THU0543 A retrospective overview of the treatment modality, outcome and relapse risk of IGG4 related disease in hong kong: a dataset of 108 patients from four regional hospitals
Authors: CP Tang, KL Lee, KW Lee, WL Ng, MC Wan, KY Yuen
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Currently most consensus suggests treating IgG4 related disease (IgG4RD) with prednisolone 0.6mg/kg/day tapering over 6 months while some experts prefer continuation of steroid up to 3 years (1–3). <h3>Objectives</h3> In this retrospective study, the treatment modality, response and relapse risk of IgG4RD patients over the past ten years from four regional hospitals in Hong Kong were analyzed. <h3>Methods</h3> Four regional hospitals participated with study period from 1/1/2006 to 30/6/2016. Patients were diagnosed IgG4RD according to the Japanese Comprehensive Diagnostic Criteria for IgG4RD. Treatment response at 6 months was recorded as complete, partial or non-remission based on the patient9s and physician9s perspective. Relapse was defined as disease progression either clinically or radiologically. <i>Statistical analysis:</i> The association between treatment response and steroid dosage was analyzed by multivariate logistic regression. Risk factors for relapse were analyzed by multivariate cox regression and the hazard ratio was reported. <h3>Results</h3> 37 cases had surgical excision and disease recurred in 5 cases (relapse rate =13.5%). 87 patients (81%) received steroid treatment. The mean starting prednisolone was 33.5mg daily, with a mean duration of 95.2 weeks. At 6 months, 5 patients (6%) had no response, 34 patients (41%) had partial remission and 44 patients (53%) had complete remission. 29 patients also received other immunosuppressants, including azathioprine (n=25), mycophenolate mofetil (MMF) (n=6), cyclosporine A (n=4), mercaptopurine (n=2) and rituximab (n=1). The overall response rate for azathioprine and MMF was 64% and 67% respectively. In the final multiple logistic regression, an initial prednisolone 30mg daily or above was associated with a higher complete remission at 6 months (OR=3.4, p=0.079) and the effect was more seen in patients with salivary and orbital involvement (OR=6.8, p=0.10) 18 patients relapsed after steroid was stopped and 6 patients relapsed while on prednisolone 2.5–7.5mg daily. The one year, two year and three year relapse rate were 13.3%, 24.1% and 26.5% respectively. In the final multivariate cox regression, the presence of maintenance steroid was associated with a lower relapse risk (Hazard ratio=0.121, p=0.000) while serum IgG4 level above twice upper limit of normal (Hazard ratio=5.283, p=0.029) and hepatobiliary involvement (Hazard ratio=2.164, p=0.095) were associated with a higher relapse risk. <h3>Conclusions</h3> Most patients (94%) had good response to steroid. Patients with hepatobiliary involvement and serum IgG4 level above twice upper limit of normal were at higher relapse risk and a low dose, maintenance prednisolone for longer period is recommended. <h3>References</h3> Kleger A, et al. IgG4-related autoimmune diseases: Polymorphous presentation complicates diagnosis and treatment. Deutsches Arzteblatt international. 2015;112(8):128–35. Ghazale A, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134(3):706–15. Kamisawa T, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1504–7. <h3>Disclosure of Interest</h3> None declared