AB0431 Anti-centromere antibody positive sjögren’s syndrome: a distinct clinical subgroup?

Abstract

Objectives To investigate whether patients with Sjögren’s syndrome(SS) can be distinguished based on the positivity of anti-centromere antibody (ACA) and if so, whether the subgroups differ in their clinical and laboratory features. Methods Eighty two consecutive patients with SS were included, with a median age of 43 years (range 15 - 71). All patients had minor salivary gland biopsy and sociodemographic, glandular, and extraglandular manifestations, and laboratory findings including autoantibodies, complement, and immunoglobulin levels, were analyzed. EULAR SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. Results The prevalence of ACA among SS patients was 13.4%. ACA positive SS patients had higher age at diagnosis (p=0.023), shorter disease duration (p=0.014), higher prevalence of Raynaud’s phenomenon (p<0.001), sclerodactyly (p=0.017) and Hashimoto’s thyroiditis (p=0.005) compared to ACA negative patients. The prevalence of anti-SSA/Ro and anti-SSB/La antibodies were significantly lower in ACA positive patients than ACA negative patients (p=0.002, both). Although SSDDI were not different between the two groups, ESSDAI was significantly lower in ACA positive patients than ACA negative patients (p=0.008). None of patients originally having ACA didn’t evolve to full blown systemic sclerosis. Conclusions These findings suggest that patients with SS who have ACA differ from classic SS patients in several clinical and laboratory parameters. ACA should be considered as one of the pathogenically relevant autoantibodies for SS. Disclosure of Interest None Declared

CLINICAL AND LABORATORY FEATURES OF ANTICENTROMERE ANTIBODY-POSITIVE SJö GREN’S SYNDROME

“…[24] удобны для использования в клинических исследованиях и в значительно меньшей степени применимы для дифференциальной диагностики в реальной клинической практике. Гипотетический пациент с АЦА-позитивной БШ и/или ПБЦ, феноменом Рейно и капилляроскопическими изменениями склеродермического типа, которые описаны при различных состояниях [30,31], а также ИПЛ, которое при лимитированной форме ССД встречается значительно реже, чем при диффузной [4], и может выявляться при БШ [33], удовлетворяет критериям ССД ACR 2013 г. [24], даже не имея типичных для лимитированной формы ССД изменений кожи (отек кистей, склеродактилия, амимия лица, телеангиэктазии).…”