Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by inflammation and fibrosis of nearly any organ in the body. Since its discovery nearly two decades ago, our understanding of its pathophysiology and clinical manifestations has grown substantially. However, lack of familiarity with IgG4-RD results in patients often being undiagnosed and under treated. The characteristic fibrotic masses of IgG4-RD may be mistaken for cancerous tumours, leading to extensive surgery. It can mimic other autoimmune diseases.
IgG4-RD can occur in nearly any organ system and most commonly affects tear ducts, salivary glands, and gastrointestinal and abdominal organs. Some manifestations of IgG4-RD put patients at high risk for serious complications, including pancreatic enlargement, retroperitoneal fibrosis, and aortitis. Cardiovascular involvement is rare; myocardial infarction is rarely reported. The current incidence of IgG4-RD is 1 to 5 people per 100,000.
Three cases will be presented. If it is recognized early, and treatment is initiated quickly, IgG4-RD responds well to treatment and excellent clinical outcomes can be affected.
The American College of Rheumatology/European League Against Rheumatism Classification Criteria provide a framework for approaching the diagnosis of IgG4-RD even though they were not intended for diagnostic purposes.
Our Speaker
Dr. Anita Lim Yee Nah
Rheumatologist
Dr. Anita Lim is a rheumatologist. A medical graduate from the University of Glasgow in 1992, Dr. Lim spent her seasoned career in Brunei and the United Kingdom where she completed her rheumatology specialist training at the Addenbrooke's Hospital, Cambridge and Norfolk and Norwich University Hospital; and subsequently many years at the National University Hospital (NUH), ultimately attaining the ranks of Senior Consultant Rheumatologist, NUH Vice Chairman Medical Board (Human Resource and Education) and serving with the NUH Hospital Ethics Committee.
She is Adjunct Assistant Professor at the Yong Loo Lin School of Medicine (NUS) at the Centre for BioMedical Ethics, NUS.
Cedar Rheumatology was set up in 2023 and is operating now at FPH.
