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Background Pulmonary arterial hypertension (PAH) is a syndrome characterized by marked remodeling of the pulmonary vasculature and leading to right heart failure and death. Selexipag, an oral prostacyclin IP receptor agonist, has been shown to decrease morbidity and mortality compared to patients treated with placebo. Purpose Our aim was to evaluate the efficacy and safety of Selexipag in patients with PAH in real-life. Methods The RAMPHA study was a multicenter, observational and retrospective trial of patients who had PAH and began taking Selexipag between 2017–2021. We analyzed baseline characteristics, risk profiles, clinical assessments that were used for risk stratification and events in follow-up time. Results 29 patients aged 48±14 were initially studied. 23 (79%) were women. Within the pulmonary hypertension-classification, 10 (34,5%) had PAH associated-congenital heart disease, 9 (31%) had idiopathic HAP, 7 (24,1%) had PAH associated with connective tissue disease, 1 had PAH associated to HIV and 2 heritable PAH. The time from PAH diagnoses to the beginning of Selexipag treatment was 54 months (IQR 89). 89,7% (26) were in treatment with doubled combination therapy with PDE5i+ARE; Sildenafil was the most widely used PDE5i and bosentan (37,9%) the most used ARE. No patients were under treatment with intravenous prostacyclin analogue, but 3 were with Treprostinil (1 of them subcutaneous and 2 inhaled) and 2 patients with iloprost. Most patients were categorized in intermediate risk profile (figure 1), using the risk stratification strategy of ESC/ERS PH guidelines. In the approach of risk assessment before start with Selexipag, clinical, functional, exercise (with 6MWT) and echocardiographic variables were used in all the patients. Biochemical variables with NT-proBNP were used in 93% of the patients. Only 15 patients had a right catheterization to get haemodynamic parameters before the treatment. At follow up, 11 patients (38%) improved functional class, only 1 patient got worst (p=0,001). 3 patients improved risk-profile in the exercise test and, in the others, a quantitative improvement was found. NT-proBNP levels were not significant better (924ng/l IQR 1209 vs 760ng/l IQR 1397). There were not changes in RV function in the echocardiographic parameters. Selexipag was well-tolerated, 86% of the patients experienced side effects, but none had to discountinue the treatment. The most common side effect was headache. The titration lasted 68 days (IQR 72) and 38% of the patients got maximum doses. At the medium-term follow-up of 52 months, the free-event survival (worsening of PAH that resulted in hospitalization; initiation of parenteral prostanoid therapy or death to PAH; or any cause) was 80% (Figure 2). Conclusion Selexipag, added as triple combination therapy in patients with PAH intermediate risk, improved risk variables, was well tolerated and achieved a medium-long-term free-event survival greater than 80%. Funding Acknowledgement Type of funding sources: None.

Background:Despite great advances in the diagnosis and treatment of lupus, the scientific community does not know the perception of our patients regarding the knowledge of the disease and the relationship between patients and disease. Fatigue had the greatest impact on activities of daily living, yet the majority reported receiving no support or poor support in managing it1.Objectives:Through this initiative, it is intended to investigate about the knowledge of the disease and impact of the disease on quality of life of Lupus patients. Another objective is to give visibility to the current needs of people living with lupus.Methods:It was performed a national survey with 1,263 interviews with Lupus patients who reside in Spanish territory and belonging to lupus patient associations in Spain. The survey was carried out by the Spanish Lupus Federation (FELUPUS) in collaboration with GSK company.Online interview lasting approximately 25 minutes. The collection of information was anonymously carried out from May 21 to June 30, 2020.Results:1.263 lupus patients were interviewed, 92% diagnosed with SLE and 8% with CLE. Survey sample is representative of the Lupus patient population in Spain [associated sampling error: 2.76%]. Questions about knowledge of the disease showed that 73% of patients considered that there is very little knowledge of the disease by society. Patient awareness of lupus increases as the disease progresses, so at the time of diagnosis, level of knowledge of the patient about Lupus is low in 92% of patients and at the moment of survey, 68% of patient had high knowledge. In terms of the perception of his illness, the affectation of the kidneys and heart (97%), fatigue and skin rashes (97%), are the statements that generate the greatest consensus. The survey about the relationship to disease demonstrated that 3 out of 4 patients have symptoms related to the disease, muscle and joint pain (75%) and fatigue (74%) are the symptoms that cause the greatest discomfort (Graph 1). Remarkable degree of agreement with the fact of not being able to sunbathe (78%), as well as the lack of energy (61%) and weakness in the body (60%). Flare-ups (86%), followed by fatigue (78%) and pain (77%) cause great concern. At diagnosis, 92% of patients have some organic involvement and regarding the diagnosis, at present, a greater number of patients present damage to the CNS (17%) and bones (21%). Many patients do not understand the concept of organ damage, wrongly relating it to fatigue (38%) or joint pain (47%).Graph 1.Conclusion:Among the conclusions of the survey, it stands out that society and the general population are unaware of what lupus is, while in the case of lupus patients, knowledge increases as the disease progresses. Citizen awareness campaigns about this disease are necessary, where patient associations together with health authorities have a crucial job. On the other hand, 92% of patients present organ damage at diagnosis. This means that we are arriving late to the diagnosis of many patients, which makes it necessary to promote a close collaboration between Primary Care and Hospitals, to refer patients as soon as they suspect SLE.References:[1]Sloan M, Harwood R, Sutton S, D’Cruz D, Howard P, Wincup C, Brimicombe J, Gordon C. Medically explained symptoms: a mixed methods study of diagnostic, symptom and support experiences of patients with lupus and related systemic autoimmune diseases. Rheumatol Adv Pract. 2020 Feb 26;4(1):rkaa006.What worries the most to Lupus patients?Question P12. Please indicate your level of concern with the following aspects of Lupus. Percentage of patients who have scored a 4 or 5 for each item (% T2B).Acknowledgements:GSK funded the study presented in the abstract.Disclosure of Interests:Patricia Fanlo Grant/research support from: GSK funded the study presented in the abstract.

Sarcoidosis is a multisystemic disease which diagnosis depends on the presence of nonnecrotizing granulomas in the biopsy. However there are variants such as necrotizing sarcoidal granulomas or nodular sarcoidosis which have atypical findings and make difficult the differential diagnosis with other infectious processes. We describe a case of a man who develops granulomas with extensive necrosis in a systemic sarcoidosis that affected the lung and the central nervous system. This finding made us to make the diagnosis of tuberculosis and delay the specific treatment.