Microscopic polyangiitis preceded by combined pulmonary fibrosis and emphysema

• Main Authors: Kyoko Gocho, Keishi Sugino, Keita Sato, Chikako Hasegawa, Toshimasa Uekusa, Sakae Homma
• Format: Article
• Language: English
• Published: Elsevier 2015-01-01
• Series: Respiratory Medicine Case Reports
• Subjects: Microscopic polyangiitis; Interstitial lung disease; Emphysema; Combined pulmonary fibrosis
• Online Access: http://www.sciencedirect.com/science/article/pii/S2213007115000088

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.