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Abstract

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Article in Japanese

Case Report

A case of IgG4-related lung disease associated with desquamative interstitial pneumonia-like features

Shinichi Iwamotoa  Yuji Ohtsukib  Hibiki Kandaa  Akihisa Sutania  Takashige Kurakia  Takeshi Isobea 

aDepartment of Internal Medicine, Division of Clinical Oncology and Respiratory Medicine, Shimane University Faculty of Medicine
bDepartment of Pathology, Matsuyama Civic Hospital

ABSTRACT

A 50-year-old male complaining of a dry cough was admitted to our hospital. Chest X-rays showed reticulonodular shadows in the bilateral lower lung fields, and video-assisted thoracic surgery was performed. We first suspected this case to have desquamative interstitial pneumonia (DIP) coexisting with nonspecific interstitial pneumonia (NSIP). The patient had glaucoma; therefore we were hesitant to start steroid therapy and urged him to stop smoking. He was unable to, however, and thereafter showed a progression of imaging features and an increase in KL-6 level. High levels of serum IgG4 and IgG4-positive plasma cell infiltration of the lungs were observed. As a result, the patient was started on prednisolone after consulting an ophthalmologist. The imaging features and the KL-6 level showed improvements. The prednisolone was thus tapered gradually in an outpatient setting, and the patient has been healthy without relapse. We ultimately diagnosed this case to have IgG4-related lung disease associated with DIP-like features in a heavy smoker.

KEYWORDS

IgG4-related lung disease  Desquamative interstitial pneumonia  Heavy smoker 

Received 12 Aug 2011 / Accepted 7 Mar 2012

AJRS, 1(6): 470-475, 2012

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