IgG4-related disease presenting with combined pulmonary fibrosis and emphysema (CPFE). (2018)

• Record Type: Journal Article
• Authors: Kono, Masato; Nakamura, Yutaro; Oyama, Yoshiyuki; Saito, Go; Koyanagi, Yu; Miyashita, Koichi; Tsutsumi, Akari; Enomoto, Yasunori; Kobayashi, Takeshi; Miki, Yoshihiro; Hashimoto, Dai; Enomoto, Noriyuki; Colby, Thomas V.; Suda, Takafumi; Nakamura, Hidenori
• Is Part Of: Respiratory medicine case reports. Volume 25(2018)
• Page Start: 257
• Page End: 260
• Publication Date: 2018
• Subjects: Combined pulmonary fibrosis and emphysema (CPFE) -- IgG4-related disease (IgG4-RD) -- Usual interstitial pneumonia (UIP); AIP autoimmune pancreatitis -- CPFE Combined pulmonary fibrosis with emphysema -- CT computed tomography -- CTD connective tissue disease -- DIP desquamative interstitial pneumonia -- DLco diffusion capacity for carbon monoxide -- FEV1.0 forced vital capacity in 1 second -- FVC forced vital capacity -- GGO ground-glass opacities -- HE hematoxylin-eosin -- HRCT high-resolution CT -- IIP idiopathic interstitial pneumonia -- IgG4-RD IgG4-related disease -- IgG4-RLD IgG4-related lung diseases -- IL interleukin -- ILD interstitial lung disease -- IPF idiopathic pulmonary fibrosis -- NSIP nonspecific interstitial pneumonia -- PFT pulmonary function tests -- RA rheumatoid arthritis -- TGF tissue growth factor -- UIP usual interstitial pneumonia; Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2018.09.017
• Languages: English
• ISSNs: 2213-0071

Abstract

Abstract: A 64-year-old man was admitted to our hospital with an abnormal chest shadow. The patient was a current-smoker and had a past illness of autoimmune pancreatitis with a high serum level of IgG4, 348 mg/dL. Chest CT showed upper-lobe emphysema, and lower-lobe reticulation with honeycombing, suggestive of combined pulmonary fibrosis with emphysema (CPFE). Surgical lung biopsy was revealed a usual interstitial pneumonia pattern with marked infiltration of IgG4-positive plasma cells. The patient was diagnosed with IgG4 related disease (IgG4-RD) presenting with CPFE. Pulmonary manifestation was improved by corticosteroid therapy. IgG4-RD may be an underlying condition in patient with CPFE.