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

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.

Acknowledgements

This work was supported by National Natural Science Foundation (81172858), Beijing Natural Science Foundation (7132206) and Chinese Medical Assoication Foundation (12040680368).