AB1244 PROGNOSTIC FACTORS IN IGG4-RELATED DISEASE: A LONG-TERM MONOCENTRIC CHINESE COHORT STUDY

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Background:Through initial response to treatment with GC, the patients with IgG4-related disease (IgG4-RD) exhibited high relapse rate after reduction or withdrawal of GC treatment, indicated the unsatisfactory prognosis for IgG4-RD. It is of clinical significance to develop new informative risk factors for refractory and relapsed disease.Objectives:To evaluate the prognosis of IgG4-RD and identify predictive factors for treatment resistance and disease relapse in a Chinese cohort.Methods:102 patients newly diagnosed with IgG4-RD were followed for 6-111 months. Clinical data were compared between patients whose disease went into remission and those who suffered refractory or relapsed disease. Predictive factors for refractory and relapsed disease were calculated by univariate analysis.Results:Among the 78 patients who received medical treatment with regular follow-up, 55 (59.8%) patients sustained clinical remission, and 23 (25%) patients suffered refractory or relapsed disease. The mortality and incidence of malignancy were both 4.35% during follow-up. Serum TNF-α ≥ 13 pg/ml, sIL-2R ≥ 1010 pg/ml, TC < 3.55 mmol/L, LDL < 2.0 mmol/L, IgG > 20.2 g/L, GC withdrawal, and treatment without immunosuppressor (IM) during the maintenance period (OR 3.23) were predictive factors for refractory and relapsed IgG4-RD. The combination of GC and IM treatment was protective (OR 0.338) against refractory and relapsed IgG4-RD.Conclusion:Serum TNF-α, sIL-2R, LDL, TC, IgG, GC withdrawal, and treatment without IM during the maintenance period were predictive factors for refractory and relapsed IgG4-RD. Treatment with GC and IM may protect against refractory and relapsed IgG4-RD.