title: Respiratory involvement in IgG4-related Mikulicz's disease.
abstract: 'Immunoglobulin G4 (IgG4)-related disease' is a new clinical concept of multi-organ diseases, with Mikulicz's disease (MD) being a clinical phenotype of IgG4-related disease. To clarify the clinical characteristics of respiratory involvement associated with IgG4-related MD, we retrospectively assessed 25 patients with MD, 11 (44%) of whom had allergic symptoms, and 7 (28%) of whom complained of respiratory problems. Thirteen patients (52%) presented with pulmonary and/or mediastinal lesions (P-MD) on chest computed tomography (CT), and 11 (44%) had lesions limited to the lacrimal and/or salivary glands (L-MD). Mean serum total protein, IgG, and IgG4 concentrations were significantly higher and CH50 was significantly lower in the P-MD than in the L-MD group. Immune complex was present only in the P-MD group. Chest CT images showed bronchial wall thickening, consolidation, nodule(s), interlobular thickening, ground glass opacity, pleural thickening/effusion, and mediastinal lymphadenopathy. Five of seven patients who underwent histological examination of the lungs had abundant IgG4-positive plasma cell infiltrates (IgG4/IgG-positive plasma cells >40%), but the other two did not. These findings suggest that respiratory lesions are not rare in patients with IgG4-related MD, and that they present with various manifestations. IgG4-related MD should be differentiated from similar diseases, such as sarcoidosis, bronchial asthma, Sjögren's syndrome, and malignant lymphoma.
authors.affiliation: Department of Internal Medicine (1), University of Toyama, 2630 Sugitani, Toyama, Toyama 930-0194, Japan. shoko-tym@umin.ac.jp
authors.forename: Shoko
authors.initials: S
authors.lastname: Matsui
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Taki
authors.forename: Koichiro
authors.initials: K
authors.lastname: Shinoda
authors.forename: Kensuke
authors.initials: K
authors.lastname: Suzuki
authors.forename: Ryuji
authors.initials: R
authors.lastname: Hayashi
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Tobe
authors.forename: Yoshiharu
authors.initials: Y
authors.lastname: Tokimitsu
authors.forename: Masayuki
authors.initials: M
authors.lastname: Ishida
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Fushiki
authors.forename: Hikaru
authors.initials: H
authors.lastname: Seto
authors.forename: Junya
authors.initials: J
authors.lastname: Fukuoka
authors.forename: Shin
authors.initials: S
authors.lastname: Ishizawa
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001249:Asthma / Q000175:diagnosis; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007765:Lacrimal Apparatus / Q000276:immunology / Q000473:pathology; D008168:Lung / Q000276:immunology / Q000473:pathology*; D008171:Lung Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D008223:Lymphoma / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D009928:Organ Specificity; D010195:Pancreatitis / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D013902:Radiography, Thoracic; D012189:Retrospective Studies; D012469:Salivary Glands / Q000276:immunology / Q000473:pathology; D012507:Sarcoidosis / Q000175:diagnosis; D012859:Sjogren's Syndrome / Q000175:diagnosis; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G