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BackgroundPericarditis is frequent in Systemic Lupus Erythematosus (SLE) and usually benign. Dedicated studies are scarce. However, recurrences can lead to repeated steroid prescriptions and further immunosuppression. The best management, including the potential benefit of colchicine, remains to be determined.ObjectivesThe aim of this study was to describe management, evolution over time and risk factor for relapse in SLE pericarditis in our University Hospital Center.MethodsCases were retrospectively collected among hospital discharge data (coding code “SLE” and “pericarditis”), from January 1997 to December 2019. All SLE cases met the ACR/EULAR 2019 classification criteria. Pericarditis cases met ESC 2015 diagnosis criteria. Patients with conditions other than lupus known to cause pericarditis were excluded as well as patients with myocarditis. A minimal follow-up of one year after pericarditis diagnosis was mandatory. Relapse- free survival was described using an actuarial survival model.ResultsAmong 103 patients identified in the database, 29 patients (women: n=25, mean age 30 +/- 13 years) were included. Median follow-up was 7 years [range: 1-22].Description of first episodes: 31% (n= 9) were inaugural of SLE; otherwise, median time elapsed since SLE diagnosis was 65 months [1.7-400]. Fifty-five percent (n=16) of first episodes occurred during a multi-systemic lupus flare. Median SLEDAI-2K was 9 [range: 4-30|. Clinical symptoms and signs were typical chest pain (93%, n=27), dyspnea (55%, n=16); pericardial rub (31%, n=9), fever (38%, n=11). EKG was abnormal in 59% of the cases (n= 17). When present, 79% of effusions (n=17/22) were circumferential, 82% (n= 18/22) were mild to moderate (Biological data showed always high CRP levels (65mg [range: 7-460]), high-titer anti-DNA (79%, n=19) but few patients had low complement levels (C3 21% (n=4/19), C4 26%(n=5/19)).Prescribed drugs were non-steroidal anti-inflammatory drugs/acetylsalicylic acid (NSAIDs/ASA) (41%, n=12), corticosteroids (66%, n=19; mean daily prednisone dose: 57.2mg +/- 13.9), colchicine 0.5 to 1mg/day (41%; n=12). There was a significant difference in SLEDAI-2K values at pericarditis onset between those treated with NSAIDs/ASA (7.5, [range: 0-16]) and those not (12, [range: 4-30]), (pRecurrences were frequent (55%, 16 patients out of 29) and multiple (1 to 6, average 3 ± 1.26). Short and long-term relapse-free survival tended to be better in patients exposed to at least 3 months of colchicine (100% vs 75% at 1 year, p=0.09) (Figure 1). There was no statistical difference (p=0.25) in terms of short-term relapse-free survival in patients treated with NDAIDs/ASA compared to those who were not. Corticosteroid prescription and previous antimalarial treatment were not associated with a poorer or better outcome during the year following remission (p=0.78). No patient has progressed to constriction.Figure 1.Relapse-free survival at 12 months according to colchicine prescriptionConclusionOur conclusions are limited due to the small number of patients and lack of multivariate analysis.Further studies are necessary to confirm the potential benefit of colchicine to prevent incessant pericarditis or relapses in this population.References[1]Kruzliak P, et al. Acta Cardiol 2013;68:629–33.Disclosure of InterestsNone declared

BackgroundRituximab (RTX) induces rapid, usually complete and prolonged depletion of circulating B cells, and also hypogammaglobulinemia in some patients. There are limited data regarding the risk of severe infection events (SIE) when initiating or continuing rituximab in patients with acquired hypogammaglobulinemia, especially in patients suffering from autoimmune diseases (ADs) other than rheumatoid arthritis (RA) (1).ObjectivesTo describe the risk of severe infectious events (SIE) following initiation (rituximab-naïve patients [RNP]) or continuation of RTX therapy (rituximab-continuing patients [RCP]) in patients suffering from severe ADs other than RA and acquired hypogammaglobulinemia.MethodsWe conducted a single-center retrospective cohort study at the University Hospital of Toulouse (France) between 2010 and 2018. Patients were included if they had received at least one dose of RTX in the year following the evidence of hypogammaglobulinemia (defined as gammaglobulins [GG]≤ 6g/L on serum protein electrophoresis) in the setting of ADs other than RA. The primary outcome was the occurrence of a SIE within 2 years after the date of first RTX infusion (T0) prescribed after the evidence of hypogammaglobulinemia. SIE were infections either fatal or requiring hospitalization.ResultsWe included 121 patients (37 RNP and 84 RCP): 48 had ANCA-associated vasculitis (AAV), 48 multiple sclerosis (MS, n=41) or neuromyelitis optica (NMO, n=7), and 21 another severe AD. RTX was prescribed as induction therapy in 39 patients and as maintenance therapy in 82; 112/121 patients were followed for 2 years. Mean GG level were 5.5 g/L (IQ25-75 4.6-5.7) at T0, 5.5 g/L (IQ25-75 5-6.4) at one year, 5.7g/L (IQ25-75 4.8-6.1) at two years and 8 patients had a decrease of their GG level below 4g/L. Ten patients received immunoglobulin replacement therapy (IGRT) mostly after infection (n=6).Twenty-six patients (23.2%) had at least one SIE during follow-up: 12.8 % in the MS/NMO group with a 2-year incidence at 6.9 (3.1-15.3) per 100 person-years, 29.5 % in the AAV group with a 2-year incidence at 18.3 (9.3-20.1) per 100 person-years, 33.3 % in the ‘other ADs’ group with a 2-year incidence at 22.2 (10.6-46.5) per 100 person-years. Infection was opportunistic in 8 patients (33.3%) and 4 died from SIE.Risk factors of SIE at T0 were male gender (61.5% vs. 39.5% pConclusionOur study provides useful information for clinicians considering initiating or continuing rituximab therapy in patients with acquired hypogammaglobulinemia before Sars-Cov2 era. Prospective studies are necessary to improve the knowledge on outcome of patients treated by rituximab despite low immunoglobulins levels. Prophylactic IGRT may be appropriate in higher risk patients, especially if the GG level is below 4 G/L.References[1]Boleto G et al. Predictors of hypogammaglobulinemia during rituximab maintenance therapy in rheumatoid arthritis: A 12-year longitudinal multi-center study. Seminars in Arthritis and Rheumatism. oct 2018;48(2):149‑54.Figure 1.Two years probability of survival without serious infectious event according to pathology group (112 patients included)Disclosure of InterestsNone declared

Background:Ear chondritis is often considered the pathognomonic feature of relapsing polychondritis (RP). Although painful redness and swelling of the pinna and a resultant cauliflower ear are universally recognized as chondritis, the complete spectrum of symptoms associated with ear chondritis have not been well described.Objectives:The study objective was to seek patient input to help characterize ear chondritis An online survey was administered in English or Spanish to participants with self-reported RP.Methods:Participants were asked questions about their ear pain, including quality, location, duration, aggravating/alleviating factors, timing of onset and duration. Participants were included who reported age ≥ 18 years, a diagnosis of RP confirmed by a physician, and sufficient symptoms to meet McAdams or Damiani’s diagnostic criteria. Participants were categorized as having “typical ear chondritis” if they reported ear pain localized to the pinna with associated redness and swelling. Atypical presentations of ear chondritis were also considered.Results:A total of 685 participants from five continents completed the survey. Among them, 659 met inclusion criteria for subsequent analysis. Most participants were female (n=574; 87%), white (n=548; 83%) and from the United States (n=484;74%). The median age was 50 years (interquartile range = 41-58). In total, 593 (90%) patients reported ear pain, 227 (38%) had “typical ear chondritis”, and 98 (16%) had cauliflower ear.Ear pain was most commonly described as burning (n=334, 56%) or throbbing (n=295, 50%). The most common location of pain was the pinna (n=373, 63%). Participants reported ear redness (n=454, 60%) and swelling (n=405, 62%). Some patients experienced only ear redness without swelling (n=286, 48%) or only ear swelling without redness (n=71, 12%). The most common aggravating factors were minor trauma (n=371, 62%) and stress (n=358, 60%). The most common alleviating factor was avoidance of touching the ear (n=374, 63%). Pain was most frequently reported during the daytime (n=355, 60%) and most likely to occur in either ear at different times (n=310, 52%). Onset could be gradual (n=198, 33%) or sudden (n=155, 26%). Pain typically lasted a few hours (n=175, 30%) or 2-3 days (n=130, 22%). The majority of patients who had pinna pain also had pain in other parts of the ear (e.g. mastoid process, inner ear, whole ear) at some point (n=394, 67%). In patients with cauliflower ear, the most common location of pain was the pinna (n=57, 58%) followed by pain inside the ear (n=53, 54%). Most participants reported at least two different types of pain (n=420, 64%).Conclusion:Ear chondritis in patients with RP has a wide range of clinical presentations and characteristics beyond the typical triad of redness, swelling, and pain localized to the pinna. The description of pain often significantly varies within the same patient. Knowledge of the various distinct characteristics of ear involvement in RP may help physicians recognize and monitor the disease more effectively.Disclosure of Interests:None declared