Article
Respiratory System
Tomoyuki Fujisawa, Yasuoki Horiike, Ryoko Egashira, Hiromitsu Sumikawa, Tae Iwasawa, Shoichiro Matsushita, Hiroaki Sugiura, Kensuke Kataoka, Mikiko Hashisako, Hideki Yasui, Hironao Hozumi, Masato Karayama, Yuzo Suzuki, Kazuki Furuhashi, Noriyuki Enomoto, Yutaro Nakamura, Naoki Inui, Takafumi Suda
Summary: The study found that radiological PPFE-like lesions are common in IIPs patients, especially in IPF and unclassifiable IIPs. Their presence is significantly associated with poor outcomes in patients.
RESPIRATORY RESEARCH
(2021)
Article
Biochemistry & Molecular Biology
Naofumi Sato, Yuji Iwanami, Kento Ebihara, Keiko Nakao, Midori Miyagi, Yasuhiko Nakamura, Kazuma Kishi, Sakae Homma, Satoru Ebihara
Summary: This study investigated the factors that cause a decrease in the percent of the predicted value of a 6-minute walk distance (%6MWD) in idiopathic pulmonary fibrosis (IPF) and idiopathic pleuroparenchymal fibroelastosis (IPPFE). The results suggest that leanness, shortness of breath, and severity of illness are the most important factors that determine exercise tolerance, regardless of disease entity in IPF and IPPFE.
Article
Medicine, General & Internal
Tsuneyuki Oda, Akimasa Sekine, Erina Tabata, Tae Iwasawa, Tamiko Takemura, Takashi Ogura
Summary: This study retrospectively reviewed the medical records of 132 idiopathic PPFE patients and 32 secondary PPFE patients, finding that the ILD pattern in secondary PPFE patients was different from idiopathic PPFE patients, but there were no significant differences in laboratory data, respiratory complications, and survival between the two groups. Presence of UIP pattern was associated with increased mortality in idiopathic PPFE patients, but not in secondary PPFE patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Medicine, General & Internal
Haruhiko Shiiya, Masaaki Sato
Summary: Pleuroparenchymal fibroelastosis (PPFE) is a new disease entity associated with interstitial pneumonia, characterized by fibrosis and elastosis predominantly in the upper lobe, leading to lung shrinkage and life-threatening complications. Patients usually have extremely low body mass index and flat chest. Lung transplantation is considered the only effective treatment for advanced PPFE patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Surgery
Haruhiko Shiiya, Jun Nakajima, Hiroshi Date, Toyofumi Fengshi Chen-Yoshikawa, Kiminobu Tanizawa, Tomohiro Handa, Takahiro Oto, Shinji Otani, Toshio Shiotani, Yoshinori Okada, Yasushi Matsuda, Takeshi Shiraishi, Toshihiko Moroga, Masato Minami, Soichiro Funaki, Masayuki Chida, Ichiro Yoshino, Go Hatachi, Yukari Uemura, Masaaki Sato
Summary: Despite extrapulmonary problems such as a flat chest, low BMI, and associated restrictive impairment persisting in patients with IPPFE, patient survival after LT for IPPFE or IPF was equivalent.
Article
Genetics & Heredity
Mouhamad Nasser, Salim Si-Mohamed, Segolene Turquier, Julie Traclet, Kais Ahmad, Francois Philit, Philippe Bonniaud, Lara Chalabreysse, Francoise Thivolet-Bejui, Vincent Cottin
Summary: The study suggests that nintedanib treatment may be associated with a slower decline in lung function in patients with PPFE, paving the way for prospective, controlled studies.
ORPHANET JOURNAL OF RARE DISEASES
(2021)
Article
Respiratory System
Masato Kono, Yutaro Nakamura, Yasunori Enomoto, Hideki Yasui, Hironao Hozumi, Masato Karayama, Yuzo Suzuki, Kazuki Furuhashi, Yoshihiro Miki, Dai Hashimoto, Tomoyuki Fujisawa, Noriyuki Enomoto, Naoki Inui, Yusuke Kaida, Koshi Yokomura, Naoki Koshimizu, Mikio Toyoshima, Shiro Imokawa, Takashi Yamada, Toshihiro Shirai, Hiroshi Hayakawa, Hidenori Nakamura, Takafumi Suda
Summary: A retrospective study on the complication of pneumothorax in patients with idiopathic PPFE revealed a high incidence rate of pneumothorax, predominantly asymptomatic and small in size, with some cases presenting recurrent pneumothorax. Male patients with PPFE pathological diagnosis, prior history of pneumothorax, and corticosteroid use were more likely to develop pneumothorax, which led to significantly poorer survival outcomes.
Article
Respiratory System
Jieun Kang, Woo Jung Seo, Eun Young Lee, Sung Hae Chang, Jooae Choe, Seokchan Hong, Jin Woo Song
Summary: This study investigated the prevalence and clinical implications of coexistent pleuroparenchymal fibroelastosis (PPFE) in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The results showed that PPFE was not rare in patients with RA-ILD and was significantly associated with an increased risk of pneumothorax and greater decline in lung function, although there was no significant association with mortality.
RESPIRATORY RESEARCH
(2022)
Review
Respiratory System
Vincent Cottin, Salim Si-Mohamed, Remi Diesler, Philippe Bonniaud, Claudia Valenzuela
Summary: This review summarizes the current understanding of pleuroparenchymal fibroelastosis (PPFE), a clinico-radiologic-pathologic interstitial lung disease characterized by fibrosis. PPFE can have different presentations depending on the underlying conditions. Idiopathic PPFE, cases associated with telomerase-related gene mutations, cases related to a history of chemotherapy, and cases combining PPFE with a pattern of usual interstitial pneumonia may have a particularly poor prognosis. Drug therapy for PPFE has not been extensively studied, but a small retrospective study suggests that nintedanib may slow disease progression. The association of PPFE features with poor prognosis in fibrosing interstitial lung diseases warrants further research on the management of PPFE.
CURRENT OPINION IN PULMONARY MEDICINE
(2022)
Article
Respiratory System
Yoshiaki Kinoshita, Takato Ikeda, Takuto Miyamura, Yusuke Ueda, Yuji Yoshida, Hisako Kushima, Masaki Fujita, Takashi Ogura, Kentaro Watanabe, Hiroshi Ishii
Summary: A prognostic prediction model for PPFE patients was developed using retrospective data, which identified lower forced vital capacity, history of pneumothorax, lower lobe interstitial lung disease, and higher serum KL-6 levels as factors significantly associated with poor prognosis. Patients were divided into three prognostic stages based on a total score, with the new model showing better predictive performance compared to the traditional GAP model.
RESPIRATORY RESEARCH
(2021)
Article
Respiratory System
Akira Yamagata, Machiko Arita, Hiromasa Tachibana, Fumiaki Tokioka, Chikatoshi Sugimoto, Hiromitsu Sumikawa, Tomonori Tanaka, Hideki Yasui, Tomoyuki Fujisawa, Yutaro Nakamura, Takafumi Suda, Tadashi Ishida
Summary: BAL lymphocytosis is associated with favorable outcomes in patients treated with anti-inflammatory drugs, indicating its potential as a predictive biomarker for identifying IIP patients likely to benefit from anti-inflammatory therapy. However, BAL lymphocytosis does not have prognostic value in patients managed without anti-inflammatory therapy.
RESPIRATORY RESEARCH
(2021)
Article
Medicine, General & Internal
Yutaro Nakamura, Kazutaka Mori, Yasunori Enomoto, Masato Kono, Hiromitsu Sumikawa, Takeshi Johkoh, Thomas Colby, Hideki Yasui, Hironao Hozumi, Masato Karayama, Yuzo Suzuki, Tomoyuki Fujisawa, Noriyuki Enomoto, Naoki Inui, Yusuke Kaida, Koshi Yokomura, Naoki Koshimizu, Mikio Toyoshima, Shiro Imokawa, Takashi Yamada, Toshihiro Shirai, Hidenori Nakamura, Hiroshi Hayakawa, Takafumi Suda
Summary: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinct interstitial pneumonia characterized by unique morphological features, and affected patients can be categorized into four different clinical phenotypes based on cluster analysis, which may help predict survival outcomes. Patients with PPFE in cluster 3, which includes elderly male smokers with a coexisting IP-like pattern, have significantly worse survival outcomes compared to those in clusters 1, 2, and 4.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Cell Biology
Naoya Ikegami, Naoki Nakajima, Akihiko Yoshizawa, Tomohiro Handa, Toyofumi Chen-Yoshikawa, Takeshi Kubo, Kiminobu Tanizawa, Akihiro Ohsumi, Yoshito Yamada, Masatsugu Hamaji, Daisuke Nakajima, Yojiro Yutaka, Satona Tanaka, Kizuku Watanabe, Yoshinari Nakatsuka, Yuko Murase, Tomoko Nakanishi, Takafumi Niwamoto, Kazuo Chin, Hiroshi Date, Toyohiro Hirai
Summary: This study evaluated the pathological findings associated with radiological PPFE-like lesions and the clinical and morphological features of patients with pathological PPFE. The results showed that pathological PPFE was related to previous pneumothorax, volume loss in the upper lobes, and a flattened chest. Patients with idiopathic disease and those with secondary disease with pathological PPFE had similar clinical, radiological, and pathological findings. However, fibroblastic foci were more common in idiopathic cases and alveolar septal thickening with elastosis or fibrosis were more common in secondary cases. The post-transplantation survival of patients with pathological PPFE was similar to those with idiopathic pulmonary fibrosis (IPF).
Article
Respiratory System
Atsuki Fukada, Yuzo Suzuki, Kazutaka Mori, Masato Kono, Hirotsugu Hasegawa, Dai Hashimoto, Koshi Yokomura, Shiro Imokawa, Yuko Tanaka, Yusuke Inoue, Hironao Hozumi, Masato Karayama, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Yutaro Nakamura, Naoki Inui, Yoshihisa Fujino, Hidenori Nakamura, Takafumi Suda
Summary: The study found that lower standardised upper-lobe volume in iPPFE patients was associated with shorter survival rates. A composite scoring model including age, sex, and standardised upper-lobe volume better predicted the risk of death compared to traditional models in iPPFE patients.
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Medicine, General & Internal
Miriana D'Alessandro, Francesca Bellisai, Laura Bergantini, Paolo Cameli, Roberto D'Alessandro, Maria Antonietta Mazzei, Francesco Gentili, Edoardo Conticini, Enrico Selvi, Bruno Frediani, Marco Matucci-Cerinic, Elena Bargagli
Summary: KL-6 levels were increased in SSc-ILD patients, with PPFE patients showing significantly higher KL-6 variation rates over 6 years of follow-up compared to non-PPFE patients. In PPFE patients, KL-6 concentrations were inversely correlated with FEV1 and FVC percentages.
EUROPEAN JOURNAL OF CLINICAL INVESTIGATION
(2021)