BackgroundAntisynthetase syndrome (ASS) is characterised by the presence of myositis, arthritis, interstitial lung disease (ILD), fever, Raynaud’s phenomenon and mechanical’s hand, in the presence of antisynthetase autoantibodies (AA), the most frequent being anti-Jo1, anti-PL7 and anti-PL12. An association between ASS and anti-Ro52 with increased ILD has been described and it is believed that the presence of both antibodies is accompanied by a more severe ILD.ObjectivesTo describe the clinical and analytical characteristics of a cohort of patients with ASS. To analyse the lung involvement in this type of patient and to determine the possible relationship between the different subtypes of ILD and the presence of anti-Ro52.MethodsRetrospective descriptive study of patients treated in our Hospital (2006–2017), with AA and at least 2 clinical characteristics. The data was obtained through the review of medical records.Variables analysed:age, sex, age, smoking, clinical presentation, diagnosis of ASS, associated neoplasia and paraneoplastic syndrome (PS)(neoplasia 3 years before or after the diagnosis of ASS), muscle enzymes (CK and aldolase), autoimmunity, glucocorticoids (GC), immunosuppressants (IS), diagnosis of ILD, HRCT pattern (High Resolution Computed Tomography) and respiratory function tests (RFT) at the beginning of ILD.ResultsWe included 27 patients (20 women), mean age 61±13 years. 7.4% smokers and 18.5% ex-smokers. 88.8% were anti-Jo1,7.4% anti-PL12% and 3.7% anti-PL7. Anti-Ro52 present in 18 patients. The most common clinical presentation:ILD 88% (59% had Ro52), followed by myositis 85% (40% are dermatomyositis), arthritis 81%, mechanic’s hand 51%, fever 37% and Raynaud’s phenomenon 25%. The classic triad (arthritis, myositis, ILD) was present in 16 patients. Three patients presented neoplasia in the course of the disease, being identified as PS. Elevation of CK in 70% and aldolase in 74%. 96% of patients have been treated with GC and IS.The HRCT patterns were:non-specific interstitial pneumonia (NSIP)(66%), usual interstitial pneumonia (UIP) (29%),organised cryptogenic pneumonia (OP)(4%), baseline RFT were performed in 19 patients. Diagnosis of ASS and ILD, both entities appear at the same time in 6 patients, in 3 patients the ILD appears before and in 14 after. In these, the median duration (range) of the ASS until the diagnosis of ILD was 1 year (0–1).There is no relationship between the HRCT and anti-Ro52 patterns (chi-square considering the exact distribution p=0.892), nor between the ILD and anti-Ro52 (Fisher exact test p=0.999).ConclusionsOur results, in general, agree with what is published in the literature. Three patients have an uncommon presentation of ASS, with a diagnosis of ILD prior to myopathy (in most of the published cases, myositis precedes or coincides with the onset of IDL), and it is important to include ASS in the differential diagnosis of ILD. In our cohort, the association between ILD and anti-Ro52 has not been demonstrated, nor among the different subtypes of ILD to Ro52. Ther...
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:Sarcoidosis is a systemic disease whose etiology is unknown. It is characterized by the formation of granulomas in any tissue of the organism. Ganglionic, pulmonary and cutaneus involvement is the most prevalent.Objectives:Describe clinical characteristics of a cohort of patients with sarcoidosis diagnosed.Define the association between the ACE’s number at diagnosis, radiological lung stage, treatment and course of disease.Evaluate if the extrapulmonary involvement is related to the course of the disease.Methods:Descriptive retrospective study of patients with S diagnosis treated in our Hospital in 2019. Data were obtained by reviewing medical records. Chi-square tests and parametric tests have been used to establish the differences described in the objectives.Results:102 patients diagnosed with sarcoidosis have been included, (51% females) with an average age of 56±11 years. Suspected diagnosis at the onset of disease was S in 70.6% of patients, followed by suspected lymphoma (20.6%). The average time for the definitive diagnosis of S was 9.5 months. 70.6% of the patients had elevated ACE titles at the beginning. Regarding the clinical manifestations, 18.6% of the patients presented fever at the beginning and 66.7% extrathoracic clinical manifestations. 72.5% have lymph node adenopathies, and in 91% there is thoracic involvement (the most frequent pulmonary stage is stage II). A biopsy was performed in 84.3% of the patients, the lung biopsy being the most performed (52.3%). 88.2% of patients received corticosteroid treatment at the onset of the disease (currently under treatment with corticosteroids 37.3%). 50% of patients are treated with immunosuppressants, Methotrexate was the most used. 5 patients are treated with biological therapy (AntiTNF).Regarding the course of the disease, 51% of the patients have a chronic course, 45.1% are in remission and 3.9% have suffered a relapse of the disease. In this study, no significant relationship was found between the ACE values at the onset of the disease, the pulmonary stage and the course of the disease.According to our data, patients presenting with extrathoracic clinical manifestations need more frequently corticosteroid treatment (p = 0.017) and immunosuppressive treatment (p = 0.001) with respect to patients who do not have an extrathoracic clinic. In addition, patients with an extrathoracic clinic present more frequently a chronic course of the disease than those who do not (p = 0.019).Conclusion:The results described in this study are similar to those found in the literature. The differences found can be explained because patients presenting with extrathoracic clinical manifestations have a more complicated management and need more treatment than those with only pulmonary involvement, even patients with radiological stage I do not usually need treatment, only surveillance.Disclosure of Interests:None declared
BackgroundIn patients with haemophilia, the development of inhibitors to factor VIII/IX (Haemophilia A and B, HA/HB), prevents adequate replacement therapy and results in increased risk of serious bleeding episodes, poor control of joint bleeding, and progressive, debilitating joint disease.ObjectivesTo describe, according to the Arnold-Hilgartner scale (AHRS), the radiological findings in a cohort of hemophilic arthropathy (HArth) patients and to analyse the relationship that may exist between the degree of joint involvement and HA/HB, severity, age and presence or absence of inhibitor.MethodsThis is a retrospective study, carried out in the Haemophilia Unit of our hospital, in patients with HArth (2007–2017). Severity of haemophilia: percentage of coagulation factor (CF) activity, moderate (1%–5%) and severe <1%. The AHRS includes 5 levels.For the study of the association of the type and degree of haemophilia; and the presence of inhibitor, with the level in the AHRS, a linear model was used. The significance in this regard of the linear contrasts of interest was studied using F tests, defining the first species error at 0.05.ResultsWe included 88 patients. Characteristics of the patients in the attached chart. No significant association was detected between the type and severity of haemophilia with the development of inhibitor.The results of the linear model only showed association (p=0.01) between the severity of haemophilia and AHRS, patients with moderate haemophilia presented a least square mean for the AHRS of 2.6 (0.3), whereas the serious ones had a value of 3.4 (0.2). The least squared means for patients with HA and HB were 2.9 (0.2) and 3.1 (0.4) respectively, the contrast did not reach statistical significance (p>0.1), and the same occurred between patients who had generated or not inhibitor 2.8 (0.2) Vs 3.1 (0.3). Apart from the severity of haemophilia, the other factor that showed a significant and significant effect (p<0.001) on the AHRS values was age. The estimated value for the regression coefficient of age on the radiological status was 0.05 (0.009).Abstract AB1134 – Table 1Type of Haemophilia Haemophilia A n (%)73 (82%)Haemophilia B n (%)14 (15%)Severity of HaemophiliaHaemophilia A severe n (%)50 (56%)Haemophilia A moderate n (%)23 (26%)Haemophilia B severe n (%)13 (14%)Haemophilia B moderate n (%)1 (1%)Age m (SD)31 (17)Development of inhibitors to factor VIII/IX n (%)16 (18%)Haemophilia A severe with inhibitors n (%)10 (11%)Haemophilia A moderate with inhibitors n (%)3 (3%)Haemophilia B severe with inhibitors n (%)3 (3%)AHRS scale 1 n (%)18 (20%)AHRS scale 2 n (%)17 (19%)AHRS scale 3 n (%)18 (20%)AHRS scale 4 n (%)15 (17%)AHRS scale 5 n (%)19 (21%)ConclusionsContrary to what has been observed in other studies, in our cohort it does not seem that the presence of inhibitor or the type of haemophilia has a negative effect on the severity of the radiological findings. The age and severity of haemophilia do seem to influence the radiological stage.Disclosure of InterestNone declared
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