SAT0230 MACROPHAGE ACTIVATION SYNDROME IN SLE AND SYSTEMIC ONSET JIA: SIMILAR OR DISSIMILAR

2020 
Background: The risk of developing lymphomas in Sjogren’s syndrome (SS) is more than 10-fold higher than in general population. Available publications describe a number of indicators considered as predictors for SS development. Objectives: To describe clinical and laboratory characteristics of patients with SS and MALT lymphoma and compare them with idem parameters in the control group. To identify factors associated with development of lymphoma. Methods: The study included 87 SS patients with MALT lymphoma. In all cases lymphoma involved the parotid salivary glands. In all cases MALT lymphoma was diagnosed simultaneously with SS. At the time of inclusion non of the patients was on immunosuppressive therapy. Fifty five SS patients without lymphoproliferative pathology composed the control group. All cases were newly diagnosed and treatment-naive. SS was diagnosed based on the ACR-EULAR criteria. The histologic and immunohistochemical diagnosis of lymphoma was performed with B-cell clonality determination in salivary gland tissue. The following clinical and laboratory parameters were monitored in both groups: rates of stage 3 xerostomia ( Pearson’s x2 criterion (analysis of contingency tables) was used for statistical analysis. The differences were considered statistically significant when p values were Results: In the study group 84 patients were females, their mean age at the onset of lymphoma was 53±10 years. The disease duration was 7 years (3-12) before the diagnosis was established. All patients in the control group were females, the mean age at diagnosis was 50.2 ±13 years. Patients’ age at diagnosis did not differ significantly between the groups. Enlarged salivary or lacrimal glands were found in all SS-MALT patients and in 18% of patients in the control group. The rates of such systemic manifestations as polyneuropathy, kidney and joint damage was low and did not differ between groups. Increased levels of anti-Ro antibodies was documented in the majority of patients in both groups (87 % and 81%, p=0.4), while aLa antibodies were significantly more common in MALT lymphoma patients (60% and 40.3%, p=0.045). Similar rates of increased IgG and IgM levels were found in both groups, while increased IgA levels were 6-fold more common in the lymphoma group (p Conclusion: Therefore, universally recognized predictors of lymphoma development, such as cryoglobulinemia and hypocomplementemia did not show reliable association with lymphoma. In analyzed cohort development of MALT lymphoma was statistically significantly associated with recurrent parotitis in past medical history, presence of lymphadenopathy at diagnostic examination, increased levels of anti-La antibodies and IgA, and hypergammaglobulinemia. Probably we should more actively treat patients with these clinical and laboratory features in order to prevent the development of lymphoma in them. Disclosure of Interests: None declared
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