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Objectives To analyze the efficacy and safety of treatment with belimumab, a monoclonal antibody that specifically binds the soluble form of the protein human B lymphocyte stimulator B (BLyS), in SLE patients refractory to standard treatment. Methods In 2006, the Study Group on Autoimmune Diseases (GEAS) of the Spanish Society of Internal Medicine created the BIOGEAS registry, a multicenter study recruiting patients with refractory systemic autoimmune diseases treated with biological agents. Results On December 31, 2013, a total of 10 patients treated with belimumab were included in the BIOGEAS Registry, 9 females and 1 male, with a mean age of 41.8 years (range 24-71 years). These patients were heavily treated, with a mean of 7 years of corticosteroid treatment, antimalarials and at least one immunosuppressive agent, mycophenolate in 9, azathioprine in 7, metothrexate in 4, cyclosphosphamide in 2, cyclosporine A in 2, tacrolimus in 2 and talidomide in 2. Belimumab (10mg/kg) was administered in combination with corticosteroids (in all patients), antimalarials (6 patients) and immunosuppressive agents (8 patients, 6 with mycophenolate). The baseline average activity score (SELENA-SLEDAI) was 12 (range 6-33); 8 patients had high titers of anti-dsDNA and/or hypocomplementemia. Therapeutic indication included refractory mucocutaneous involvement (4 patients), refractory vasculitis (3 patients), systemic disease (2 patients) and refractory lupus nephritis (1 patient). After a mean follow-up of 7 months (range 2-12), clinical response could be evaluated in 9 patients, 8 classified as responders (improvement greater than 80% in 5 patients), while the remaining patient was classified as stable. Two patients (20%) developed adverse events (raised transaminases and systemic cryptococcosis due to severe CD4 lymphopenia); there were no deaths or lupus flares. Conclusions These postmarketing data in Spanish SLE patients in whom belimumab was used following the recommendations approved by the FDA/EMA (refractory disease to standard treatment, clinical and immunological active disease, SELENA-SLEDAI>6) have showed promising results, with a good therapeutic response and a safety profile similar to other biological therapies used off-label in patients with SLE. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5447

Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sjögren syndrome (SS). In the 1990s, a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease, liver function and a complete panel of autoantibodies. Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS. The most recent studies have shown that, after eliminating hepatotoxic drugs and fatty liver disease, the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases. The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important, since the two processes require different therapeutic approaches and have different prognoses. With respect to viral infections, chronic HCV infection is the main cause of liver involvement in SS patients from the Mediterranean area, while chronic HBV infection may be the main cause of liver involvement in SS patients from Asian countries. After eliminating viral hepatitis, primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS. PBC-related SS patients may have a broad spectrum of abnormalities of the liver, including having no clinical or analytical data suggestive of liver disease. Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I), and nearly 10% of these patients have an AIH-PBC overlap. Finally, IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis, especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present.

ObjectivesTo analyse the epidemiological, clinical and immunological characteristics of an international cohort of patients diagnosed with primary Sjögren syndrome (SS) according to the 2002 AE criteria.MethodsThe Big Data Sjögren Project is an international, multicentre registry formed in 2014 to take a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases. By January 2015, 5027 consecutive patients fulfilling the 2002 classification criteria for primary SS were included from 9 European and 4 American countries.ResultsThe cohort included 4714 (94%) women (female:male ratio, 15:1), with a mean age at diagnosis of primary SS of 54 years (range, 10-97), of which 94% were Caucasian and 88% lived in European countries. The frequency of fulfilment of the 2002 criteria was: 94.4% for dry eye, 92.9% for dry mouth, 88.5% for positive salivary gland biopsy, 85.9% for positive ocular tests, 74.8% for positive oral tests and 70.9% for positive Ro/La autoantibodies. As a minimum of 4 of the 6 criteria are required for fulfilment, the percentage of diagnostic tests performed varied: Ro/La autoantibodies were tested in 99.5% of patients, ocular diagnostic tests (Schirmer's test and/or corneal stainings) were made in 90.2%, oral tests in 76.7% and salivary gland biopsy in 72% of patients. Systemic involvement at diagnosis was retrospectively measured using ESSDAI definitions in 3314 patients and included articular involvement (35.2%), glandular involvement (19.3%), lymphadenopathy (10.7%), cutaneous involvement (9%), constitutional involvement (8.4%), respiratory involvement (7.3%), peripheral nervous system involvement (5.2%), renal involvement (2.2%), central nervous system involvement (1.7%) and muscular involvement (1.2%). With respect to laboratory abnormalities, 43.3% of patients showed biological abnormalities and 24.7% haematological abnormalities according to ESSDAI definitions.ConclusionsIn this international cohort of 5027 patients with primary SS, most non-sicca clinical features and laboratory abnormalities present at diagnosis are not included in the current criteria; their inclusion in future proposed classification criteria should be evaluated.Disclosure of InterestNone declared