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

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

Respiratory Medicine Case Reports (Jan 2018)

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

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

Affiliations

Masato Kono: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan; Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Japan; Corresponding author. 2-12-12 Sumiyoshi, Nakaku, Hamamatsu, Shizuoka, 430-8558, Japan.
Yutaro Nakamura: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Japan
Yoshiyuki Oyama: Department of Respiratory Medicine, Sizuoka Saiseikai General Hospital, Japan
Go Saito: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Yu Koyanagi: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Koichi Miyashita: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Akari Tsutsumi: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Yasunori Enomoto: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Japan
Takeshi Kobayashi: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Yoshihiro Miki: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Dai Hashimoto: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan
Noriyuki Enomoto: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Japan
Thomas V. Colby: Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA
Takafumi Suda: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Japan
Hidenori Nakamura: Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan

Journal volume & issue: Vol. 25
pp. 257 – 260

Abstract

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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. Keywords: Combined pulmonary fibrosis and emphysema (CPFE), IgG4-related disease (IgG4-RD), Usual interstitial pneumonia (UIP)

Published in Respiratory Medicine Case Reports

ISSN: 2213-0071 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the respiratory system
Website: http://www.journals.elsevier.com/respiratory-medicine-case-reports/

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