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Background:Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis that is associated with early mortality. The development or progression of ILD can occur at any time, so patients should be monitored regularly, particularly in the first years after diagnosis.Treatment should be considered when the disease is clinically significant, particularly when there is evidence of progression based on a decrease in lung function, progression of fibrosis on the HRCT or worsening of respiratory symptoms.Objectives:-To relate the type of systemic sclerosis (SS) with pulmonary involvement with the radiological pattern.-To study if there is a relationship between the antibodies and the aforementioned affectation.Methods:Retrospective descriptive study of patients treated in our Hospital (2009-2019) by the Rheumatology and Internal Medicine department diagnosed with systemic sclerosis and interstitial lung disease.The data were obtained through the review of medical records.We have included data from patients who have a diagnosis of limited or diffuse systemic sclerosis or overlap with interstitial lung involvement.Results:Of the 213 patients with systemic sclerosis in our database 43 had interstitial lung involvement (20.2%). 79% of the patients with ILD (34) had a non-specific interstitial pneumonia type (NSIP) radiological pattern and 21% of the patients (9) had a pattern of usual interstitial pneumonia (UIP)Among the patients with ILD with a NSIP -type radiological pattern, 19 patients were diagnosed with diffuse SS, 9 patients with overlap syndrome and 6 with limited SS.Of the patients with ILD with radiological pattern type UIP, 5 patients were diagnosed with diffuse SS, 3 patients with overlap syndrome and 1 patient was diagnosed with limited SS.TABLE 1.RELATIONSHIP BETWEEN SS TYPE AND RADIOLOGICAL PATTERNNSIPUIPLIMITED SS6 (17.6%)1 (11.1%)DIFFUSE SS19 (55.8%)5 (55.5%)OVERLAP9 (26.4%)3 (33.3%)Among the patients with the NSIP pattern, 17 had positive SCL70 antibody, 3 positive ANA patients and 1 patient had positive anti-centromere antibody.Of the patients with UIP type interstitial pneumopathy, 8 patients had anti-SCL70 antibody, 3 patients ANA positive antibody and 2 patients anti-centromere positive antibody.TABLE 2.RELATIONSHIP BETWEEN TYPE OF AB AND RADIOLOGICAL PATTERNNSIPUIPAnti SCL70178Anti centromere12ANA33Regarding treatment, 21 patients were taking Mycophenolate, 16 patients required cyclophosphamide and 6 patients rituximab.No patient in our cohort died due to interstitial lung disease.Conclusion:The data obtained are consistent with what is collected in the medical literature.The subtype of scleroderma most related to ILD was diffuse SS. The most frequent antibody was anti-SCL 70.Regarding the treatment,the most used in ILD in our center was the mycophenolate.From our sample analyzed when applying the likelihood ratio (RV) a value of 47,186 is obtained, which has an associated probability of 0, which is less than 0.05, leads to reject the null hypothesis (there is no dependence between antibodies and type of radiological pattern of ILD in SS), concluding that there is dependence between the analyzed variables.After this analysis, we can conclude that in our sample there is a relationship between the type of interstitial pneumopathy pattern and the antibody present in patients with SS.Disclosure of Interests:None declared

1Seville, Seville, SpainBackgroundAssessment of pain improvement during treatment for Rheumatoid Arthritis (RA) may be useful to clinical decision between providers and their patients (pts).Baricitinib (BARI) once daily, an oral, selective Janus Kinase (JAK)1/JAK2 inhibitor, reduced disease activity levels in Rheumatoid Arthritis (RA) patients (pts) with an inadequate response (IR) to methotrexate (MTX).ObjectivesTo Evaluate the likelihood of achieving different levels of pain control with BARI 2 mg or 4 mg in patients with RA with inadequate response to traditional DMARDs or biological DMARDs.MethodsProspective observational registry of pts with RA who start treatment with BARI, in a third level Spanish Hospital (October 2017- June 2018). BARI 2 mg is started in patients with inadequate response to traditional DMARDs and BARI 4 mg in patients with inadequate response to biological DMARDs. The pts were assessment of pain was assessed with 0-100 mm visual analog scale (VAS) at each study visit. The likelihood of achieving >=25%, >=50% and >=70% pain VAS improvement through week 12 and analyze if there are significant differences between the group of patients with BARI 2 mg and BARI 4 mg (Mann-Whitney test). The statistical study was carried out with the SPSS15 computer package.ResultsWe included 38 pts (28 women), mean age 52 ± 12 years. Pain VAS improvement for all patients, baseline pain and weeks 12. The frequency is the percentage of improvement with respect to the baseline. In BARI 2 mg group, 58% of pts (p75) have experienced a decrease greater than pain VAS improvement than baseline and in BARI 4 mg group, 55% of pts (p75) have experienced a decrease greater than pain VAS improvement than baseline.No statistically significant differences were found in the two treatment groups (BARI 2 mg and BARI 4 mg) (p 0.847).ConclusionOur results, in general, agree with what is published in the literature (RA treated with BARI reported greater improvements in pain control when compared to adalimumab or placebo, a post-hoc analysis of the Phase 3 RA-BEAM study). BARI treated pts reported significantly greater and more rapid reductions in pain severity as measured by the pain VAS, improvements were sustained 12 weeks, without finding differences in pts receiving BARI 2 mg or BARI 4 mg.Disclosure of InterestsNone declared

Background: Familial Mediterranean fever (FMF) is the most frequent monogenic periodic fever syndrome and is characterized by recurrent episodes of fever, serositis, arthritis, dermal manifestations and long-term renal complications (amyloidosis is the most important complication). The genetic mutation of the disease is found in the MEFV gene located on the short arm of chromosome 16 and is inherited in an autosomal recessive manner. It affects the populations of the Mediterranean basin and is diagnosed according to the clinical evaluation. Objectives: To describe the clinical characteristics of a cohort of patients with FMF and to study the different genetic mutations located in the MEFV gene. Methods: Retrospective descriptive study of patients treated in our Hospital (2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018), with FMF diagnosis and MEFV gene mutation. The data was obtained through the review of medical records. Results: We included 52 patients (29 men), mean age 41 years. The following mutations have been identified in alleles of the MEFV gene: Non-pathogenic in 34 patients (65%) (p 202Q 73%, p148Q 17%, p. 319K 6% and p339F 3%). Pathogenic in 18 patients (34%) (pr 202Q 22%, eg 148Q 27%, pr 653H 5%, pm 694V 16%, pi 159T 5%). In 17% of the patients family history was documented. Elevation of acute phase reactants in 41 patients (79%) (C Reactive Protein 75%, Globular sedimentation rate 65%). Echocardiography was performed in 14 patients, with diagnosis of pericardial effusion in 6 of them. Two patients develop renal amyloidosis, one of them (homozygous for the mutation 148Q) died due to this complication. 54% of patients use colchicine as initial treatment, achieving 50% good response with control of symptoms. 19% undergo treatment with glucocorticoids (0.5-1 mg/ kg/day), needing to add methotrexate in 1 patient and hydroxychloroquine in another. Four also use biological therapy (1 tocilizumab, 2 anakinra, 1 canakinumab) and 2 thalidomide to control skin manifestations. Conclusion:The genetic study confirms the diagnosis of FMF, allowing it to be differentiated from other SAIs. It also has prognostic value, depending on the mutation detected and if it affects one or both alleles. In our series, the most prevalent symptoms in patients with pathogenic mutations were fever, abdominal pain and arthromyalgia.

BackgroundSarcoidosis (S) is a systemic granulomatous disease of unknown etiology. The most frequent affectation is pulmonary, ocular and cutaneous, although sarcoidosis can damage other organs, such as the musculoskeletal system.ObjectivesTo describe the clinical characteristics and the radiological pattern, in a cohort with predominant pulmonary S, as well as to establish the relationship between the angiotensin converting enzyme (ACE) levels and the S course (chronification or remission).MethodsThis is a retrospective descriptive study of patients treated in our hospital, since 2008 to 2018, with diagnosis of S. The data was obtained through the review of medical records. The delay in the diagnosis of S was defined as the difference in months between the initial diagnostic suspicion and the final diagnosis of S.We use Chi-square tests to study the association between ACE levels and the course of the disease.ResultsFifty-five patients (31 women) were included, with a mean age of 52 ± 12 years. The first diagnosis was: 85% S, 10% lymphoma and 4% tuberculosis. The median of months for the definitive diagnosis of S was 5.5 months.Extrapulmonary clinical manifestations in table 1.The ACE is increased in 38 patients (70%). Simple x-ray and high resolution tomography of chest were done in all patients. Pulmonary stage 2 was the most frequent (51%), followed by stage 3 (16%), stage 0 (14%) and stage 4 (9%).In 90% of the patients, histological confirmation was obtained by transbronchial (47%), cutaneous (11%) or lymph node biopsy (29%).94% of the patients have been treated with oral glucocorticoids, 52% associate immunosuppressive therapy (Methotrexate 27% and Azathioprine 14%) or biological treatment (1 patient with Adalimumab and another with Infliximab). In 54% of the patients the S had a chronic course and in 43% S remitted.Increased levels of ACE were associated with clinical remission of the disease and normal levels with chronicity (p: 0.013). EXTRATHORACIC CLINICAL MANIFESTATIONS N(%) Skin15(27)Neurological4(7)Cardiac8(14)Renal2(4)Ocular7(13)Monoarthrittis3(5)Poliarthritis6(11)Bilateral swelling in ankles6(11)Hepatosplenomegaly8(14)ConclusionThe results of our study resembles, in general, what has been published in the literature. In our study, elevated ACE is associated with remission of the disease, contrary to the published, in which increased levels of ACE in symptomatic patients may reflect disease activity.Disclosure of Interests None declared