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Scand J Rheumatol. 2014;43(1):70-4. doi: 10.3109/03009742.2013.822094. Epub 2013 Oct 18.

IgG4-related disease in a Chinese cohort: a prospective study.

Author information

1
Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education , Beijing , China.

Abstract

OBJECTIVES:

To report the clinical, laboratory, and histological characteristics in a cohort of 28 Chinese patients with immunoglobulin G4-related disease (IgG4-RD).

METHOD:

The patients were admitted to, or were out-patients in, Peking Union Medical College Hospital (PUMCH) between January 2011 and May 2012 according to the criteria for IgG4-RD. Clinical presentations, imaging studies, serum Ig subclass assays, and histological examinations were performed in all patients.

RESULTS:

The 28 patients (male-to-female ratio 1.8:1) enrolled in this study had a mean age at onset of disease of 51.5 years and the duration of symptoms before diagnosis was 20.4 months; 57% of the patients presented with a history of allergic disease. The most common symptoms were salivary and lacrimal gland swelling, and abdominal pain. Most patients (93%) presented with multiple organ involvement. Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were observed in 16 (57%) and seven (25%) patients, respectively. The mean serum IgG4 level was 1142.3 mg/dL (range 149-3870 mg/dL). Eosinophilia was noted in 11 (39%) patients. IgE levels were elevated in all of eight patients examined. Lymphocyte and plasma cell infiltration and IgG4-positive plasma cell infiltration, together with tissue fibrosis, were the predominant histological findings. Most patients (93%) were given prednisone (0.5-0.8 mg/kg/day). Immunosuppressive agents were administered in 19 (68%) patients. Patients were followed for a mean of 6.3 months; efficacy was noted in more than 90% of cases.

CONCLUSIONS:

IgG4-RD is a chronic, systemic, multiorgan inflammatory disorder. IgG4-RD patients generally response well to glucocorticoids but the treatment should be individualized.

PMID:
24134471
DOI:
10.3109/03009742.2013.822094
[Indexed for MEDLINE]

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