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

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.

• Authors: Masato Kono, Yutaro Nakamura, Yoshiyuki Oyama, Go Saito, Yu Koyanagi, Koichi Miyashita, Akari Tsutsumi, Yasunori Enomoto, Takeshi Kobayashi, Yoshihiro Miki, Dai Hashimoto, Noriyuki Enomoto, Thomas V Colby, Takafumi Suda, Hidenori Nakamura
• Journal: Respiratory medicine case reports (Respir Med Case Rep) Vol. 25 Pg. 257-260 ( 2018) ISSN: 2213-0071 [Print] England
• PMID: 30302309 (Publication Type: Case Reports)