title: Microscopic polyangiitis preceded by combined pulmonary fibrosis and emphysema.
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.
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan.
authors.forename: Kyoko
authors.initials: K
authors.lastname: Gocho
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan.
authors.forename: Keishi
authors.initials: K
authors.lastname: Sugino
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan.
authors.forename: Keita
authors.initials: K
authors.lastname: Sato
authors.affiliation: Department of Pathology, Toho University Omori Medical Center, Japan.
authors.forename: Chikako
authors.initials: C
authors.lastname: Hasegawa
authors.affiliation: Department of Pathology, Japan Labor Health and Welfare Organization Kanto Rosai Hospital, Japan.
authors.forename: Toshimasa
authors.initials: T
authors.lastname: Uekusa
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1, Omori-nishi, Ota-ku, Tokyo 143-8541, Japan.
authors.forename: Sakae
authors.initials: S
authors.lastname: Homma
keywords: Combined pulmonary fibrosis; Emphysema; Interstitial lung disease; Microscopic polyangiitis