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Funding Acknowledgements Type of funding sources: None. INTRODUCTION CRH in patients with ischemic heart disease is recommended by the different clinical practice guidelines with an IA level of evidence, with an important role in reducing cardiovascular mortality and hospital readmissions during follow-up. OBJECTIVE The goal of this study is to show the 4-year clinical results of a population of patients who participated in an CRH program after an Acute Coronary Syndrome (ACS). METHODS Between May/2014 and September/2017, 221 patients who had recently presented an ACS completed the 12 weeks of phase II of the CRH program at our center. In May/2020 we collected epidemiological, clinical and echocardiographic information at the time of the acute cardiovascular event; and we evaluate the current vital status of the patients and the incidence of readmissions for: angina, HF, new ACS, or arrhythmic events. RESULTS Of the 221 patients, 182 were men (82%). The mean age of our population was 58.3 ± 7.8 years. 58% (129 patients) suffered from ST-elevation ACS. The mean time of hospital stay was 6.20 ± 2.9 days. An echocardiogram was performed at discharge, which showed an average LVEF of 56 ± 6%. Eight patients (4%) developed early Ventricular Fibrilation (VF) during the acute phase of ACS. Among the classic CVRF, smoking (79%) was the most prevalent, followed by dyslipidemia (53%) and hypertension (47%). The mean time from hospital discharge to the start of phase II RHC was 42 ± 16 days. The overall incidence of events was 9%: 10 patients suffered reinfarction during follow-up, and 7 were readmitted for unstable angina, all of whom underwent PCI; no patient was admitted for HF; and none of the 8 patients with early VF had a new tachyarrhythmia, registering a single admission for VT during follow-up. None of the patients had sustained ventricular tachyarrhythmias during exercise-training. At the mean 4.5-year follow-up, 218 patients were still alive (98%). CONCLUSION The incidence of CV events in the follow-up of our cohort was low, which can be explained by the fact that it is a young population, with an LVEF at low limits of normality at discharge, which is one of the most important predictors in the prognosis after an ischemic event. As an improvement, we must shorten the time until the start of phase II of the program. CRH shows once again its clinical benefit after an ACS, in consonance with the existing evidence. Abstract Figure. Outcomes of a CRH program.

Background Anti-MDA5 antibodies are a known set of antibodies observed mainly in dermatomyositis, typically associated with cutaneous involvement, presence and often rapid progression (RP) of interstitial lung disease (ILD) and amyopathic muscle involvement. Despite the increased attention, described cohorts involves only a limited number of cases Objectives to define clinical characteristics of a large cohort of anti-MDA5 antibodies positive patients Methods Retrospective assessment of anti-MDA5 positive patients from centres referring to our group Results 82 anti-MDA5 positive cases (56 females) were collected. In median: onset age was 44 Y (IQR 22.57), diagnostic delay 4 Mo (IQR 1–10), follow-up 13 Mo (IQR 3–43). Fifty-three patients had ILD, that was RP in 24 cases (15 at ILD onset, 9 after ILD onset). Fifthteen ILD patients were admitted in Intensive Care Unit (ICU): 4 were treated with Extracorporeal-Membrane-Oxygenation, 7 with mechanical-invasive and 2 with mechanical-non-invasive ventilation. Forthy-nine patients had muscle involvement (39 symptomatic), 42 arthritis, 54 cutaneous involvement, 9 history of malignancy. Twelve patients died (ILD=7, ILD and sepsis=3, neoplasia=1, not specified=1), 7 of those in ICU Conclusions In our cohort, ILD was the most frequent finding. A RP of ILD was common, occurring also in ILD with symptomatic/chronic onset. The low rate of survival in ICU raised the problem of follow up and early treatment of ILD. In our cohort arthritis was common and muscle involvement mainly symptomatic. Finally, the high percentage of observed malignancies suggests a careful neoplastic screening and follow up Reference [1] Labrador-Horrillo M, et al. J Immunol Res2014;2014:290797. Disclosure of Interest None declared

Background Haemophagocytic syndrome is an increasingly-recognised condition in adults, characterised by a wide range of aetiologies, symptoms and outcomes, but with a common aetiopathogenic pathway leading to organ damage: an excessive hyperinflammatory response. Objectives To analyze the main epidemiological, clinical features, analytical parameters, therapies and outcomes of patients with haemophagocytic syndrome. Methods In June 2013, the Study Group on Autoimmune Diseases (GEAS) of the Spanish Society of Internal Medicine created the national registry of patients with haemophagocytic syndrome. Patients fulfilled the diagnostic guidelines proposed by the Histiocyte Society in 1991 and updated in 2004. Results On December 31, 2013, a total of 50 patients with haemophagocytic syndrome were included in the GEAS-SEMI Registry, 31 (62%) men and 19 (38%) women, with a mean age at diagnosis of disease of 49.16 years (range 12-84 years); 18% were not born in Spain. The main signs and symptoms at presentation were fever in 46 (92%) patients, splenomegaly in 34 (68%), hepatomegaly in 27 (54%) and polyadenopathies in 25 (50%) patients.The main organs involved were the lungs in 23 (46%), the kidneys in 15 (30%) and the CNS in 8 (16%). The main analytical markers consisted of severe anemia (mean 7.56 g/L), thrombocytopenia (mean 55436.74/mm 3 ), hypertriglyceridemia (mean 432.89 mg/dL); raised transaminases were found in 40 (80%) patients, raised LDH in 36/48 (75%), and hyponatremia in 30/47 (64%). All patients showed high ferritin serum levels (mean 20392.84, range 413-190544 ng/mL). Tissular hemophagocytosis was confimed in bone marrow smear in 36 (72%) cases, bone marrow biopsy in 10 (20%), liver biopsy in 8 (16%) and other tissues in two. The main associated diseases were infections in 29 (48%) cases, autoimmune/rheumatological in 8 (16%) and hematological neoplasia in 12 (24%). Vital support was required in 28 (56%) patients. Therapies included corticosteroids in 40 patients, etoposide in 12, cyclosporin A in 12, methotrexate in 4, tacrolimus in 2, rituximab in 3, IVIG in 2 and chemotherapy in 5. Death occurred in 26 (52%) patients; the mortality rates according to the baseline disease was 8/12 (67%) in patients with hematological neoplasia, and 16/29 (55%) in those with infectious disease and 1/8 (12%) in those with autoimmune/rheumatic disease. Conclusions The rate of mortality of adult patients diagnosed with haemophagocytic syndrome exceeds 50% of cases, especially in patients with underlying hematological diseases. An early diagnosis is essential in order to initiate appropriate therapy and improve survival of patients with this challenging condition. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.6017