The clinical characteristics of 346 patients with IgG4-related disease / 中华内科杂志
Chinese Journal of Internal Medicine
; (12): 644-649, 2017.
Article
in Zh
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| ID: wpr-611066
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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 followedup 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.
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Zh
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Chinese Journal of Internal Medicine
Year:
2017
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Article