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

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...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6175766/
https://www.ncbi.nlm.nih.gov/pubmed/30302309
http://dx.doi.org/10.1016/j.rmcr.2018.09.017
Descripción
Sumario: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.

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