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Rheumatology (Oxford). 2015 Nov;54(11):1982-90. doi: 10.1093/rheumatology/kev203. Epub 2015 Jun 22.

Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients.

Author information

1
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.
2
General Internal Medicine Department, Peking Union Medical College Hospital, Beijing.
3
Department of Rheumatology, The People's Hospital of Baoan, Shen Zhen.
4
Department of Rheumatology, The People's Hospital of Linfen, Shan Xi, China and.
5
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, zhangwen91@sina.com.
6
Private residence, Charlottesville, VA, USA.

Abstract

OBJECTIVE:

To characterize the clinical features of IgG4-related disease (IgG4-RD) in China.

METHODS:

A prospective cohort study of IgG4-RD was carried out in Peking Union Medical College Hospital between 2011 and 2013. Patients with newly diagnosed IgG4-RD were enrolled.

RESULTS:

A total of 118 patients with IgG4-RD were enrolled, including 82 males and 36 females, aged 53.1 (s.d. 13.6) years. The most common symptom at onset was lacrimal gland swelling (38/32.2%). A range of organs were involved: 77 patients (65.3%) had lymphadenopathy, 76 (64.4%) had sialadenitis, 60 (50.8%) had dacryoadenitis, 45 (38.1%) had autoimmune pancreatitis, 32 (27.1%) had pulmonary involvement, 31 (26.3%) had periaortitis/retroperitoneal fibrosis, 29 (35.4% of male patients) had prostatitis and 29 (24.6%) had renal involvement. In addition, there were 21 (17.8%) cases of sclerosing cholangitis, 15 (12.7%) of sinusitis and 10 (8.5%) of inflammatory pseudotumour. Uncommon manifestations included mediastinal fibrosis, skin involvement, sclerosing thyroiditis, hypophysitis, orchitis and colitis. Multiple organ involvement was observed in 93 patients, whereas only 4.2% had only a single organ involved. A history of allergy was reported in 73 (61.9%) patients. The serum IgG4 level was elevated in 97.5% and was correlated with the number of organs involved. Most patients were treated with glucocorticoids alone or in combination with immunosuppressive drugs, and the majority usually improved within 3 months.

CONCLUSION:

IgG4-RD is a systemic inflammatory and sclerosing disease. Parotid and lacrimal involvement (formerly called Mikulicz's disease), lymphadenopathy and pancreatitis are the most common manifestations. Patients with IgG4-RD showed favourable responses to treatment with glucocorticoids and immunosuppressive agents.

KEYWORDS:

IgG4; IgG4-related disease; Mikulicz’s disease; autoimmune pancreatitis; prospective cohort

PMID:
26106212
DOI:
10.1093/rheumatology/kev203
[Indexed for MEDLINE]

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