H Chen1, W Lin, Q Wang, Q Wu, L Wang, Y Fei, W Zheng, G Fei, P Li, Y Z Li, W Zhang, Y Zhao, X Zeng, F Zhang. 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.
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.
Authors: Zachary S Wallace; Vikram Deshpande; Hamid Mattoo; Vinay S Mahajan; Maria Kulikova; Shiv Pillai; John H Stone Journal: Arthritis Rheumatol Date: 2015-09 Impact factor: 10.995
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