Respiratory investigation · 2025
Characteristics, clinical findings, and treatment of idiopathic pulmonary fibrosis in Japanese patients registered with a medical subsidy system for intractable diseases.
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Nishikiori Hirotaka, Nakamura Koshi, Takei Reoto, Kondoh Yasuhiro, Suda Takafumi, Chiba Hirofumi
Аннотация
BACKGROUND: The Japanese government operates a medical subsidy system for intractable diseases, including idiopathic pulmonary fibrosis (IPF). Registering with this system requires filling out a clinical survey form, which encompasses multiple survey items regarding the patient's disease and functional status. In this study, we retrospectively analyzed the forms of new applicants with IPF in 2019 to evaluate the clinical and functional characteristics.
METHODS: The following patient data were collected: sex, age, smoking status, history of surgical lung biopsy, disease severity (using Japanese severity classification [JSC]), serum biomarkers, findings on chest high-resolution computed tomography (HRCT), functional status, symptoms, and treatment.
RESULTS: Of the 4796 patients reviewed (76.1 % males; mean age = 73.4 ± 8.1 years), 23.6 % had a mild disease (JSC stages I-II) and 76.4 % had a severe disease (stages III-IV). The HRCT of most patients revealed honeycombing, traction bronchiectasis and/or bronchiolectasis, reticular shadows, and subpleural shadows. The positivity rates for elevated serum levels of Krebs von Lungen-6 (KL-6) and surfactant protein-D (SP-D) were 92.6 % and 89.3 %, respectively. As the severity increased, the biomarker positivity rate increased. Approximately half of the patients with milder diseases experienced transportation challenges, and 30 % complained of pain and/or discomfort and anxiety and/or depression.
CONCLUSIONS: In approximately 90 % of patients, serum KL-6 and SP-D levels increased and the positive rate increased as the disease severity increased. Even patients with mild diseases experience challenges in transportation as well as pain, discomfort, anxiety, or depression.
METHODS: The following patient data were collected: sex, age, smoking status, history of surgical lung biopsy, disease severity (using Japanese severity classification [JSC]), serum biomarkers, findings on chest high-resolution computed tomography (HRCT), functional status, symptoms, and treatment.
RESULTS: Of the 4796 patients reviewed (76.1 % males; mean age = 73.4 ± 8.1 years), 23.6 % had a mild disease (JSC stages I-II) and 76.4 % had a severe disease (stages III-IV). The HRCT of most patients revealed honeycombing, traction bronchiectasis and/or bronchiolectasis, reticular shadows, and subpleural shadows. The positivity rates for elevated serum levels of Krebs von Lungen-6 (KL-6) and surfactant protein-D (SP-D) were 92.6 % and 89.3 %, respectively. As the severity increased, the biomarker positivity rate increased. Approximately half of the patients with milder diseases experienced transportation challenges, and 30 % complained of pain and/or discomfort and anxiety and/or depression.
CONCLUSIONS: In approximately 90 % of patients, serum KL-6 and SP-D levels increased and the positive rate increased as the disease severity increased. Even patients with mild diseases experience challenges in transportation as well as pain, discomfort, anxiety, or depression.