The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded. Demographics and pSS characteristics were recorded. The EGM were defined by EULAR-SS disease activity index (ESSDAI). Patients were divided into two groups according to their use or not of HCQ therapy. We evaluated the use of HCQ and its relationship to EGM. HCQ therapy was defined as the continuous use of the drug for at least 3 months. A descriptive analysis of demographics and pSS characteristics was performed. We compared the incidence of EGM between groups defined by HCQ therapy using chi test or Fisher's exact test. A total of 221 patients were included (97.3% women), mean age, 55.7 years (SD 14). Mean age at diagnosis, 48.8 years (SD 15); median disease duration, 60 months (IQR 35-84). One hundred and seventy patients (77%) received HCQ. About half of the patients had at least one EGM during the course of the disease, 20% of them developed an EGM before the onset of the sicca syndrome and 26% simultaneously with dryness symptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs 63.5%, p < 0.001). Considering each EGM individually, the following manifestations were more frequent in the non-treated group: arthritis (p < 0.001), fatigue (p < 0.001), purpura (p = 0.01), Raynaud phenomenon (p = 0.003), and hypergammaglobulinemia (p = 0.006). Immunosuppressive treatment was indicated on 28 patients (12.7%), 13 of which were receiving also HCQ. The first reason for those treatments was the presence of arthritis in 12/28 patients (42.8%), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due to the presence of glomerulonephritis, vasculitis, and interstitial lung disease. None of the patients received biologic therapy. The lower incidence of EGM was observed in patients on HCQ therapy supports its efficacy in pSS. However, further large scale prospective studies are needed to confirm these findings.

Background Primary Sjögren syndrome (pSS) is a chronic autoimmune disease with its main target being exocrine glands, and is the connective tissue disease more frequently associated with other autoimmune diseases. The aim of this study was to assess the frequency of another autoimmune rheumatic disease (ARD) developed in primary Sjögren syndrome (pSS) patients and to describe it’s clinical, serological and histologic characteristics. Materials and methods This is a retrospective cohort study. Data of patients with pSS diagnosis (American-European criteria 2002), included in the GESSAR database (Grupo de Estudio Síndrome de Sjögren, Sociedad Argentina de Reumatología) were analyzed. The development of a second ARD was registered during the follow up. Results 681 patients were included, 94.8% female. The mean age was 54 (SD 14) years and mean age at diagnosis of 50 (SD 13) years. The mean follow-up was 4.7 (SD 4.9) years; 30 patients (4.41%, CI 95%: 3.1–5.7) developed a second ARD during the follow up, incidence rate was 9.1/1000 patients-year (IR 95%: 5.8–12.4/1000 patients-year), the most frequent being rheumatoid arthritis (RA). 96% out of these 30 patients had xerophthalmia, 86.2% xerostomia, 92% positive Schirmer test, 88.24% positive Rosa Bengala test, lisamine green or Ocular Staining Score, 81.2% positive unstimulated salivary flow, 82.1% Ro(+) and 33.33% La(+). Minor salivary gland biopsy had been performed in 14 of the 30 patients, 12 with positive results. There were no statistically significant differences respect baseline characteristics when comparing the patients who developed another ARD to the ones that did not. Conclusions Of all the patients analyzed, 4.4% presented another ARD during their follow-up. It is important to be aware of this, to make an early and proper diagnosis and treatment of our patients.

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BackgroundPeripheral neuropathy (PN) is usually is a late onset event in primary Sjögren Syndrome (pSS) associated with purpura, cryoglobulinemia, hypocomplementemia and increased risk of lymphoma.ObjectivesTo describe the frequency of PN in patients with pSS and identify related factors.MethodsAdult patients in the GESSAR database who met 2002 criteria for pSS. Demographic, clinical, laboratory and electromyogram (EMG) findings were recorded. PN was defined with clinic manifestations and EMG. Other causes of PN were excluded. To compare groups, all patients with PN were included (cases) and a random sample of patients without PN (controls) with a 1:4 ratio was used. Mann-Whitney was used for numeric variables and χ2or Fisher's for categorical. An α of 0.05 was considered significant.ResultsOf 368 patients, 95% were female. Mean age at analysis was 55 y/o (21-87) and 50 y/o (20-89) at diagnosis. The frequency of PN was 11.68% (43/368). Sensory PN was found in 63% (28/43), predominantly small fibers involvement in 41.8% (18/43), axonal PN in 20.9% (9/43) and ataxic in 2.3% (1/43). Somatosensory manifestations were found in 37% (16/43) with axonal involvement in 30.2% (13/43) and mononeuritis multiplex in 6.9% (3/43), none had autonomic PN. When comparing groups (43 vs 172 controls) patients with PN had a higher frequency of vasculitis (11.7% vs 1.7%; p=0.002), purpura (23.8% vs 4.7%, p=0.0001), renal tubular acidosis (7.6% vs 1.2%, p=0.020), leucopenia (30.7% vs. 12.1%, p=0.005), low C3 (48.5% vs. 10.3%, p=0.0001) and C4 (66.6% vs. 18.2%, p=0.0001), (+) Anti-Ro/SSA (85.3% vs. 66.6%, p=0.019), (+) RF (72.5% vs. 52.1%, p=0.022), cryoglobulinemia (42.1% vs. 10.9%, p=0.0001) and higher frequency of hypergammaglobulinemia (60.5% vs 44.6%, p=0.09), Raynaud's (27.5% vs 11.6%, p=0.051) and glomerulonephritis (4.6% vs 0.5%, p=0.018), although without statistical significance.ConclusionsThe frequency of PN was 12%, similar to other cohorts. Small fibers and axonal somatosensory PN were the most common. PN was significantly associated with vasculitis, purpura, renal tubular acidosis, cryoglobulinemia, leucopenia, hypocomplementemia and anti-Ro and RF positivity.Disclosure of InterestNone declared

Background Interstitial lung disease (ILD) in primary Sjögren Syndrome (pSS) includes Non Specific Interstitial Pneumonia (NSIP), Usual Interstitial Pneumonia (UIP), Lymphocytic interstitial pneumonia (LIP) and Organized Pneumonia (OP), all related to poor outcome. The existing frequency of ILD is unknown in Argentina. Objectives To describe the frequency of ILD in pSS using the Argentinean Group of Study for Sjögren Syndrome (GESSAR) database and to identify the associated features of the disease. Methods GESSAR database included 374 patients who fulfilled the 2002 criteria for pSS. The diagnosis of ILD was based on High Resolution Chest Computed Tomography (CT) according to the American-European Consensus for ILD. A control/case analysis was performed including patients with (cases) and without ILD (controls) with a 4:1 ratio. Mann-Whitney was employed for numeric variables and chi square or fisher's test for categorical variables. A p<0.05 was considered statistically significant. Results Of the 374 patients analyzed, 95% were women. Mean age at inclusion was 55,67 (range 25-83) and 51,07 (range 21-80) at diagnosis. The frequency of ILD was 6,41% (24/374). Mean age at inclusion was 61,91 (range 51-72) and 52.2 at diagnosis (range 34-69). NSIP was found in 75%, followed by UIP in 20,2% and OP in 4,8%. Ground glass opacities: 87,5%, honeycombing: 20% (2 patients had both). FEV1 and FVC were below predicted values in 58,3% and 54,1% respectively. DLCO was done in 66,6%, with a mean value of 64,6% and 80,2% when DLCO was corrected with VA. Patients with ILD more frequently showed sialoadenitis (28%), purpura (16,6%), Raynaud's (20,8%), non-erosive arthritis (41%), polyclonal hypergammaglobulinemia (41%), hypocomplementemia (29%), leucopenia (28%), cryoglobulinemia (4,1%), PBC (4,5%), anti-Ro/SSA (80%), urticarial vasculitis (4,7%) and glomerulonephritis (9,52%). After case/control comparison, only glomerulonephritis reached statistical significance (p=0.042). Conclusions Although the frequency of ILD was characteristically low in our cohort, NSIP was the most frequent. A significant correlation was found between those patients with ILD and glomerulonephritis. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.2619