Poster Presentations
Lee Kar Hoo, Lee, Wendy Lee Wan Hui, Lee, Benjamin Sachdev Manjit Singh, Sachdev, Cheong Yaw Kiet, Cheong, Ahmad Tirmizi, Jobli, Lee Tung Hiong, Lee, Ling Guo Rey, Ling, Sharifah Aishah, Wan, Teh Cheng Lay, Teh
Abstract
Background: IgG4- related disease (IgG4- RD) is a novel entity of disease \nfirst recognised in 2003. It has wide spectrum of clinical manifestations, \nvirtually affecting any organ. It’s epidemiology remain \npoorly described and treatment guideline still lacking. \nMethods: We describe 7 IgG4RD cases diagnosed and treated in 2 \nhospitals with rheumatologist in Sarawak from 2015 till 2020. \nResults: 4 males and 3 females were reported with age during diagnosis \nranging from 34 to 74 years old. 4 patients had tumefactive \nlesions (lacrimal gland and salivary gland swelling), 2 had obstructive \nsymptoms (obstructive uropathy due to retroperitoneal fibrosis, \nobstructive jaundice secondary to autoimmune pancreatitis) \nand 1 had renal failure and proteinuria as presenting symptoms. \nTime taken from onset of symptoms to diagnosis range from 8 \nmonths to 23 years. In all cases, histopathological findings were \nthe prompt towards diagnosis of IgG4- RD. All patients fulfilled the \nACR- EULAR classification criteria whereas only 3 met the 2010 \nJCR comprehensive diagnostic criteria, due to lack of IgG4 level in \nothers. All patients showed rapid response to steroid therapy, complete \nresolution of salivary gland swelling were seen in 3 months, \nstent removal in those with obstructive symptoms were achieved in \n6 months whereas resolution of proteinuria was seen in 2 months. \nAll remained in remission currently with or without treatment. \nInitial dose of prednisolone used ranged from 0.2 to 0.6mg/kg/day, \nall with good effect. \nConclusion: IgG4- RD is indolent but often highly destructive. It is \ntreatable and if diagnosed early, damage is potentially reversible. In \nterms of diagnosis, we feel that the ACR- EULAR classification criteria \nis more practical compared to the JCR criteria in places where \nresources are limited and laboratory testing of serum IgG4 level is \nnot available. Good awareness and high index of suspicion among \nclinicians, radiologists and pathologists are thus essential in timely \ndiagnosis and prompt treatment of IgG4- RD.