• Ailing Liu, Qianwen Zhang, Bo Liu, Ning Xu, and Aijun Li.
• Department of Pulmonary Diseases, Weihai Municipal Hospital, Weihai, Shandong, P.R. China.
• Medicine (Baltimore). 2017 Dec 1; 96 (50): e9211e9211.

RationaleImmunoglobulin (Ig) G4-related disease (IgG4-RD) is a rare and chronic progressive autoimmune disease. It is a novel clinical entity characterized by elevated serum IgG4 concentration and tissue infiltration by IgG4 plasma cells. IgG4-related lung disease (IgG4-RLD) has been described as interstitial pneumonia and inflammatory pseudotumor, with various abnormal radiographic patterns. We report a case of IgG4-related lung disease with bilateral diffuse infiltration.Patient ConcernsA 65-year-old woman was admitted to our hospital because of cough, sputum, and fever. Chest computed tomography (CT) revealed multifocal areas of consolidations, nodules, and ground glass opacities in both lungs. She still had fever after anti-infective therapy.DiagnosesBronchial bronchoscopy and percutaneous lung biopsy were performed, resulted in a pathological diagnosis of IgG4-RLD.InterventionsThe patient was started on oral prednisolone 30 mg/day for 28 days, and then was gradually tapered.OutcomesAfter one week treatment, the temperature was stable, CT showed significant improvement in the areas of consolidations and nodules.LessonsIt is a typical case of IgG4-RLD. This case indicated that suggestive evidence, radiological appearances, serum tests, pathological characteristics, and classic therapy IgG4-RLD. It is a rare disease that needs our more attention in future.Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

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