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IgG4-related fibrosing mediastinitis: clinical presentation, treatment efficacy and comparison with IgG4-RD without fibrosing mediastinitis
P. Zhang1, X. Han2, J. Li3, Z. Liu4, H. Lu5, X. Luo6, C. Liu7, L. Peng8, Y. Fei9, X. Zeng10, W. Zhang11, X. Zeng12
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of Rheumatology, The Second Hospital of Dalian Medical University, Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
- Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China. zhangwen91@sina.com
- Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China. zxjpumch@126.com
CER13324
2020 Vol.38, N°6
PI 1206, PF 1214
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PMID: 32896252 [PubMed]
Received: 15/03/2020
Accepted : 20/07/2020
In Press: 04/09/2020
Published: 03/12/2020
Abstract
OBJECTIVES:
This study aimed to investigate the clinical characteristics and treatment efficacy of immunoglobulin G4 (IgG4)-related fibrosing mediastinitis (IgG4-RFM) and to compare IgG4-RFM patients with IgG4-related disease (IgG4-RD) patients without fibrosing mediastinitis (FM).
METHODS:
Twenty IgG4-RFM patients and 60 randomly matched IgG4-RD patients without FM from a prospective cohort at the Peking Union Medical College Hospital (PUMCH) were enrolled from 2011 to 2019. Patient demographic data, clinical characteristics, laboratory parameters and treatment efficacy were analysed.
RESULTS:
The prevalence of IgG4-RFM in our cohort was 2.8%. The average age was 51.7±14.8 years, and the patients were predominantly male (60.0%). Periaortic masses (75.0%) and paravertebral masses (35.0%) were the most common characteristic imaging findings of IgG4-RFM. Compared with male patients with IgG4-RFM, a lower percentage of female patients had abdominal aorta involvement (p=0.015). IgG4-RFM patients had a shorter disease duration; lower percentage of allergy history, submandibular gland involvement, and pancreas involvement; lower serum IgG4; higher erythrocyte sedimentation rate (ESR) and high-sensitivity C-reactive protein (hsCRP) levels; and a higher percentage of single organ involvement than patients without FM (p<0.001, p=0.008, p=0.033, p=0.001, p=0.027, p=0.007, p=0.004 and p=0.047, respectively). After treatment, 94.7% of patients achieved a mediastinal soft tissue reduction of >30%.
CONCLUSIONS:
IgG4-RFM is a distinct fibrotic subtype of IgG4-RD. Periaortic masses and paravertebral masses were the most common characteristic imaging findings of IgG4-RFM. Most IgG4-RFM patients respond well to glucocorticoid (GC) and immunosuppressant treatments.
