Dados do Trabalho

Title

HEMATOLOGICAL MANIFESTATIONS IN SJOGREN’S SYNDROME: ANALYSIS OF CLINICAL AND LABORATORIAL PARAMETERS OF THE DISEASE

Background

Sjögren's Syndrome (SS) is a systemic autoimmune inflammatory disease marked by increased humoral activity of lymphocytes. Hematologic abnormalities and development of lymphoproliferative disorders can occur in this condition. The objective was to evaluate the frequency of hematological manifestations in patients with SS, and to assess their association with clinical ad laboratory findings of the disease.

Materials and methods

Retrospective observational study that included 174 patients with SS, in the period between January 2000 and December 2017. Data were analyzed by SPSS 18.0 using chi-square (x2) for qualitative variables and Student t test for quantitative variables, with significance at p ≤ 0.05. The measure of association used was Prevalence Ratio (PR) with Confidence Interval (95% CI).

Results

Hematologic abnormalities were common, the most prevalent was hypergammaglobulinemia (57.9%), followed by anemia (55.7%), predominantly normocytic-normochromic, and leucopenia (46.6%). Among the clinical and laboratory parameters of the disease, the presence of lymphoma, anti-SSA/Ro and anti-SSB/La positivity, and high β2-microglobulin were associated with hematological abnormalities, especially cytopenias. B-cell non-Hodgkin's lymphoma was rare and occurred in just 1.1% of patients.

Conclusions

Hematological disorders are common in patients with SS, especially cytopenia and hypergammaglobulinemia, and are associated with elevated serum levels of β2-microglobulin and anti-SSA/Ro and anti-SSB/La positivity. We suggest that in the diagnostic evaluation of patients presenting with cytopenia and hypergammaglobulinemia of unknown cause, consider SS as a possible diagnosis and request for the initial evaluation the antinuclear antibodies (ANA) and rheumatoid factor (RF) test as well as anti-SSA/Ro and anti-SSB/La autoantibodies.