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: Yunyun Fei, Juhong Shi, Wei Lin, Yu Chen, Ruie Feng, Qingjun Wu, Xin Gao, Wenbing Xu, Wen Zhang, Xuan Zhang, Yan Zhao, Xiaofeng Zeng, Fengchun Zhang
• Journal: Medicine (Medicine (Baltimore)) Vol. 94 Issue 50 Pg. e2150 (Dec 2015) ISSN: 1536-5964 [Electronic] United States
• PMID: 26683924 (Publication Type: Journal Article, Observational Study, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulin G
• Immunosuppressive Agents
• C-Reactive Protein

Topics

• Adult
• Aged
• C-Reactive Protein
• China
• Female
• Humans
• Immunoglobulin G (immunology)
• Immunosuppressive Agents (therapeutic use)
• Lung Diseases (diagnostic imaging, drug therapy, immunology)
• Lymphatic Diseases (diagnostic imaging, drug therapy, immunology)
• Male
• Middle Aged
• Positron-Emission Tomography
• Prospective Studies
• Thorax (immunology)
• Tomography, X-Ray Computed