Fatal cases of pulmonary alveolar proteinosis: a nationwide surveillance in Japan
Y. Inoue (Osaka, Japan), E. Yamaguchi (AIchi, Japan), Y. Setoguchi (Tokyo, Japan), T. Ichiwata (Tokyo, Japan), M. Ebina (Miyagi, Japan), K. Cho (Hokkando, Japan), R. Tazawa (Niigata, Japan), H. Ishii (Tokyo, Japan), T. Kasai (Osaka, Japan), M. Akira (Osaka, Japan), T. Arai (Osaka, Japan), K. Uchida (Tokyo, Japan), H. Kida (Osaka, Japan), K. Morimoto (Nagasaki, Japan), M. Kitaichi (Wakayama, Japan), C. Sugimoto (Osaka, Japan), K. Nakata (Niigata, Japan), K. Katayama (Osaka, Japan), N. Takeuchi (Osaka, Japan), A. Matsumuro (Osaka, Japan)
Source: International Congress 2018 – Progress in rare interstitial lung diseases
Session: Progress in rare interstitial lung diseases
Session type: Oral Presentation
Number: 3783
Disease area: Interstitial lung diseases
Abstract [Background] Pulmonary alveolar proteinosis (PAP) is a chronic rare lung disease characterized by the intra-alveolar accumulation of surfactant lipids and proteins. 10 year survival of autoimmune PAP was 89.7% (n=117, WASOG, 2017) in our institute, however, we recently reported that fibrosis develops in approximately 20% of patients with PAP in their long clinical courses, and the prognosis of them was poor (AJR. 207:544, 2016). To clarify the characteristics of fatal cases of PAP, we retrospectively analyzed the diseases cases from our Japanese cohort database. [Subject and methods] We collected 579 cases with PAP from 95 Japanese institutes, and 81 cases (14.0%) were dead. Within 81 cases, we collected the detail data from 34 cases. [Results] 60% was autoimmune/idiopathic PAP, 34% was secondary PAP. Median age of 34 fatal cases (65% male) was 59 (range 28-82) years old. 59% was smokers (Current and former), and 21% had dust exposure. At the diagnosis, 21% of patients had interstitial pneumonia/pulmonary fibrosis (IP/PF), 21% had collagen vascular diseases, 18% had incfectious disease, 3% had malignant diseases. However during the followup periods, 9% had IP/PF, 24% had incfectious disease, 24% had malignant diseases. Causa of the death was 26% was malignantn diseases, 18% in fections, 12% was PAP, 6% IP/PF, 6% was cerebro vascular diseases. [Conclusions] Patients with PAP had more comorbidities than previously reported. Managemnents of them would be cricial for better prognosis. [Grant] This study was supported by the grant from AMED #17930161 (YI)
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Y. Inoue (Osaka, Japan), E. Yamaguchi (AIchi, Japan), Y. Setoguchi (Tokyo, Japan), T. Ichiwata (Tokyo, Japan), M. Ebina (Miyagi, Japan), K. Cho (Hokkando, Japan), R. Tazawa (Niigata, Japan), H. Ishii (Tokyo, Japan), T. Kasai (Osaka, Japan), M. Akira (Osaka, Japan), T. Arai (Osaka, Japan), K. Uchida (Tokyo, Japan), H. Kida (Osaka, Japan), K. Morimoto (Nagasaki, Japan), M. Kitaichi (Wakayama, Japan), C. Sugimoto (Osaka, Japan), K. Nakata (Niigata, Japan), K. Katayama (Osaka, Japan), N. Takeuchi (Osaka, Japan), A. Matsumuro (Osaka, Japan). Fatal cases of pulmonary alveolar proteinosis: a nationwide surveillance in Japan. 3783
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