E Bartoloni

Primary Sjögren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients

OBJECTIVEnThe aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients serological profile on disease severity and on immunosuppressive drug utilization.nnnMETHODSnCumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression.nnnRESULTSnThe cohort included 1115 (1067 female, 48 male) pSS patients. Severe extraglandular manifestations were detectable in 15% of the patients and were represented by active synovitis (11%), axonal sensory-motor neuropathy (2%), severe leucocytopenia (14%), cutaneous vasculitis (6%) and non-Hodgkins lymphoma (4.5%). We found that low C3/C4, hypergammaglobulinaemia, RF and cryoglobulinaemia were markers of severity for pSS. According to the number of serological variables, the patients were subdivided into three distinct groups: favourable (no serological markers), intermediate (one serological marker) and poor (two or more serological markers). In comparison with the other two patient groups, pSS patients presenting with two or more adverse determinants had a higher frequency of severe visceral disease complications and required more aggressive therapeutic interventions.nnnCONCLUSIONnThis study confirmed that the prevalence of the pSS high-risk subset for severe systemic manifestations is ∼15%. Serological markers might help in the early identification of patients who are candidates to receive more aggressive treatments.

The Need to Target Mucosa-Associated Lymphoid Tissue for Preventing Lymphoma in Rheumatoid Factor-Positive Patients with Sjögren's Syndrome: Comment on the Article by Nocturne et al

This is an area for which there is even less scientific evidence. Although retroactive alterations to recommendations generated through GRADE are not possible, we solicited public comment a priori as noted in the response to the Calabrese letter above. We share the concerns of Yazici et al and believe that such aspects are well-suited for consideration for inclusion in a future ACR guideline, which can ideally be developed and updated using a more nimble process. We also concur in calling for more high-quality evidence related to the use of DMARDs and biologic agents in high-risk patient populations, i.e., RA patients with hepatitis, cancer, heart failure, or serious infections, areas in which there are few studies. We appreciate Yazici and colleagues for drawing attention to this important issue.

THU0268 Prevalence and features of celiac disease in patients with systemic autoimmune diseases: results of a large multicenter study

Background Celiac disease (CD) is an inflammatory and immune-mediated gluten-dependent enteropathy occurring in genetically susceptible individuals. CD is recognized to affect between 0.6% and 1% of worldwide population, with wide regional differences. Disease clinical features are protean and highlight the systemic nature of the disease. In recent years, an increased prevalence of CD has been also reported in patients with connective tissue diseases (CTDs). This association may be due to a shared genetic predisposition, to immunological mechanisms and/or exposure to a common triggering event. However, this observation remains controversial since data are usually based on descriptive case reports. Different methods of antibody detection and enrolled population sample size may contribute to result discordance. Moreover, CD diagnosis is often delayed because disease clinical spectrum may be atypical mimicking rheumatologic conditions and autoimmune disease itself may display typical symptoms of CD. Undoubtedly, awareness of CD occurrence in CTDs is important to prevent potential long-term systemic complications related to an unrecognized CD in these patients. Objectives To evaluate the prevalence of overt and subclinical CD and features of the disease in a large cohort of patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and primary Sjögrens syndrome (pSS) with a multicenter prospective study involving 9 Italian Rheumatology Units. Methods Data from consecutive 580 SLE, 354 pSS and 524 SSc patients were collected. Disease-specific features were registered in patients with known CD. Remaining patients were tested for IgA transglutaminase (Eu-tTG® human IgA new, Eurospital S.p.A., Trieste). Anti-endomysium (EMA) IgA and IgG were tested in IgA tTG positive and borderline patients. Esophagogastroduodenoscopy with duodenal biopsy was proposed in IgA tTG+/EMA+, IgA tTG-/EMA+ and IgA tTG±/EMA+ patients. Results CD prevalence was 1,7% in SLE, 7% in pSS and 1,3% in SSc patients. Higher frequency of elevated liver enzymes was detected in SLE-CD and of herpetiform dermatitis in SSc-CD patients in comparison to the other groups (p<0.05 for both). Interestingly, pSS-CD and SSc-CD patients were younger and had a lower age at diagnosis in comparison to pSS and SSc without CD (p<0.05 for all). Of interest, higher prevalence of CD was detected in SSc patients with diffuse form in comparison to limited SSc (86% vs 14%, p=0.002). Conclusions The results of the present large multicenter study confirm higher prevalence of CD in CTD patients, especially in pSS. Screening of CD may be considered in younger patients with CTD and lower age at diagnosis. The strong association of CD with the diffuse type of SSc is of note and suggests that different, still unexplored, pathogenic mechanisms may characterize the two subsets of the disease. Disclosure of Interest None declared

THU0352 Worldwide Heterogeneous Diagnostic Approach To Primary Sjögren Syndrome in 8315 Patients (EULAR-SS Task Force Big Data Sjögren Project)

Objectives To analyse the diagnostic approach used in the largest international cohort of patients with primary Sjögren syndrome (pSS) Methods The Big Data Sjögren Project is a multicentre registry formed in 2014 by experts participating in the EULAR-SS Task Force. By January 2016, the database included 8315 consecutive patients fulfilling the 2002 criteria. The main features at diagnosis/recruitment (time of criteria fulfilment) were collected and analysed Results The cohort included 7753 (93%) women (mean age at diagnosis 53 years). Sicca symptoms were reported in more than 90% of patients at diagnosis (92% for dry eye and 92.5% for dry mouth). The diagnostic tests used included the determination of anti-Ro/La antibodies in 8250 (99%) patients, Schirmer test in 6205 (75%), salivary gland biopsy in 5988 (72%), salivary flows in 4941 (59%), corneal stainings in 3304 (40%), scintigraphy in 2578 (31%) and sialography in 873 (10%) patients. The mean number of diagnostic tests included in the 2002 AE criteria was 4.85 ± 1.44 (of a maximum of 8 different tests), and was higher in patients with negative ANA (5.18 vs 4.80 in ANA+, p<0.001) or negative RF (5.06 vs 4.81 in RF+, p<0.001), and was also higher in American patients vs European or Asian patients (5.28 vs 4.83 and 4.63, respectively). A correlation was found between the number of tests carried out and the number of 2002 criteria finally fulfilled (R=0.48) Conclusions We found a heterogeneous diagnostic approach of pSS with respect to the number of 2002AE diagnostic tests carried out; the approach varied significantly according to geographic origin and baseline immunological profile Disclosure of Interest None declared