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[The clinical characteristics of 346 patients with IgG4-related disease].
Zhonghua Nei Ke Za Zhi 2017; 56(9):644-649ZN

Abstract

Objective:

To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China.

Methods:

IgG4-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 IgG4-RD responder index (IgG4-RD RI) was 13.21±5.70. History of allergy was found in 172 (49.7%) patients. As to the laboratory tests, elevated serum IgG4 levels were confirmed in 285 (94.1%) patients, which was positively correlated with IgG4-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:

IgG4-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 IgG4-RD.

Authors+Show Affiliations

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableDepartment of Rheumatology, the Ministry of Education Key Laboratory of Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100032, China.

Pub Type(s)

Journal Article

Language

chi

PubMed ID

28870031

Citation

Zhang, P P., et al. "[The Clinical Characteristics of 346 Patients With IgG4-related Disease]." Zhonghua Nei Ke Za Zhi, vol. 56, no. 9, 2017, pp. 644-649.
Zhang PP, Zhao JZ, Wang M, et al. [The clinical characteristics of 346 patients with IgG4-related disease]. Zhonghua Nei Ke Za Zhi. 2017;56(9):644-649.
Zhang, P. P., Zhao, J. Z., Wang, M., Feng, R. E., Liu, X. W., Lai, X. M., ... Zhang, W. (2017). [The clinical characteristics of 346 patients with IgG4-related disease]. Zhonghua Nei Ke Za Zhi, 56(9), pp. 644-649. doi:10.3760/cma.j.issn.0578-1426.2017.09.005.
Zhang PP, et al. [The Clinical Characteristics of 346 Patients With IgG4-related Disease]. Zhonghua Nei Ke Za Zhi. 2017 Sep 1;56(9):644-649. PubMed PMID: 28870031.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [The clinical characteristics of 346 patients with IgG4-related disease]. AU - Zhang,P P, AU - Zhao,J Z, AU - Wang,M, AU - Feng,R E, AU - Liu,X W, AU - Lai,X M, AU - Li,X J, AU - Zeng,J G, AU - Shi,H J, AU - Zhu,H D, AU - Xue,W, AU - Zhang,H, AU - Chen,Y Y, AU - Fei,L Y, AU - Peng,X F, AU - Zeng,F C, AU - Zhang,Y M, AU - Zhang,W, PY - 2017/9/6/entrez PY - 2017/9/6/pubmed PY - 2019/2/5/medline KW - Clinical characteristics KW - Cohort study KW - IgG(4) related disease SP - 644 EP - 649 JF - Zhonghua nei ke za zhi JO - Zhonghua Nei Ke Za Zhi VL - 56 IS - 9 N2 - Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-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 IgG4-RD responder index (IgG4-RD RI) was 13.21±5.70. History of allergy was found in 172 (49.7%) patients. As to the laboratory tests, elevated serum IgG4 levels were confirmed in 285 (94.1%) patients, which was positively correlated with IgG4-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: IgG4-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 IgG4-RD. SN - 0578-1426 UR - https://www.unboundmedicine.com/medline/citation/28870031/[The_clinical_characteristics_of_346_patients_with_IgG4_related_disease]_ L2 - http://journal.yiigle.com/LinkIn.do?linkin_type=pubmed&issn=0578-1426&year=2017&vol=56&issue=9&fpage=644 DB - PRIME DP - Unbound Medicine ER -