Survival and acute exacerbation for patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias: 5-year follow-up analysis of a prospective multi-institutional patient registry

被引:10
|
作者
Tsubouchi, Kazuya [1 ]
Hamada, Naoki [2 ]
Tokunaga, Shoji [3 ]
Ichiki, Katsuyuki [4 ]
Takata, Shohei [5 ]
Ishii, Hiroshi [6 ]
Kitasato, Yasuhiko [7 ]
Okamoto, Masaki [8 ]
Kawakami, Satoru [9 ]
Yatera, Kazuhiro [10 ]
Kawasaki, Masayuki [11 ]
Fujita, Masaki [2 ]
Yoshida, Makoto [12 ]
Maeyama, Takashige [13 ]
Harada, Taishi [14 ]
Wataya, Hiroshi [15 ]
Torii, Ryo [16 ]
Komori, Masashi [17 ]
Mizuta, Yuichi [18 ]
Tobino, Kazunori [19 ]
Harada, Eiji [20 ]
Yabuuchi, Hidetake [21 ]
Nakanishi, Yoichi [22 ]
Okamoto, Isamu [1 ]
机构
[1] Kyushu Univ, Grad Sch Med Sci, Dept Resp Med, Fukuoka, Japan
[2] Fukuoka Univ, Sch Med, Dept Resp Med, Fukuoka, Japan
[3] Kyushu Univ Hosp, Med Informat Ctr, Fukuoka, Japan
[4] Kirigaoka Tsuda Hosp, Kitakyushu, Japan
[5] Natl Hosp Org, Fukuoka Higashi Med Ctr, Dept Resp Dis, Fukuoka, Japan
[6] Fukuoka Univ, Chikushi Hosp, Dept Resp Med, Chikushino, Japan
[7] Japan Community Hlth Care Org Kurume Gen Hosp, Dept Resp Med, Kurume, Japan
[8] Kurume Univ, Sch Med, Dept Internal Med, Div Respirol Neurol & Rheumatol, Kurume, Japan
[9] Kyushu Rosai Hosp, Div Resp Med, Kitakyushu, Japan
[10] Univ Occupat & Environm Hlth Japan, Dept Resp Med, Kitakyushu, Japan
[11] Natl Hosp Org Omuta Natl Hosp, Dept Resp Med, Omuta, Japan
[12] Natl Hosp Org, Fukuoka Natl Hosp, Dept Resp Dis, Fukuoka, Japan
[13] Hamanomachi Hosp, Dept Resp Med, Fukuoka, Japan
[14] Japan Community Hlth Care Org Kyushu Hosp, Dept Resp Med, Kitakyushu, Japan
[15] Saiseikai Fukuoka Gen Hosp, Dept Resp Med, Fukuoka, Japan
[16] Univ Occupat & Environm Hlth, Wakamatsu Hosp, Dept Resp Med, Kitakyushu, Japan
[17] Steel Mem Yawata Hosp, Dept Resp Med, Kitakyushu, Japan
[18] St Marys Hosp, Dept Resp Med, Kurume, Japan
[19] Aso Iizuka Hosp, Div Resp Med, Iizuka, Japan
[20] Kitakyushu Municipal Med Ctr, Dept Resp Med, Kitakyushu, Japan
[21] Kyushu Univ, Grad Sch Med Sci, Dept Hlth Sci, Fukuoka, Japan
[22] Kitakyushu City Hosp Org, Kitakyushu, Japan
关键词
Clinical Epidemiology; Interstitial Fibrosis; CLASSIFICATION; DIAGNOSIS; UPDATE;
D O I
10.1136/bmjresp-2023-001864
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
ObjectiveFew prospective cohort studies with relatively large numbers of patients with non-idiopathic pulmonary fibrosis (non-IPF) of idiopathic interstitial pneumonia (IIP) have been described. We aimed to assess disease progression and cause of death for patients with non-IPF IIPs or IPF under real-life conditions.MethodsData were analysed for a prospective multi-institutional cohort of 528 IIP patients enrolled in Japan between September 2013 and April 2016. Diagnosis of IPF versus non-IPF IIPs was based on central multidisciplinary discussion, and follow-up surveillance was performed for up to 5 years after patient registration. Survival and acute exacerbation (AE) were assessed.ResultsIPF was the most common diagnosis (58.0%), followed by unclassifiable IIPs (35.8%) and others (6.2%). The 5-year survival rate for non-IPF IIP and IPF groups was 72.8% and 53.7%, respectively, with chronic respiratory failure being the primary cause of death in both groups. AE was the second most common cause of death for both non-IPF IIP (24.1%) and IPF (23.5%) patients. The cumulative incidence of AE did not differ significantly between the two groups (p=0.36), with a 1-year incidence rate of 7.4% and 9.0% in non-IPF IIP and IPF patients, respectively. We found that 30.2% and 39.4% of non-IPF IIP and IPF patients, respectively, who experienced AE died within 3 months after an AE event, whereas 55.8% and 66.7% of such patients, respectively, died within 5 years after registration.ConclusionCloser monitoring of disease progression and palliative care interventions after AE are important for non-IPF IIP patients as well as for IPF patients.
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页数:11
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