Background Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic immune-mediated disease unifying what were previously considered to be unrelated individual organ disorders. The diagnosis and treatment of this characteristic fibroinflammatory condition continues to evolve, but generally remains an under-recognised disease. Local data outside Japanese and Caucasian populations is lacking and few studies have examined factors to predict disease severity or disease prognostication.
Objectives We conducted this study to review the clinical features, treatment practices and factors associated with more extensive disease involvement in Hong Kong.
Methods We retrospectively evaluated all patients with IgG4-RD over the past 13 years in our centre 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 One hundred and four patients (55 from our centre and 49 from literature review) were identified. Patients were predominantly older men (mean age 61.9 years, male:female ratio 3:1). Hepatobiliary and pancreatic (40.4%), salivary gland (33.6%), lymph nodes (29.8%) and eye (19.2%) were the most common systems involved. Lymphadenopathy was associated with glucocorticoid use (OR=2.65, p=0.034). Over 90% of patients had a serum greater than 135 mg/dl and a IgG4/total IgG ratio greater than 8%. Pre-treatment serum IgG4 levels correlated with the number of organ systems involved (β=0.347, p=0.004), and specifically with salivary gland involvement (mean 1109 mg/dl vs. 599 mg/dl, p=0.012).
Conclusions We identified pre-treatment serum IgG4-RD to be associated with multi-system disease, especially with salivary gland involvement, highlighting the potential for its use in disease prognostication and monitoring. The reason for this particular correlation remains uncertain but highlights the importance of screening for salivary gland involvement in all IgG4-RD patients, especially in the presence of higher serum IgG4 levels. We also describe the clinical features and treatment modalities of the largest cohort of IgG4-RD in Hong Kong thus far. Increased physician awareness and multidisciplinary efforts are required for optimal management of this masquerading disease. Future studies, especially focusing on treatment strategies within the contexts of different epidemiology and patient characteristics, are urgently needed.
Disclosure of Interest None declared