AB0562 Extraglandular manifestations in patients with primary sjÖgren syndrome in a tertiary hospital in madrid
Authors: C.M. Pijoan Moratalla, C. Sobrino-Grande, C. De la Puente-Bujidos
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Primary Sjögren syndrome (pSS) is a chronic autoinmune disorder characterised by generalised dryness. In a variable percentage of cases (up to 50%) patients can present extraglandular disease, which frequently determines the prognosis. <h3>Objectives</h3> To determine the frequency of both glandular and extraglandular disease in patients with SSp seen in a tertiary hospital in Madrid and to compare them with the frequency observed in the large cohorts (SER and EULAR). <h3>Methods</h3> A descriptive, observational cross-sectional study was conducted. We included patients with diagnosis of pSS according to the ACR/EULAR Classification criteria (2016) attended in our Rheumatology Unit from 2012 to 2017. A database was created, including clinical and epidemiological data and a descriptive analysis was carried out comparing the results with those obtained in the Sjögren-SER proyect and EULAR group. <h3>Results</h3> 106 patients with pSS were included. 92.5% were female (98), with a mean age at diagnosis of 45 years (range: 32–58). Frequency of exocrine gland disease is shown in table 1. Dry eye was the most frequent symptom (91%), with nearly half of them presenting ocular complications. 69.8% complained of dry mouth and 18.9% associated complications such as dysphagia and oral candidiasis. 16 patients (15%) suffered from recurrent parotiditis and 13 (12.3%) from salivary gland enlargement. Glandular disease also included xerosis (25%), dyspareunia (11.3%), upper respiratory tract dryness (12.3%) and atrophic chronic gastritis (14%). Frequency of extraglandular disease is shown in table 2. Chronic fatigue was the most frequent symptom, similar to the observed in both cohorts (50.9%), followed by arthralgia which was less frequent than in the Spanish cohort (40.6% vs 34.5%). 35 patients suffered from inflammatory arthritis and 3 cases associated fibromyalgia, less than the expected (2.8% vs 14.6% and 22%–33%). Sixteen patients suffered from interstitial lung disease, this being higher than the observed in both cohorts (15.1% vs 6.64% and 5%). Fewer patients suffered from depression compared with the EULAR group (24.5% vs 40%). Both peripheral neuropathy and renal disease were diagnosed in a percentage of patients similar to the expected (11.3% vs 8.92% y 1.88% vs 1.83% respectively). 7 patients had autoimmune thyroid disease. Finally, 5 patients (4.7%) developed lymphoma, 3 of them being MALT lymphoma of the parotid gland which is higher than the observed in the Spanish cohort but it is within the EULAR range. The frequency of Raynaud's phenomenon, cutaneous vasculitis, lymphadenopathy, splenomegaly and pericarditis were similar to those observed both in the Spanish national group and EULAR cohort. <h3>Conclusions</h3> Extraglandular disease in SS, although less frequent than sicca symptoms, have a special relevance when it comes to patient management. In our study the frequency of both glandular and extraglandular disease was similar to the observed in Sjögren-SER and EULAR groups although there were some differences. Fibromyalgia was less frequent in our group, whereas interstitial lung disease and peripheral neuropathy were more prevalent. <h3>Disclosure of Interest</h3> None declared