Background:Apremilast (APR), a small molecule inhibitor of phosphodiestersa-4, has been shown to be effective in the treatment of oral and genital ulcers in Behçet’s disease (BD). BD is a systemic vasculitis with great heterogeneity of symptoms and manifestations. It can affect the blood vessels, mucosa, skin, joints, eyes, nervous system and digestive system. APR is indicated for treatment of oral ulcers in BD in some countries such as the USA and Japan.Objectives:To evaluate the efficacy and safety of APR treatment in BD patients.Methods:Single-center descriptive study of BD patients on APR treatment from February 2017 to December 2019. Demographic, clinical and analytical data were collected.Results:10 patients (9 women) were included, with a mean age at the beginning of APR treatment of 37±12 years and a mean disease evolution of 100±105 months. 8 of them showed HLAB51 positivity.Before treatment with APR, 4 patients were refractory to DMARDs (2 MTX, 2 AZA) and one patient to 2 anti-TNF (ADA, IFX). All patients were under treatment with colchicine and 5 with steroids before APR therapy. The concomitant treatment with APR were: corticosteroids (5), NSAIDs (2), colchicine (8), MTX (2), AZA (2).The main symptoms at the beginning of the APR treatment were: oral ulcers (100%), genital ulcers (60%) and arthritis/arthralgia (90%). We observed a clinical improvement after 3 months of treatment of 90% of oral ulcers, 100% of genitals and 55% of joint symptoms. The patients had a mean follow-up of 37±12 months and they maintain the therapy response during the APR treatment.Patients presented adverse events, some of them transitory: headache (5), diarrhea (5), nausea (3), dysthymia (1), tremors (2), herpes zoster (1) and autolytic ideation (1). The treatment was withdrawn in 4 patients with a mean duration of 11 ± 13 months. 2 of the 4 adverse events were by autolytic ideation and nausea, 1 for genesic desire and 1 for persistence of joint injury. The APR doses were reduced to 30 mg per day in 4 patients, resolving the adverse events and persisting with a good response. In addition, dose reduction of colchicine and prednisone was achieved in 4 patients.We observed other previous manifestations of BD such as uveitis (4), neurobehçet (3), cutaneous (folliculitis/pseudofoliculits) (4) and venous thrombosis (1). Cutaneous manifestations were resolved and the rest of previous manifestations remain without clinical changes during the follow-up.Conclusion:We observed an improvement in the most common manifestations of BD and a safety profile similar to those described in other studies. We observed a resolution of mucocutaneous manifestations, a variable response in joint manifestations and stability in neurological manifestations. Adverse effects referred included gastrointestinal and headache, most of which were transient and were resolved with adjustment of treatment.Disclosure of Interests:None declared

Background:Spondyloarthropathy patients have reduced health-related quality of life (HRQL) compared to general population. Biological treatment strategies in real life aim to reduce patients’ symptoms and different HRQL parameters would share the same behavior as patients’ symptoms and disease activity.Objectives:We aim to assess the differences in HRQL reported by spondyloarthropathy patients during the first six months of biological therapy according to the treatment effectiveness.Methods:We performed a prospective observational study of six months of follow-up in spondyloarthropathy patients who are newly on biological therapy. A basal visit and 1, 3 and 6 months follow-up visits were conducted. We analyzed changes during follow-up in the HRQL parameters reported by patients through AsqoL and ASAS-health-index questionnaires. Moreover we measured functional disability through HAQ and BASFI index, disease activity by BASDAI, ASDAS-CRP and ASDAS-ESR index.Statistical analyses were achieved using R software, through multivariate linear regression models for continuous data and Bayesian mixed ordinal regression models with monotonic effect for ordinal data. The corresponding odd ratios (OR) were calculated with their confidence intervals (CI 95%).Results:We included 53 patients (71.77% male), the 73.58% diagnosed with ankylosing spondylitis (AS) and the 26.42% with axial spondyloarthritis. Mean age at the beginning of treatment was 48.74 (11.21) years, mean age at diagnosis was 41.57 (11.97) and mean disease evolution was 7.19 (9.24) years. 60.42% of them exhibited HLAb27 positivity.34 patients started biological therapy with TNF-α inhibitors and 19 with IL-17 inhibitors. The 81.13% of patients were under monotherapy, and the other 18.87% was treated with DMARDs. The 77.36% of the total number of patients was on the biological therapy at 6 months of follow-up, while the 22.64% discontinued at 6 months of follow-up (9 due to inefficacy and 3 due to adverse effects).42 patients completed the follow-up at 6 months, and 3 patients achieved until visit 3 due to treatment failure. In 1 case visit 1 and in 7 cases visit 3 could not be performed due to COVID19 pandemic situation.We observed a significant correlation among AsqoL and ASAS-hi values with disease activity indexes (BASDAI, ASDAS-CRP, ASDAS-ESR) and functional disability (HAQ, BASFI). The statistical analyses showed a significant association where AsqoL and ASAS-hi values are significantly decreased in treatment failures compared to the successful treatment (, and in patients with previous biological therapy compared to naïve patients. No effect of years of disease evolution and the type of biological treatment in the AsqoL and ASAS-hi values was observed. (See Table 1) These results were consistent with the significant association found among the disease activity and functional disability with the biological therapy efficacy and the previous biological treatment.Table 1.Comparison with effectiveness of biological therapyOdds Ratio (OR)95% CIComparison with previous biological therapyOdds Ratio (OR)95% CIAsqoL1.5021.123–2.033AsqoL10.7041.232–108.504ASAS-HI1.561.184–2.078ASAS-HI11.5531.299–108.974BASDAI1.51.199–2.04BASDAI11.961.823–86.901BASFI1.3481.036–1.76BASFI18.372.15–176.988ASDAS-CRP1.5381.176–2.036ASDAS-CRP8.8692.001–42.272ASDAS-ESR1.9441.166–3.384ASDAS-ESR44.6212.886–1077.816HAQ1.5241.154–2.027HAQ24.1111.779–371.742Conclusion:The AsqoL and ASAS-hi questionnaire results are correlated with disease activity and functional disability and showed the same behaviour as these parameters, being also associated to the biological therapy efficacy as well as to previous biological therapies. The HRQL variables would be additional clinical results that make it possible to achieve a better management of biological therapies in spondyloarthropathy patients.Disclosure of Interests:None declared.

Background:Rhupus syndrome (RhS) is a rare combination of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Different studies describe RhS cases that begin with erosive arthritis and the presence of rheumatoid factor (RF) and/or anti CCP and then the SLE symptoms.Objectives:Despite the fact that RhS shows a low prevalence, it would be useful to know clinical characteristics of RhS patients since their therapy and outcome differ from those having RA or SLE alone.Methods:Retrospective study with systematic revision of electronic clinical records of RhS patients was performed. Demographic, clinical and immunological data were collected.Results:Eight RhS patients were included (all fulfilled SLICC 2012 criteria for SLE and ACR 2010 for RA). Mean age was 67.3 (45-84) years (7 were female).In 3 cases RA was the first diagnosis with a mean evolution of 4.5 years until SLE diagnosis. In contrast, in 5 cases SLE was the first diagnosis with a mean evolution of 7.2 years until RA diagnosis. Photosensitivity and arthritis were the predominant clinical manifestations. One patient presents pericarditis and other case showed rheumatoid nodules in elbows. Renal, pulmonary or neurological affection was no reported.4 patients were under biological/JAK inhibitors therapies (2 abatacept, 1 rituximab and 1 baricitinib) with favorable response of treatment.Conclusion:In contrast to other series, only the 37.5% of our RhS cases begins with polyarticular seropositive arthritis. The 62.5% started with SLE symptoms as haematological alterations, cutaneous and serological manifestation, and showed longer progression to have polyarticular affection. Thus, RhS diagnosis is earlier in patients that begin with RA symptoms. 4 RhS patients were refractory to DMARd treatments, where biological/JAK inhibitors therapies are needed.Disclosure of Interests:None declared

Background:Systemic Sclerosis (SSc) is an autoinmune disease that can affect several organs and its mortality is fundamentally related to its pulmonary involvement.It is mandatory to seek for biomarkers that help us with early diagnosis and that are also useful for predicting organic involvement, so that we can adjust the diagnostic and therapeutic approach.Objectives:Our aim was to check if the presence of CXCL4, CXCL8 and GDF-15 is greater in the disease than in healthy population, and also their involvement in organic damage.Methods:Observational and cross-sectional study, with a prospectively performed protocol, of patients diagnosed of SSc according to ACR/EULAR 2013 criteria. Demographic, clinical, analytical, activity (EUSTAR index), severity (Medsger scale and modified Rodnan index), health perception (SF36) and disability (HAQ and Cochin test) variables were collected. Moreover, Videocapillaroscopy (VCL) and Respiratory Function Test were made, as well High Resolution Lung Tomography and Echocardiography in order to describe pulmonary features. Serum levels of CXCL4, CXCL8 and GDF-15 were measured in SSc patients and in healthy controls.Results:A total of 42 patients (95.4% women) were included, with an average age of 59.2 years. The median of years since diagnosis was 4, by 6 since the first non-Raynaud symptom. 20 patients were diagnosed with limited SSc, 20 patients diffusely and 2 patients with SSc without scleroderma. 42 healthy controls were also included.We found significantly higher levels of GDF-15 in patients with SSc (P<0.001), without significant differences in CXCL4 and CXCL8 levels between patients with SSc and healthy controls.The presence of GDF-15 was associated with diffuse SSc (P=0.009), pulmonary arterial hypertension (P=0.038), interstitial lung disease (P=0.004), decreased forced vital capacity (FVC), (P=0.002), high serum titles of antiScl70 (P=0.006), increased disease activity measured by EUSTAR index (P=0.001), as with capillary dilations in Capillaroscopy (P=0.015).Moreover, we found an association between CXCL4 levels and the consumption of complement C3 fraction (P=0.008) and skin involvement (higher Rodnan modified score), (P = 0.001); not finding association with lung involvement or other features (spirometric or analytical changes, capillaroscopy or functional tests).Attending to CXCL8, it was associated to consumption of the C4 fraction of complement (P=0.013) and the presence of tortuosities in capillaroscopy (P=0.02) with no other significant findings.Conclusion:The presence of GDF-15 is associated with diffuse SSc, lung impairment, disease activity and changes in capillaroscopy. In addition, CXCL4 was only associated with skin involvement, while CXCL8 was not related to any organic damage in our patients.Disclosure of Interests:None declared

Background:Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and is often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension.Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from an early diagnosis of pulmonary involvement.Objectives:Our aim was to assess the pulmonary function in patients diagnosed of Early SSc.Methods:Retrospective observational study of a wide and unselected series of patients diagnosed as Early-SSc from a single university hospital from 2012 to 2019. Patients were classified as Early-SSc following Le Roy criteria. Despite this, patients already did not meet 2013 ACR/EULAR classification criteria for SSc. We reviewed pulmonary function through conventional spirometry and diffusing capacity of lung for carbon monoxide (DLCO).Results:We included 56 patients with a mean age of 52.3±12.1 years (96.4% women; 3.6% men).At the diagnosis of Early-SSc, no one of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 18 patients (32.1%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 24 patients (42.8%).After a mean follow-up period of 38.3±2.4 months, 29 (51.8%) patients fulfilled 2013 ACR/EULAR criteria. The average time between diagnosis of Early-SSc and achieve SSc classification was 24.4±1.8 months. The remaining 27 patients continued classified as Early-SSc.An analysis of the subgroup of patients which progressed to SSc showed that DLCO was decreased in 15 of those 29 patients (51.7%) and 18 of 29 patients (62.1%) presented decreased MMEF 25-75. Comparing with the subgroup of patients which not progressed to SSc were significant differences (Decreased DLCO: 51.7% vs 11.1%; p=0.02 and decreased MMEF 25-75: 42.8% vs 22.2%; p=0.05).The analysis of pulmonary function of the subgroup of patients continued classified as Early-SSc after follow-up period did not show significative changes after follow-up.Conclusion:In our study, a third of the patients classified as Early-SSc presented at diagnosis abnormal values of DLCO and/or signs of small airway obstruction without the presence of a restrictive ventilatory pattern. Moreover, this pulmonary disfunction was significantly more frequent in patients who progressed to definitive SSc. Patients which remains classified as Early-SSc did not experience significative changes.Our results support the concept that pulmonary function was impaired in Early-SSc and that I should probably be considered for future Early-SSc classification criteria.Disclosure of Interests:None declared