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Updated: Apr 6, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Microscopic polyangiitis preceded by combined pulmonary fibrosis and emphysema
Kyoko Gocho1, Keishi Sugino1, Keita Sato1
1Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan.
Abstract:
A 73-year-old male metalworker was admitted to our hospital with a 3-year history of progressive dry cough. Chest high-resolution computed tomography revealed emphysematous changes and reticular lesions, which is referred to as combined pulmonary fibrosis and emphysema (CPFE). Surgical lung biopsy specimens revealed unclassified interstitial pneumonia, including a nonspecific interstitial pneumonia pattern and usual interstitial pneumonia pattern. Two years after his first admission he developed rapid progressive renal dysfunction with an elevated level of myeloperoxidase-antineutrophil cytoplasmic antibody (428 EU). A renal biopsy specimen revealed interstitial nephritis and glomerulonephritis. Consequently, microscopic polyangiitis preceded by CPFE was diagnosed. Despite transient exacerbation of renal involvement, his general condition remained mostly stable during a 2-year period of corticosteroid treatment. He ultimately died from severe pneumococcal pneumonia associated with acute lung injury.
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