P H Li1, K L Ko2, C Tk Ho1, L L Lau3, R Ky Tsang4, T T Cheung5, W K Leung2, C S Lau1. 1. Division of Rheumatology & Clinical Immunology, Department of Medicine, Queen Mary Hospital, Pokfulam, Hong Kong. 2. Division of Gastroenterology & Hepatology, Department of Medicine, Queen Mary Hospital, Pokfulam, Hong Kong. 3. Department of Ear, Nose & Throat, Queen Mary Hospital, Pokfulam, Hong Kong. 4. Division of Otorhinolaryngology - Head and Neck Surgery, Department of Surgery, Queen Mary Hospital, Pokfulam, Hong Kong. 5. Division of Hepatobiliary & Pancreatic Surgery and Liver Transplantation, Department of Surgery, Queen Mary Hospital, Pokfulam, Hong Kong.
Abstract
INTRODUCTION: Immunoglobulin G4-related disease remains an under-recognised and evolving disease. Local data are sparse and previous publications have been limited to individual case reports or case series only. We conducted this study to review the clinical features, treatment practices, and factors associated with multisystem involvement in Hong Kong. We described the clinical features and treatment modalities of the largest cohort of immunoglobulin G4-related disease in our locality thus far. METHODS: We retrospectively evaluated all patients with immunoglobulin G4-related disease between January 2003 and December 2015 in Queen Mary Hospital and combined this with patient data extracted from previous local publications. We analysed the clinical features, treatment practices, and factors associated with the number of organ systems involved. RESULTS: A total of 104 patients (55 from Queen Mary Hospital and 49 from literature review) were identified. Patients were predominantly older men (mean [standard deviation] age, 61.9 [12.7] years; male-to-female ratio=3:1) and 94.4% had elevated pre-treatment serum immunoglobulin G4 levels. Hepatobiliary and pancreatic system (40.4%), salivary gland (33.7%), lymph node (29.8%), and eye (19.2%) were the most common organ systems involved. Lymphadenopathy was associated with glucocorticoid use (odds ratio=2.65; 95% confidence interval, 1.08-6.54; P=0.034). Pre-treatment serum immunoglobulin G4 levels correlated with the number of organ systems involved (β=0.347; P=0.004) and, specifically, more associated with patients having salivary gland involvement than those without (mean, 1109 mg/dL vs 599 mg/dL; P=0.012). CONCLUSION: We identified pre-treatment serum immunoglobulin G4 to be associated with multisystem disease, especially with salivary gland involvement, highlighting its potential for disease prognostication and monitoring. Increased physician awareness and multidisciplinary efforts are required for early diagnosis and optimal management of this masquerading disease.
INTRODUCTION: Immunoglobulin G4-related disease remains an under-recognised and evolving disease. Local data are sparse and previous publications have been limited to individual case reports or case series only. We conducted this study to review the clinical features, treatment practices, and factors associated with multisystem involvement in Hong Kong. We described the clinical features and treatment modalities of the largest cohort of immunoglobulin G4-related disease in our locality thus far. METHODS: We retrospectively evaluated all patients with immunoglobulin G4-related disease between January 2003 and December 2015 in Queen Mary Hospital and combined this with patient data extracted from previous local publications. We analysed the clinical features, treatment practices, and factors associated with the number of organ systems involved. RESULTS: A total of 104 patients (55 from Queen Mary Hospital and 49 from literature review) were identified. Patients were predominantly older men (mean [standard deviation] age, 61.9 [12.7] years; male-to-female ratio=3:1) and 94.4% had elevated pre-treatment serum immunoglobulin G4 levels. Hepatobiliary and pancreatic system (40.4%), salivary gland (33.7%), lymph node (29.8%), and eye (19.2%) were the most common organ systems involved. Lymphadenopathy was associated with glucocorticoid use (odds ratio=2.65; 95% confidence interval, 1.08-6.54; P=0.034). Pre-treatment serum immunoglobulin G4 levels correlated with the number of organ systems involved (β=0.347; P=0.004) and, specifically, more associated with patients having salivary gland involvement than those without (mean, 1109 mg/dL vs 599 mg/dL; P=0.012). CONCLUSION: We identified pre-treatment serum immunoglobulin G4 to be associated with multisystem disease, especially with salivary gland involvement, highlighting its potential for disease prognostication and monitoring. Increased physician awareness and multidisciplinary efforts are required for early diagnosis and optimal management of this masquerading disease.
Authors: Ji Zongfei; Ma Lingying; Zhang Lijuan; Sun Ying; Chen Rongyi; Liu Dongmei; Kong Xiufang; Dai Xiaomin; Ma Lili; Chen Huiyong; Jiang Lindi Journal: Clin Rheumatol Date: 2020-11-05 Impact factor: 2.980
Authors: Ji Zongfei; Chen Rongyi; Cui Xiaomeng; Ma Lili; Ma Lingying; Kong Xiufang; Dai Xiaomin; Zhang Zhuojun; Chen Huiyong; Sun Ying; Jiang Lindi Journal: Front Immunol Date: 2020-07-08 Impact factor: 7.561