Background: Raynaud's phenomenon of the tongue is a rare manifestation that may be associated with systemic diseases. The clinical manifestations, etiologies and management of this condition are poorly described. Methods: We report 10 cases of lingual Raynaud's phenomenon (LRP) and 26 cases from a structured literature review. Results: In 38.8% of cases, the LRP occurred in the context of a previously diagnosed systemic sclerosis; 16.6% followed radiotherapy for head and neck cancer; and 27.8% of patients presented with an idiopathic-like form. The manifestations classically included a syncopal phase (91.7%) associated with hypoesthesia (88.9%) and possible dysarthria (52.8%). Atypical presentations with a primary cyanotic phase were also observed, particularly in the context of vasculitis, notably cryoglobulinemic vasculitis (four patients). Active smoking was a significant triggering factor in idiopathic forms (60%). Across all patients-both primary and secondary forms-the most common triggering factor was cold exposure (75%). Vasodilator use showed good efficacy and should be considered for all highly symptomatic patients. Conclusions: In summary, LRP is more frequently associated with systemic sclerosis, manifesting as blanching of the tongue associated with hypoesthesia and dysarthria in more than half of cases. Vasodilators may reduce symptoms. Larger studies are needed to confirm these findings.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. Objectives: We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management. Methods: This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification [1] and LVI as defined by the Chapel Hill nomenclature [2], together with controls matched for age, sex, and AAV type. Results: We included 26 patients, 15 (58%) of whom were men, with a mean age of 56.0 ±17.1 years. The patients had granulomatosis with polyangiitis (n=20), or microscopic polyangiitis (n=6). LVI was inaugural in 18 of 26 patients, but occurred after the diagnosis of AAV in the remaining eight patients. The affected vessels included the aorta (n=18; 69%) supra-aortic trunks (n=9; 35%), lower-limb arteries (n=5; 19%), mesenteric arteries (n=5; 19%), renal arteries (n=4; 15%), and upper-limb arteries (n=2; 8%).The diagnosis of LVI was incidental in 15 patients (58%), occurring during imaging for a flare-up of AAV. In patients with symptoms (n=11), pain was the most common symptom (n=10), particularly back or abdominal pain for aortic injury (n=6), neck pain for carotid injury (n=1), inguinal pain for iliac injury (n=1), and abdominal pain for mesenteric artery injury with ischemia (n=2). Imaging showed wall thickening (n=10; 38%), perivascular inflammation (n=8; 31%), aneurysms (n=5; 19%), and stenosis (n=4; 15%).Comparisons with the control group (52 patients) revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95% CI: 1.11-10.01, p=0.03]), but not with cardiovascular risk factors (OR=0.70 [95% CI: 0.23-2.21, p=0.60]), or AAV relapse (OR=2.01 [95% CI: 0.70-5.88, p=0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92%), mostly cyclophosphamide (n=10, 38%) or rituximab (n=9, 35%). Conclusion: Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI. References: [1] Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022 Mar;81(3):315–20.[2] Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1–11. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background: Systemic lupus erythematosus (SLE) is characterized by a difficult-to-predict fluctuation of disease activity which makes it difficult for patients to promptly identify a flare. Furthermore, SLE is characterized by an impaired of quality of life with a need to better evaluate and improve patient-reported outcomes (PROs). Therefore, patient-centered tools to self-evaluate disease activity and disease burden are a necessary step toward self-empowerment of SLE patient. Objectives: To develop and to evaluate the characteristics of a self-administered questionnaire (LUPIN) to assess SLE disease activity and lupus-relevant PROs. Methods: Patient representatives from a national lupus association (AFL+) and lupus experts developed a self-administered questionnaire which included several domains of SLE perceived activity (Figure 1) and the SF-36 quality of life questionnaire. Prior to a medical consultation, the patient filled the questionnaire which was sealed. Subsequently, the physician evaluated the patient (blindly of the patient responses) and filled patient characteristics, a physician global assessment (PhGA) and a SLE disease activity Index 2K (SLEDAI-2K). The questionnaires were distributed nationally to 31 centers of metropolitan France and overseas territories. Correlations between patient response in individual domains and physician assessment were evaluated using Spearman’s regression and the p-values were adjusted for multiple testing using the Bonferroni method. Results: 325 questionnaires were analyzed at the time of the submission. The mean age was 44 (±14.4) years and 85,2% were women. The mean duration of disease was 12.7 (±9.42) years. Most patients had history of articular and cutaneous involvements (86.6% and 72.6%, respectively) and 34.4% had history of lupus nephritis. The mean SLEDAI was 3 (±3.70) and 67 (20.6%) patients had a clinical SLEDAI ≥ 4. The PhGA correlated moderately with the cSLEDAI (r = 0.52). As expected, there was a weak albeit statistically significant correlation between components of the LUPIN questionnaire and the SLEDAI/clinical SLEDAI and PhGA (r < 0.25 for all; Figure 2). However, there were very good correlations between several components of the LUPIN questionnaire and several of the SF-36 questionnaire domains (r = -0.68 for pain evaluation; r = -0.65 for physical activity evaluation; r = -0.60 for fatigue evaluation; p < 0.0001 for all). Conclusion: Several components of the LUPIN questionnaires have significant correlation with domains of the SF-36 quality of life questionnaire making LUPIN a patient-friendly tool to evaluate patient-reported outcomes now that it has been implemented on smartphones. As expected in a cross-sectional study, there were only weak correlations between domains of the LUPIN questionnaire and physician-assessed disease activity. However, the changes in LUPIN scores assessed prospectively may be more sensitive to detect disease activity fluctuation, which is currently evaluated in a follow-up study. REFERENCES: NIL. Acknowledgements: All patients and physicians who participated in the study. The AFL+ patient association represented by her president Marianne Riviere. Disclosure of Interests: Marc SCHERLINGER Amgen, AstraZeneca, Biogen, BMS, Fresenius Kabi, Galapagos, GSK, Nordic Pharma, Novartis, Sandoz., Jean-François KLEINMANN: None declared, Antonin FOLLIASSON: None declared, Marianne RIVIERE: None declared, Raphaëlle Rybak: None declared, Sabine Malivoir: None declared, Jean-François Viallard: None declared, Frédéric RENOU: None declared, Estibaliz Lazaro: None declared, Christophe Richez: None declared, Pascal Roblot: None declared, Mathieu Puyade: None declared, Clara Baverez: None declared, Arnaud Hot: None declared, Christophe Deligny: None declared, Nicolas Baillet: None declared, Daniel Wendling: None declared, Julien Campagne: None declared, Christian Agard: None declared, Roland Jaussaud: None declared, Thomas MOULINET: None declared, Denis WAHL: None declared, Gilles Blaison: None declared, Elisabeth Diot: None declared, Nicole Ferreira-Maldent: None declared, Isabelle Marie: None declared, Nicolas Girszyn: None declared, Laurent Perard: None declared, Marc André: None declared, Pauline Orquevaux: None declared, amelie servettaz: None declared, François Chasset: None declared, Baptiste HERVIER: None declared, Thierry Martin: None declared, Cécile Fermont: None declared, Emmanuelle David: None declared, LOIC RAFFRAY: None declared, Ludovic Trefond: None declared, Perrine Smets: None declared, Julie Lescanff: None declared, Jacques-Eric Gottenberg: None declared, Xavier Mariette: None declared, Zahir AMOURA: None declared, Jean SIBILIA: None declared.Figure 1The LUPIN questionnaire (paper or electronic form). Figure 2Spearman’s correlation matrix between components of the LUPIN questionnaire, the SF-36 questionnaire and the physician’s assessment of disease activity. Each value corresponds to the spearman r value, in bold when there is a statistically significant correlation after adjusting p-value for multiple testing using the Bonferroni method.

Introduction Le lupus érythémateux systémique (LES) affecte principalement les femmes en âge de procréer et est associé à un risque accru de poussées au cours de la grossesse et de complications obstétricales maternelles et fœtales [1]. Les hormones stéroïdes féminines jouent un rôle important dans sa physiopathologie en modulant la réponse immunitaire [2].La fertilité des patientes lupiques peut être altérée, le plus souvent de façon indépendante du lupus, avec nécessité d’utiliser des hormones sexuelles exogènes. Nous avons évalué le devenir des grossesses obtenues après utilisation de ces traitements en comparaison aux grossesses spontanées chez les patientes lupiques incluses dans l’étude prospective du GR2 (Groupe de Recherche sur la Grossesse et les Maladies Rares, NCT02450396). Patients et méthodes Nous avons analysé toutes les grossesses chez des femmes atteintes de LES (selon les critères SLICC 2012) incluses dans l’étude GR2 depuis 2014, conçues avant le 01/08/2022, avec des données de fin de grossesse disponibles et dont le mode de conception (naturel ou assisté) était connu. Les grossesses assistées étaient obtenues par induction de l’ovulation (IO), insémination intra-utérine (IUI) après stimulation ovarienne ou par fécondation vitro (FIV), avec ou sans injection intracytoplasmique de spermatozoïde (ICSI). L’activité de la maladie était évaluée à l’aide du SLE Disease Activity Index-2000 (SLEDAI-2K) adapté à la grossesse (SLEPDAI) [1]. Les séquelles étaient définies selon le SLICC-Damage index [1]. Les poussées étaient définies selon le Selena Flare index (SFI) [1]. Les complications obstétricales étaient définies par le score composite suivant : mort fœtale in utero inexpliquée à partir de 12 semaines d’aménorrhée (SA), prématurité (< 37 SA) liée à une insuffisance placentaire, petit poids pour l’âge gestationnel (PPAG) (≤ 3e percentile) et/ou mort néonatale [1]. Les taux de naissances vivantes et les incidences de poussées de la maladie et de complications obstétricales des grossesses assistées étaient comparées aux grossesses naturelles. Les grossesses par FIV/ICSI étaient évaluées séparément et comparées au même groupe contrôle. Résultats 630 grossesses ont été analysées, dont 53 assistées et 577 naturelles chez respectivement 48 et 478 femmes. Deux grossesses assistées (3,8 %) ont été obtenues par IO, 12 (22,6 %) par IUI, et 39 (73,6 %) par FIV/ICSI. Un syndrome des antiphospholipides associé était présent dans 3 (5,7 %) grossesses assistées (vs n=71, 12,3 %, p=0,18). À l’inclusion, l’âge gestationnel médian était de 9 SA pour les deux groupes, tandis que l’âge moyen était plus élevé (35,8 ans vs 32,3 ans, p<1,10-4) et les grossesses gémellaires plus fréquentes (5/50, 10,0 % vs 20/554, 4,5 % ; p=0,047) pour les grossesses assistées. Les caractéristiques de la maladie lupique n’étaient pas significativement différentes entre les deux groupes. Le LES était cliniquement inactif dans 51 (96,2 %) grossesses assistées (vs 511/570, 89,6 % ; p=0,15), la majorité (35/45, 77,8 %) n’avait pas de séquelles (vs 448/513, 87,3 % ; p=0,07) et presque toutes (n=52, 98,1 %) étaient sous hydroxychloroquine (vs n=561, 97,2 % ; p=1,0). Le taux de naissances vivantes était similaire entre les grossesses assistées et naturelles (n=46, 86,8 % vs n=505, 87,5 % ; p=0,83). Au moins une poussée est survenue au cours de 9 (17 %) grossesses assistées (vs 96/565, 17 % ; p=0,74), la plupart cutanéomuqueuses et articulaires. Deux (3,8 %) poussées sévères sont survenues dans ce groupe (vs 21/565, 3,7 % ; p=0,99). Parmi les grossesses évolutives au-delà de 12 SA, au moins une complication obstétricale est survenue dans 8 grossesses assistées sur 48 (16,7 %) (vs 86/525, 16,4 % ; p=0,98). Les incidences cumulées des poussées et des complications obstétricales ne différaient pas entre les deux groupes. Les résultats étaient similaires pour les analyses limitées aux grossesses obtenues par FIV/ICSI. Aucun évènement thrombotique n’est survenu parmi les grossesses assistées. Conclusion Chez des patientes ayant globalement un lupus stable ou inactif, le recours aux traitements de fertilité n’augmentait pas significativement le risque de complications en comparaison aux grossesses spontanées. Ces résultats fournissent des données rassurantes et soutiennent les recommandations actuelles visant à autoriser l’utilisation de ces traitements chez les femmes lupiques infertiles sous réserve de conditions optimales de conception [2].

Objectives: Heart involvement is one of the leading causes of death in SSc. The prevalence of SSc-related cardiac involvement is poorly known. Our objective was to investigate the prevalence and prognosis burden of different heart diseases in a nationwide cohort of patients with SSc.Methods: We used data from a multicentric prospective study using the French SSc national database. Focusing on SSc-related cardiac involvement, we aimed to determine its incidence and risk factors.Results: Of the 3528 patients with SSc, 312 (10.9%) had SSc-related cardiac involvement at baseline. They tended to have a diffuse SSc subtype more frequently and to have more severe clinical features, and presented more cardiovascular risk factors. From the 1646 patients available for follow-up analysis, SSc-related cardiac involvement was associated with an increased risk of death. There was no significant difference in overall survival between SSc-related cardiac involvement, ischaemic heart disease or pulmonary arterial hypertension. Regarding survival analysis, 98 patients developed SSc-related cardiac involvement at 5 years (5-year event rate 11.15%). Regarding reduced left ventricular ejection fraction <50% and left ventricular diastolic dysfunction, the 5-year event rate was 2.49% and 5.84%, respectively. Pericarditis cumulative incidence at 5 years was 3%. Diffuse SSc subtype was a risk factor for SSc-related cardiac involvement and pericarditis. Female sex was associated with less left ventricular diastolic dysfunction incidence.Conclusions: Our results describe the incidence and prognostic burden of SSc-related cardiac involvement at a large scale, with gender and diffuse SSc subtype as risk factors. Further analyses should assess the potential impact of treatment on these various cardiac outcomes.

Background and aimsSystemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments.MethodsWe used data from a national multicentric prospective study using the French SSc national database. We estimated the average treatment effect (ATE) of sildenafil, bosentan, angiotensin-converting enzyme (ACE) inhibitors and iloprost on diastolic dysfunction, altered ejection fraction <50% and pulmonary arterial hypertension (PAH) using a causal method, namely the longitudinal targeted minimum loss-based estimation, to adjust for confounding and informative censoring.ResultsWe included 1048 patients with available data regarding treatment. Regarding sildenafil analyses, the ATE on diastolic dysfunction at 3 years was −2.83% (95% CI −4.06; −1.60, p<0.00001), and the estimated ATE on altered ejection fraction <50% was −0.88% (95% CI −1.70; −0.05, p=0.037). We did not find a significative effect on PAH. Regarding bosentan, ACE inhibitors and iloprost, none of them neither showed a significant effect on diastolic dysfunction, altered ejection fraction <50% or PAH.ConclusionsUsing causal methods, our study is the first and largest suggesting that sildenafil might have benefits among SSc patients regarding diastolic dysfunction and altered ejection fraction occurrence. However, further studies assessing the effect of vasodilators on heart-related outcome among SSc patients are needed to confirm those exploratory results.

Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice. Patients are informed about routine checkups, treatment side effects, and the need for regular vaccinations, especially if they are receiving immunosuppressive treatment. They are also advised on lifestyle factors such as the risks of smoking, sun exposure, and dietary adjustments, especially when they are receiving corticosteroids. The importance of contraception, particularly when teratogenic medications are being used, and regular cancer screening are emphasized. Support networks can help relieve a patient's isolation. The first-line medical treatment of SLE is hydroxychloroquine (HCQ), possibly combined with an immunosuppressant and/or low-dose corticosteroid therapy. The treatment of flares depends on their severity, and typically involves HCQ and NSAIDs, but may be escalated to corticosteroid therapy with immunosuppressants or biologic therapies in moderate to severe cases. Because there is no curative treatment, the goals of therapy are patient comfort, preventing progression and flares, and preserving overall long-term health and fertility. The frequency of follow-up visits depends on disease severity and any new symptoms. Regular specialized assessments are necessary, especially when treatment changes, but a frequency of every 3 to 6 months is recommended during periods of remission and monthly during active or severe disease, especially in children. These assessments include both clinical and laboratory tests to monitor complications and disease activity, with specific attention to proteinuria.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management. Methods: This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification and LVI as defined by the Chapel Hill nomenclature, together with controls matched for age, sex, and AAV type. Results: We included 26 patients, 15 (58 %) of whom were men, with a mean age of 56.0 +/- 17.1 years. The patients had granulomatosis with polyangiitis (n = 20), or microscopic polyangiitis (n = 6). The affected vessels included the aorta (n = 18; 69 %) supra-aortic trunks (n = 9; 35 %), lower-limb arteries (n = 5; 19 %), mesenteric arteries (n = 5; 19 %), renal arteries (n = 4; 15 %), and upper-limb arteries (n = 2; 8 %). Imaging showed wall thickening (n = 10; 38 %), perivascular inflammation (n = 8; 31 %), aneurysms (n = 5; 19 %), and stenosis (n = 4; 15 %). Comparisons with the control group revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95 % CI: 1.11-10.01, p = 0.03]), but not with cardiovascular risk factors (OR=0.70 [95 % CI: 0.23-2.21, p = 0.60]), or AAV relapse (OR=2.01 [95 % CI: 0.70-5.88, p = 0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92 %), mostly cyclophosphamide (n = 10, 38 %) or rituximab (n = 9, 35 %). Conclusion: Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI.

Introduction & objectives This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren's Disease (SjD), and patients with sicca manifestations from other causes. Methods Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc). Results SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure. Conclusion SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets.

Objective: High-resolution computed tomography (HRCT) may lack sensitivity for the early detection of interstitial lung disease associated with systemic sclerosis (SSc-ILD). Lung ultrasound is an emerging technique for the diagnosis of SSc-ILD. This cross-sectional study aimed to describe the prevalence of ultrasound interstitial syndrome in SSc patients with normal HRCT and pulmonary function tests (PFT). Methods: Thirty SSc patients with normal HRCT, FVC > 80% predicted and DLCO > 70% predicted were included. Echocardiography and PFT including impulse oscillometry and cardiopulmonary exercise testing were performed. Lung ultrasound was analyzed by two blinded operators. Patients were classified into two groups, according to the presence or absence of ultrasound interstitial syndrome, defined as the sum of B-lines in all thoracic areas ≥10 and/or pleural line thickness >3 mm on at least one thoracic area and/or a pleural line irregularity score >16%. Results: Ultrasound interstitial syndrome was present in 12 patients (40%). Inter-reader agreement for the diagnosis of ultrasound interstitial syndrome defined by the Kappa coefficient was 0.93 (95%CI 0.79–1.00). Patients with ultrasound interstitial syndrome were younger (37 years vs. 53 years, p = 0.009), more often had pitting scars (n = 7/12 vs. 3/18, p = 0.045) and had lower FVC (102 vs. 110% pred, p = 0.009), TLC (114 vs. 122% pred, p = 0.042) and low-frequency respiratory system reactance (Xrs5 Z-score 0.16 vs. 1.02, p = 0.018), while pulmonary gas exchange was similar. Conclusions: Ultrasound interstitial syndrome was detected in 12/30 SSc patients with normal HRCT and PFT. Patients with ultrasound interstitial syndrome had differences in lung function consistent with reduced respiratory compliance, suggesting minimal and/or early suspected SSc-ILD.

Peu d’études ont évalué l’ouverture buccale (OB) dans la sclérodermie systémique (ScS). Aucune n’a étudié les trajectoires des OB. L’objectif de l’étude rapportée ici était de caractériser les trajectoires d’OB dans la ScS et d’évaluer l’OB en tant que facteur pronostic dans la ScS. Nous avons réalisé une étude multicentrique rétrospective des patients inclus dans la cohorte nationale Française de ScS ayant au moins une mesure d’OB. Nous avons décrit les caractéristiques à l’inclusion des patients selon leur mesure d’OB à l’inclusion, modélisé les trajectoires d’OB, et évaluer l’intérêt pronostic de l’OB dans la ScS. Nous avons inclus 1101 patients. L’OB à l’inclusion était associée à la gravité de la maladie. De plus, après analyse par courbes de survies de Kaplan-Meier, une OB à l’inclusion inférieure à 30 mm était associée à une survie à 30 ans plus mauvaise (p < 0,01) et à un risque plus important d’hypertension artérielle pulmonaire (p < 0,05). L’analyse longitudinale a montré que les trajectoires individuelles d’OB étaient hétérogènes entre les patients. Le meilleur modèle de trajectoires d’OB obtenu selon une modélisation mixte à processus latents a montré que 88,8 % des patients avaient une trajectoire d’OB stable et que les patients pouvaient être classés en 3 clusters. Ces trois clusters étaient prédictifs de la survie à 30 ans (p < 0,05) et de l’apparition d’une pneumopathie intersitielle diffuse (PID) (p < 0,05). Enfin, le modèle a identifié un groupe de 9,5 % de patients, caractérisés par une ScS cutanée diffuse (dcScS) (p < 0,05) et une OB à l’inclusion élevée (p < 0,05) mais décroissante sur 1 an qui présentent un sur-risque de PID (p < 0,0001) et de décès (p < 0,0001). L’OB, qui est une mesure simple et fiable, pourrait être utilisée pour prédire la gravité de la maladie et la survie dans la ScS. Bien que la mesure d’OB soit stable chez la plupart des patients atteints de ScS, les patients atteints de dcScS avec une OB élevée mais décroissante en un an présentent un risque de PID et de mortalité plus important.

BackgroundBenralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series. MethodsWe conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab. The primary endpoint was the rate of complete response defined as no disease activity (Birmingham Vasculitis Activity Score=0) and a prednisone dose & LE;4 mg/day. Partial response was defined as no disease activity and a prednisone dose & GE;4 mg/day. ResultsSixty-eight patients were included, including 31 (46%) who had previously received mepolizumab. The use of benralizumab was warranted by uncontrolled asthma in 54 (81%), persistent ear, nose and throat (ENT) manifestations in 27 (40%) and persistent glucocorticoids (GCs) use in 48 (74%) patients. Median (IQR) follow-up after starting benralizumab was 23 (9-34) months. Thirty-three patients (49%) achieved a complete response, 24 (36%) achieved a partial response and 10 (15%) did not respond. Among the 57 patients who initially responded, 10 (18%) eventually required further line treatments. GCs were discontinued in 23 patients (38%). Prior mepolizumab use was associated with a higher rate of primary failure (26.7% vs 5.4%, p=0.034) and less frequent GCs discontinuation (14.8% vs 55.9%, p=0.001). Vasculitis flares occurred in 7 patients (11%) and were associated with histological evidence of vasculitis and/or antineutrophil cytoplasmic antibodies positivity at benralizumab initiation (p=0.004). ConclusionsBenralizumab appears to be an effective treatment for refractory asthma or ENT manifestations in EGPA and allows GC-sparing. However, its efficacy was lower after prior failure of mepolizumab.

Few studies have evaluated mouth opening (MO) in systemic sclerosis (SSc). None have studied MO trajectories. To study MO trajectories in SSc. This multicentre study included patients enrolled in the French national SSc cohort with at least one MO assessment, described patients based on MO baseline measure, modelled MO trajectories and associated MO measures with SSc prognosis. We included 1101 patients. Baseline MO was associated with disease severity. On Kaplan–Meier analysis, MO <30 mm was associated with worse 30-year-survival ( p  < 0.01) and risk of pulmonary arterial hypertension ( p  < 0.05). Individual MO trajectories were heterogenous among patients. The best model of MO trajectories according to latent-process mixed modelling showed that 88.8% patients had a stable MO trajectory and clustered patients into three groups that predicted SSc survival ( p  < 0.05) and interstitial lung disease (ILD) occurrence ( p  < 0.05). The model highlighted a cluster of 9.5% patients with diffuse cutaneous SSc (dcSSc) ( p  < 0.05) and high but decreasing MO over 1 year ( p  < 0.0001) who were at increased risk of poor survival and ILD. MO, which is a simple and reliable measure, could be used to predict disease severity and survival in SSc. Although MO remained stable in most SSc patients, dcSSc patients with high but decreasing MO were at risk of poor survival and ILD.

Background Heart involvement is one of the leading causes of death in systemic sclerosis (SSc). Cardiac manifestations are heterogeneous. In particular, prevalence of SSc-related cardiopathy is poorly known due to a lack of consensus in its definition. Objectives Our objective was to investigate the prevalence and prognosis burden of the different heart diseases in a nationwide cohort of patients with SSc. Methods We used data from a national multicentric prospective study using the French SSc national database. We described the characteristics of 3 different types of heart involvement: SSc-related cardiopathy, pulmonary arterial hypertension and ischaemic heart disease. We analyzed overall survival and survival according to the heart disease. Focusing on SSc-related cardiopathy, we aimed to determine its incidence and risk factors. Results Over the 3528 patients with SSc available for baseline analyses 312 (10.9%) had SSc-related cardiopathy at baseline. They tended to have a diffuse SSc subtype more frequently, had more severe clinical features, and presented more cardiovascular risk factors. From the 1646 patients available for follow-up analysis, SSc-related cardiopathy was associated with an increased risk of death. No significant difference of overall survival was found between SSc-related cardiopathy, ischaemic heart disease or pulmonary arterial hypertension. Concerning survival analysis, 98 patients developed SSc-related cardiopathy at 5 years (5-year event-rate: 11.15% [9.01; 13.23]). Regarding reduced LVEF < 50% and left ventricular diastolic dysfunction, the 5-year event-rate were 2.49% [CI95%: 1.13; 3.83] and 5.84% [CI95%: 4.02; 7.62], respectively. The pericarditis cumulative incidence at 5 years was 3% [CI95%: 1.91; 4.08]). Diffuse SSc subtype was a risk factor of SSc-related cardiopathy and pericarditis (adjusted HR: 1.42 [CI95%: 1.02; 1.97], p = 0.04 and adjusted HR: 1.79 [CI95%: 1.06; 3.02], p = 0.03, respectively). Female sex was associated with less diastolic dysfunction incidence (adjusted HR: 0.39 [CI95%: 0.24; 0.63], p = 0.0001). Conclusion Our results further describe at a large scale the incidence and prognostic burden of SSc-related cardiopathy, with gender and diffuse SSc subtype as risk factors. Further analyses should assess the potential impact of treatment on these various cardiac outcomes. References [1]Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 2017;76:1897–905. https://doi.org/10.1136/annrheumdis-2017-211448. [2]Bulkley BH, Ridolfi RL, Salyer WR, Hutchins GM. Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction. Circulation 1976;53:483–90. https://doi.org/10.1161/01.cir.53.3.483. [3]Hoogen F van den, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative. Arthritis & Rheumatism 2013;65:2737–47. https://doi.org/10.1002/art.38098. Acknowledgements: NIL. Disclosure of Interests Alexis F. Guedon: None declared, Fabrice Carrat: None declared, Luc Mouthon: None declared, David Launay: None declared, Benjamin Chaigne: None declared, Gregory Pugnet: None declared, Jean-Christophe Lega: None declared, Arnaud Hot: None declared, Robin Dhote: None declared, Thomas Papo: None declared, Emmanuel Chatelus: None declared, Bernard Bonnotte: None declared, Jean-Emmanuel Kahn: None declared, Elisabeth Diot Speakers bureau: ED received fees as speaker in symposium from Boehringer Ingelheim., Boris Bienvenu: None declared, Nadine Magy-Bertrand: None declared, Viviane Queyrel: None declared, Alain Le Quellec: None declared, Pierre Kieffer: None declared, Zahir Amoura: None declared, Jean-Robert Harlé: None declared, Vincent Cottin: None declared, Jean-Baptiste Gaultier: None declared, Marie-Hélène Balquet: None declared, DENIS WAHL: None declared, Olivier Lidove Grant/research support from: OL received several fees for congress travels and experts’ use from Amicus Therapeutics, Sanofi-Genzyme, and Takeda, Anne-Laure Fauchais: None declared, Yannick Allanore: None declared, Patrick Jégo: None declared, Christian Agard: None declared, olivier fain: None declared, Arsene Mekinian Grant/research support from: AM is investigator of CELGENE, ROCHE, CHUGAI founded trials with APHP and Hopital 15-20 promotion; AM received several fees for congress travels and experts’ use from LFB, SANOFI, SHIRE, and CELGENE, Eric Hachulla: None declared, Sebastien RIVIERE: None declared.

La pyélonéphrite xanthogranulomateuse est une pyélonéphrite chronique caractérisée par une réaction granulomateuse inflammatoire destructrice du parenchyme rénal. C’est une entité rare dont l’atteinte peut être diffuse avec une extension vers les organes de voisinage, notamment vers la peau.Un patient âgé de 73 ans, présentait depuis trois ans des nodules fistulisés et douloureux de la paroi abdominale. La tomodensitométrie et l’imagerie par résonance magnétique abdominale concluaient au diagnostic d’une pyélonéphrite xanthogranulomateuse avec extension à la peau, au côlon et au muscle psoas. Une double antibiothérapie permettait une amélioration des lésions cutanées. Une néphrectomie totale gauche était proposée au patient, mais refusée par ce dernier qui a été perdu de vue par la suite.Nous rapportons une observation originale de pyélonéphrite xanthogranulomateuse révélée par des nodules cutanés de la paroi abdominale avec une extension vers la peau, le côlon et le muscle psoas.Xanthogranulomatous pyelonephritis is a chronic pyelonephritis characterized by an inflammatory granulomatous reaction that destroys the renal parenchyma. It is an uncommon entity. Diffuse inflammation has the potential to spread to nearby organs, especially the skin.A 73-year-old patient presented with a three-year history of painful and fistulized nodules on the abdominal wall. The results of abdominal computed tomography and magnetic resonance imaging revealed xanthogranulomatous pyelonephritis with extension to the skin, colon, and psoas muscle. The skin lesions were improved by a double antibiotic therapy. The patient was advised to have a radical left nephrectomy, but he refused surgery and was then lost to follow-up.We report an uncommon case of xanthogranulomatous pyelonephritis revealed by cutaneous nodules of the abdominal wall, with an extension toward the skin, the colon and the psoas muscle.

BACKGROUND:Adverse pregnancy outcomes in women with primary Sjögren's syndrome have only been evaluated retrospectively using heterogeneous methods and with contradictory results. We aimed to describe adverse pregnancy, delivery, and birth outcome risks in pregnant women with primary Sjögren's syndrome compared with those of a matched general population in France, and to identify factors predictive of disease flares or adverse pregnancy outcomes. METHODS:We conducted a multicentre, prospective, cohort study in France using the GR2 (Groupe de Recherche sur la Grossesse et les Maladies Rares) registry. Women from the GR2 study were eligible if they had conceived before March, 2021, had primary Sjögren's syndrome according to the American College of Rheumatology and European Alliance of Associations for Rheumatology (EULAR) 2016 classification criteria, and had an ongoing pregnancy at 12 weeks of gestation. In women who entered in the registry with pregnancies before 18 weeks of gestation, we sought to identify factors associated with primary Sjögren's syndrome flare (≥3-point increase in EULAR Sjögren's Syndrome Disease Activity Index [ESSDAI] score) or adverse pregnancy outcomes (fetal or neonatal death, placental insufficiency leading to a preterm delivery [<37 weeks of gestation], or small-for-gestational-age birthweight). A matched controlled study compared adverse pregnancy, delivery, and birth outcome rates between pregnant women with primary Sjögren's syndrome from the GR2 registry and matched controls from the general population included in the last French perinatal survey (Enquête Nationale Périnatale 2016). FINDINGS:1944 pregnancies were identified in the GR2 cohort, of which 106 pregnancies in 96 women with primary Sjögren's syndrome were included in this analysis. The median age at pregnancy onset was 33 years (IQR 31-36). 87 (83%) of 105 pregnancies (with ethnicity data) were in White women, 18 (17%) were in Black women; 92 (90%) of 102 had previous systemic activity (ESSDAI score of ≥1; data missing in four pregnancies), and 48 (45%) of 106 had systemic activity at inclusion. Of 93 pregnancies included at week 18 of gestation or earlier, primary Sjögren's syndrome flares occurred in 12 (13%). No baseline parameters were associated with primary Sjögren's syndrome flare. Four twin pregnancies and one medical termination were excluded from the adverse pregnancy outcome analysis; of the remaining 88, adverse pregnancy outcomes occurred in six (7%). Among pregnancies in women with data for antiphospholipid antibodies (n=55), antiphospholipid antibody positivity was more frequent among pregnancies with adverse outcomes (two [50%] of four pregnancies) compared with those without adverse outcomes (two [4%] of 51 pregnancies; p=0·023). Anti-RNP antibody positivity was also more frequent among pregnancies with adverse outcomes than those without, although this was not statistically significant. In the matched controlled study, adverse pregnancy outcomes occurred in nine (9%) of 105 pregnancies in women with primary Sjögren's syndrome and 28 (7%) of the 420 matched control pregnancies; adverse pregnancy outcomes were not significantly associated with primary Sjögren's syndrome (odds ratio 1·31, 95% CI 0·53-2·98; p=0·52). INTERPRETATION:Pregnancies in women with primary Sjögren's syndrome had very good prognoses for mothers and fetuses, with no overall increase in adverse pregnancy outcome risk compared with the general population. Women with antiphospholipid antibodies or anti-RNP antibodies require close monitoring, because these factors might be associated with a higher risk of adverse pregnancy outcomes. FUNDING:Lupus France, Association des Sclérodermiques de France, Association Gougerot Sjögren, Association Francophone Contre la Polychondrite Chronique Atrophiante, AFM-Telethon, Société Nationale Française de Médecine Interne, Société Française de Rhumatologie, Cochin Hospital, French Health Ministry, Fondation for Research in Rheumatology, Association Prix Véronique Roualet, Union Chimique Belge.