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[The clinical characteristics of 346 patients with IgG 4 -related disease].
- Source :
-
Zhonghua nei ke za zhi [Zhonghua Nei Ke Za Zhi] 2017 Sep 01; Vol. 56 (9), pp. 644-649. - Publication Year :
- 2017
-
Abstract
- Objective: To analyze the clinical characteristics of IgG <subscript>4</subscript> -related disease (IgG <subscript>4</subscript> -RD)so as to improve the understanding of IgG <subscript>4</subscript> -RD in China. Methods: IgG <subscript>4</subscript> -RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66.5%) and 116 females (33.5%). The mean age of disease onset was (53.8±14.2) years old. The mostly common involved organs were lymph nodes (56.4%) and submandibular glands (52.6%). Other affected organs and manifestations included: swelling of the lacrimal glands (46.5%), autoimmune pancreatitis (38.4%), pulmonary involvement (28.0%), sclerosing cholangitis (25.4%), naso-sinusitis (23.4%), parotid gland swelling (21.7%), retroperitoneal fibrosis (19.9%), large arteries involvement (9.5%), kidney involvement (obstructive nephropathy caused by retroperitoneal fibrosis was excluded) (6.9%), skin lesions (6.4%). Rare features consisted of thyroid glands, pituitary glands, gastrointestinal tract, pachymeningitis, pericardium, sclerosing mediastinitis and orchitis. The majority of patients had multi-organ involvement, such as 74.3% patients with 3 and more, 18.2% and 7.5% patients with 2 and single organ involvement respectively. The average IgG <subscript>4</subscript> -RD responder index (IgG <subscript>4</subscript> -RD RI) was 13.21±5.70. History of allergy was found in 172 (49.7%) patients. As to the laboratory tests, elevated serum IgG <subscript>4</subscript> levels were confirmed in 285 (94.1%) patients, which was positively correlated with IgG <subscript>4</subscript> -RD RI. There were 33.5% patients receiving monotherapy of glucocorticoid, 52.6% treated with glucocorticoids combined with immunosuppressive agents, 4.9% patients with immunosuppressant only, and 9.0% patients with mild disease not receiving medication. The majority (336, 97.1%) patients improved the above regimens. Conclusion: IgG <subscript>4</subscript> -RD is a systemic fibro-inflammatory disease with multiple organ involvement. The mostly common involved organs include lymph node, submandibular glands, and pancreas. Glucocorticoids and immunosuppressive agents were effective for IgG <subscript>4</subscript> -RD.
- Subjects :
- Adult
Aged
China epidemiology
Female
Glucocorticoids therapeutic use
Humans
Immunoglobulin G4-Related Disease complications
Immunoglobulin G4-Related Disease drug therapy
Immunosuppressive Agents therapeutic use
Male
Middle Aged
Treatment Outcome
Asian People
Immunoglobulin G immunology
Immunoglobulin G4-Related Disease immunology
Pancreatitis pathology
Subjects
Details
- Language :
- Chinese
- ISSN :
- 0578-1426
- Volume :
- 56
- Issue :
- 9
- Database :
- MEDLINE
- Journal :
- Zhonghua nei ke za zhi
- Publication Type :
- Academic Journal
- Accession number :
- 28870031
- Full Text :
- https://doi.org/10.3760/cma.j.issn.0578-1426.2017.09.005