title: Intrathoracic Involvements of Immunoglobulin G4-Related Sclerosing Disease.
abstract: To investigate clinical and radiological features of IgG4-related disease (IgG4-RD) patients with intrathoracic involvement. A prospective cohort study was performed and IgG4-RD patients were enrolled from January 2011 to March 2015 in Peking Union Medical College Hospital, in which the clinical and radiological characteristics of IgG4-RD patients with intrathoracic involvement were summarized. Out of total 248 cases with IgG4-RD, 87 cases had intrathoracic lesions, including 58 male cases and 29 female cases, with average age of 54.19 ± 13.80 years. Hilar and mediastinal lymphadenopathy were the most common manifestations of IgG4-related intrathoracic disease, accounting for 52.9% (46/87). Other imaging findings of pulmonary disease included: solid nodular (25.3%), round-shaped ground-glass opacities (9.2%), alveolar-interstitial type (20.7%), bronchovascular type (23.0%), pleural effusion (4.6%), and pleural nodules or thickening (16.1%). Only 27 patients presented with respiratory symptoms, including cough, breathless, chest pain, and asthma. Compared with patients without intrathoracic disease, IgG4-related intrathoracic disease had higher IgG4 and C-reactive protein level, and higher incidence of allergy, fever, and multi-organ involvement. Most of lung interstitial disease, mediastinal mass, and bronchial thickening were sensitive to corticosteroid and immunosuppressant therapy, while 36.3% (8/22) of solitary nodular lesions were unresponsive to treatment. Eight patients were on no treatment, with 5 cases remained stable, 2 patients improved spontaneously, and 1 patient was lost follow-up. Intrathoracic lesions are not rare in patients with IgG4-RD, involving bronchial thickening, nodules, ground glass opacity, pleural thickening/effusion, lymphadenopathy, etc. Efficacy of corticosteroid and immunosuppressant therapy were noted in most of patients with lung interstitial disease, mediastinal mass, and bronchial thickening.
authors.affiliation: From the Departments of Rheumatology (YF, WL, YC, QW, WZ, XZ, YZ, XZ, FZ), Respiratory (JS, WX), Pathology (RF), and Radiology (XG), Peking Union Medical College Hospital, Beijing, China.
authors.forename: Yunyun
authors.initials: Y
authors.lastname: Fei
authors.forename: Juhong
authors.initials: J
authors.lastname: Shi
authors.forename: Wei
authors.initials: W
authors.lastname: Lin
authors.forename: Yu
authors.initials: Y
authors.lastname: Chen
authors.forename: Ruie
authors.initials: R
authors.lastname: Feng
authors.forename: Qingjun
authors.initials: Q
authors.lastname: Wu
authors.forename: Xin
authors.initials: X
authors.lastname: Gao
authors.forename: Wenbing
authors.initials: W
authors.lastname: Xu
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhang
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhao
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Zeng
authors.forename: Fengchun
authors.initials: F
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000368:Aged; D002097:C-Reactive Protein; D002681:China; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology*; D008206:Lymphatic Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D011446:Prospective Studies; D013909:Thorax / Q000276:immunology*; D014057:Tomography, X-Ray Computed
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D002097:C-Reactive Protein