252-0331, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6983-907X","authenticated-orcid":false,"given":"Kenji","family":"Izuhara","sequence":"additional","affiliation":[{"name":"Division of Medical Biochemistry, Department of Biomolecular Sciences, Saga Medical School, 5-1-1, Nabeshima, Saga 849-8501, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kunio","family":"Dobashi","sequence":"additional","affiliation":[{"name":"Jobu Hospital for Respiratory Diseases, 586-1 Taguchi-machi, Maebashi, Gunma 371-0048, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takeshi","family":"Hisada","sequence":"additional","affiliation":[{"name":"Gunma University Graduate School of Health Sciences, 3-39-22 sho-wa machi Maebashi, Gunma 371-8514, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2019,5,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1136\/thorax.55.2.143","article-title":"Adult familial cryptogenic fibrosing alveolitis in the United Kingdom","volume":"55","author":"Marshall","year":"2000","journal-title":"Thorax"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1378\/chest.11-2812","article-title":"High-resolution CT scan findings in familial interstitial pneumonia do not conform to those of idiopathic interstitial pneumonia","volume":"142","author":"Lee","year":"2012","journal-title":"Chest"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1056\/NEJMoa1013660","article-title":"A common MUC5B promoter polymorphism and pulmonary fibrosis","volume":"364","author":"Seibold","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1056\/NEJMoa066157","article-title":"Telomerase mutations in families with idiopathic pulmonary fibrosis","volume":"356","author":"Armanios","year":"2007","journal-title":"N. Engl. J. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1900164","DOI":"10.1183\/13993003.00164-2019","article-title":"Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Lung Disease: The GOLD Science Committee Report 2019","volume":"53","author":"Singh","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Ohta, S., Okamoto, M., Fujimoto, K., Sakamoto, N., Takahashi, K., Yamamoto, H., Kushima, H., Ishii, H., Akasaka, K., and Ono, J. (2017). The usefulness of monomeric periostin as a biomarker for idiopathic pulmonary fibrosis. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0174547"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2034","DOI":"10.1378\/chest.127.6.2034","article-title":"Familial idiopathic pulmonary fibrosis: Clinical features and outcome","volume":"127","author":"Lee","year":"2005","journal-title":"Chest"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"449","DOI":"10.2169\/internalmedicine.53.1311","article-title":"Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia","volume":"53","author":"Furuhashi","year":"2014","journal-title":"Intern. Med. (Tokyo, Japan)"},{"key":"ref_9","first-page":"38","article-title":"Hermansky-Pudlak syndrome type 4 with interstitial pneumonia","volume":"9","author":"Sakata","year":"2013","journal-title":"Respir. Med. Case Rep."},{"key":"ref_10","first-page":"48","article-title":"Management of a patient with familial idiopathic pulmonary fibrosis","volume":"30","author":"Boing","year":"2013","journal-title":"Sarcoidosis Vasc. Diffuse Lung Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1164\/rccm.200211-1311OC","article-title":"Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis","volume":"168","author":"Collard","year":"2003","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.resinv.2016.09.003","article-title":"Prognostic value of serial serum KL-6 measurements in patients with idiopathic pulmonary fibrosis","volume":"55","author":"Wakamatsu","year":"2017","journal-title":"Respir. Investig."},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Sokai, A., Tanizawa, K., Handa, T., Kanatani, K., Kubo, T., Ikezoe, K., Nakatsuka, Y., Tokuda, S., Oga, T., and Hirai, T. (2017). Importance of serial changes in biomarkers in idiopathic pulmonary fibrosis. ERJ Open Res., 3.","DOI":"10.1183\/23120541.00019-2016"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Nakano, A., Ohkubo, H., Fukumitsu, K., Fukuda, S., Kanemitsu, Y., Takemura, M., Maeno, K., Ito, Y., Oguri, T., and Niimi, A. (2018). Remarkable Improvement in a Patient with Idiopathic Pulmonary Fibrosis after Treatment with Nintedanib. Intern. Med. (Tokyo, Japan).","DOI":"10.2169\/internalmedicine.1890-18"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.pupt.2017.05.007","article-title":"IL-13 and idiopathic pulmonary fibrosis: Possible links and new therapeutic strategies","volume":"45","author":"Passalacqua","year":"2017","journal-title":"Pulm. Pharmacol. Ther."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"L1046","DOI":"10.1152\/ajplung.00139.2012","article-title":"Periostin promotes fibrosis and predicts progression in patients with idiopathic pulmonary fibrosis","volume":"303","author":"Naik","year":"2012","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/j.resinv.2014.12.003","article-title":"Serum level of periostin can predict long-term outcome of idiopathic pulmonary fibrosis","volume":"53","author":"Tajiri","year":"2015","journal-title":"Respir. Investig."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1183\/09031936.00059810","article-title":"Periostin, a matrix protein, is a novel biomarker for idiopathic interstitial pneumonias","volume":"37","author":"Okamoto","year":"2011","journal-title":"Eur. Respir. J."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1165\/rcmb.2011-0115OC","article-title":"Periostin, a matricellular protein, plays a role in the induction of chemokines in pulmonary fibrosis","volume":"46","author":"Uchida","year":"2012","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1164\/ajrccm.159.2.9806064","article-title":"Lung epithelium-specific proteins: Characteristics and potential applications as markers","volume":"159","author":"Hermans","year":"1999","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/S2213-2600(14)70047-5","article-title":"Genetic determinants of pulmonary fibrosis: Evolving concepts","volume":"2","author":"Spagnolo","year":"2014","journal-title":"Lancet Respir. Med."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e587","DOI":"10.21037\/jtd.2018.06.96","article-title":"Accumulation of periostin in acute exacerbation of familial idiopathic pulmonary fibrosis","volume":"10","author":"Murata","year":"2018","journal-title":"J. Thorac. Dis."}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/55\/5\/161\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T12:53:15Z","timestamp":1760187195000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/55\/5\/161"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,5,17]]},"references-count":22,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2019,5]]}},"alternative-id":["medicina55050161"],"URL":"https:\/\/doi.org\/10.3390\/medicina55050161","relation":{},"ISSN":["1648-9144"],"issn-type":[{"type":"electronic","value":"1648-9144"}],"subject":[],"published":{"date-parts":[[2019,5,17]]}}}