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Background Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature. Objective To determine the mortality and its predictors in a long-term follow-up of a bi-centric cohort of SSc patients. Methods A retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital. Standardized Mortality Ratio (SMR), mortality at 1, 3, 5, 10, and 15 years of disease and causes of death were described. Predictors of mortality using Cox regression were assessed. Results Three hundred seventy-five patients were included: 63 with diffuse cutaneous SSc, 279 with limited cutaneous SSc, and 33 with sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46–1.97). The overall survival rates were 97.6% at 1 year, 93.4% at 3 years, 87.1% at 5 years, 77.9% at 10 years, and 61.3% at 15 years. Sixty-nine deaths were recorded. 46.4% were SSc related deaths secondary to interstitial lung disease (ILD) (34.4%), pulmonary hypertension (31.2%), and digestive tract involvement (18.8%). 53.6% were non-related to SSc: cardiovascular disorders (37.8%) and various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors were identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p=0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; p=0.0174), cardiac involvement (HR=2.86; p=0.0012), and the fact of being male (HR=3.25; p=0.0004). Conclusion Long-term data confirmed high mortality of SSc. Male sex, DLCO 5mg/l were identified as independent predictors of mortality. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-021-02672-y., • Male sex, cardiac involvement, DLCO 5 mg/l are strong predictors of mortality in systemic sclerosis. • This study shows the survival of subtypes and in particular sine scleroderma. • Sine scleroderma subtype has better survival than diffuse or limited cutaneous subtypes. • Non-systemic sclerosis-related deaths are more frequent than systemic sclerosis-related deaths. • Cardiovascular events non-systemic sclerosis-related are the main deaths. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-021-02672-y.

Objectives Excessive and inappropriate production of pro-inflammatory cytokines plays a key role in Still’s disease. Janus kinase inhibitor (JAKi) agents mainly block pro-inflammatory cytokine pathways, notably IL-6 and IFN. The objective was to assess the efficacy and safety of JAKi agents in difficult-to-treat systemic JIA or adult-onset Still’s disease (AOSD). Methods This retrospective study was based on a national survey conducted in the departments of rheumatology, paediatric rheumatology and internal medicine of French hospitals regarding systemic JIA and AOSD patients who received JAKi agents. The data were collected with a standardized questionnaire and analysed at different times (treatment initiation, months 1, 3 and 6 and the end of follow-up). Results Nine patients (seven adults) were included. All patients showed inadequate response to CS or conventional synthetic or biologic DMARDs. Baricitinib was used in five patients, ruxolitinib in two, tofacitinib in two and upadacitinib in one. A JAKi was used combined with CS in all but two patients. A JAKi was associated with anakinra and CS in one patient, and with MTX, anakinra and CS in another. The median (range) follow-up was 16 (1–33) months. Two cases out of nine showed complete remission, 3/9 partial response and 4/9 treatment failure. At the last visit, CS could be decreased but not stopped. Tolerance of the JAKi was acceptable (no severe adverse events). Conclusion JAKi agents may be a therapeutic option for some patients with difficult-to-treat Still’s disease, especially those with partial response to medium- or high-dose CS or biologics.

Fabry disease is the second most frequent lysosomal storage disorder. It is a X-linked genetic disease secondary to alpha-galactosidase A enzyme deficiency. This is a progressive and systemic disease that affects both males and females. Classical symptoms and organ involvements are acral pain crisis, cornea verticillata, hypertrophic cardiomyopathy, stroke and chronic kidney disease with proteinuria. Nevertheless, organ damages can be missing or pauci-symptomatic and other common symptoms are poorly recognised, such as gastrointestinal or ear involvement. In classical Fabry disease, symptoms first appear during childhood or teenage in males, but later in females. Patients may have non-classical or late-onset Fabry disease with delayed manifestations or with single-organ involvement. Recognition of Fabry disease is important because treatments are available, but it may be challenging. Diagnosis is easy in males, with dosage of alpha-galactosidase A enzyme activity into leukocytes, but more difficult in females who can express normal residual activity. Other plasmatic biomarkers, such as lyso-globotriaosylceramide (lyso-Gb3), are interesting in females, but need to be associated with GLA gene analysis. In this review, we aimed at summarize the main clinical manifestations of Fabry disease and propose a practical algorithm to know how to diagnose this complex disease.

Fabry disease is a frequent lysosomal storage disorder secondary to the deficiency of alpha-galactosidase A enzyme. This X-linked genetic disease realizes progressive and systemic manifestations that affect both male and female. Fabry disease may present as "classical", as "late-onset" or "non-classical" forms. Symptoms and organ involvements of classical Fabry disease are acral pain crisis, cornea verticillata, hypertrophic cardiomyopathy, stroke and chronic kidney disease with proteinuria. Other common symptoms are often poorly recognized, such as gastrointestinal or ear involvements. In classical Fabry disease, symptoms first appear during childhood or during teenage years in males, but later in females. Patients with non-classical or late-onset Fabry disease have delayed manifestations or a single-organ involvement. Diagnosis is therefore difficult when classical organ involvements are missing, in paucisymptomatic patients or in late-onset forms. Recognition of Fabry disease is important because effective treatments are available. They have to be prescribed early. In male, diagnosis is made with alpha-galactosidase A enzyme activity dosage in leukocyte, that is very low or null in classical forms and under 30 percent in late-onset forms. Diagnosis is more challenging in females who may express normal residual enzyme activity. Other plasmatic biomarkers, such as lyso-globotriaosylceramide are interesting, especially in females. In this review, we aimed to summarize main clinical manifestations of Fabry disease to know when to evoke Fabry disease and propose a practical diagnosis algorithm to know how to diagnose.

BACKGROUND Portal vein Doppler ultrasound pulsatility measured by transoesophageal echocardiography is a marker of the haemodynamic impact of venous congestion in cardiac surgery. We investigated whether the presence of abnormal portal vein flow pulsatility is associated with a longer duration of invasive life support and postoperative complications in high-risk patients. METHODS In this multicentre cohort study, pulsed-wave Doppler ultrasound assessments of portal vein flow were performed during anaesthesia before initiation of cardiopulmonary bypass (before CPB) and after separation of cardiopulmonary bypass (after CPB). Abnormal pulsatility was defined as portal pulsatility fraction (PPF) ≥50% (PPF50). The primary outcome was the cumulative time in perioperative organ dysfunction (TPOD) requiring invasive life support during 28 days. Secondary outcomes included major postoperative complications. RESULTS 373 patients, 71 (22.0%) had PPF50 before CPB and 77 (24.9%) after CPB. PPF50 was associated with longer duration of TPOD (median [inter-quartile range]; before CPB: 27 h [11-72] vs 19 h [8.5-42], P=0.02; after CPB: 27 h [11-61] vs 20 h [8-42], P=0.006). After adjusting for confounders, PPF50 before CPB showed significant association with TPOD. PPF50 after CPB was associated with a higher rate of major postoperative complications (36.4% vs 20.3%, P=0.006). CONCLUSIONS Abnormal portal vein flow pulsatility before cardiopulmonary bypass was associated with longer duration of life support therapy after cardiac surgery in high-risk patients. Abnormal portal vein flow pulsatility after cardiopulmonary bypass separation was associated with a higher risk of major postoperative complications although this association was not independent of other factors. CLINICAL TRIAL REGISTRATION NCT03656263.

Mucopolysaccharidosis are lysosomal storage diseases, secondary to the accumulation of mucopolysaccharides. Type 1 mucopolysaccharidosis is the most common form and affects between 0.69 and 1.66 newborns per 100,000. The severity of mucopolysaccharidosis is variable with lethal forms in utero and attenuated forms diagnosed in adults. The most common symptoms are short stature, facial dysmorphism, chronic articular pains that can mimic chronic inflammatory rheumatism, axial and peripheral bone involvement, hepatosplenomegaly and an early carpal tunnel. Depending on the type of mucopolysaccharidosis, corneal, cerebral or cardiac involvements are possible. Screening is based on the analysis of urinary glycosaminoglycans. The deficient enzyme assay and the gene analysis confirm the diagnosis. Mucopolysaccharidosis recognition is important for patient management and family screening. In addition, specific enzyme replacement therapy exists for certain types of mucopolysaccharidosis. Role of clinician is important to evoke and diagnose mucopolysaccharidosis.

In France, clinical research involving human beings is regulated by the Jarde's law since November 2016. The law distinguishes interventional and non-interventional studies. Both need to be authorized by a Persons Protection Committee. Studies performed on medical data collected during standard clinical care are not considered as studies involving human beings. Medical data are personal data. French Data Protection Authority National has an important role, guarantying the respect of regulation on personal data. We summarize in this article the different types of studies and the role of regulatory authorities.

Objective To assess the safety and the efficacy of TNF-α antagonists and tocilizumab in patients with Takayasu arteritis (TAK). Methods A total of 209 patients with TAK [median age 29 years (interquartile range 7–62)], 186 (89%) females] were included. They received either TNF-α antagonists [n = 132 (63%) with 172 lines; infliximab (n = 109), adalimumab (n = 45), golimumab (n = 8), certolizumab (n = 6) and etanercept (n = 5)] or tocilizumab [n = 77 (37%) with 121 lines; i.v. and s.c. in 95 and 26 cases, respectively]. Results A complete response at 6 months was evidenced in 101/152 (66%) patients on TNF-α antagonists and 75/107 (70%) patients on tocilizumab. Age ≥30 years [odds ratio 2.09 (95% CI 1.09, 3.99)] was associated with complete response, whereas vascular signs [OR 0.26 (95% CI 0.1, 0.65)], baseline prednisone ≥20 mg/day [OR 0.51 (95% CI 0.28, 0.93)] were negatively associated with the complete response to TNF-α antagonists or tocilizumab. During a median follow-up of 36 months, 103 relapses were noted. Supra-aortic branches and thoracic aorta involvement [HR 2.44 (95% CI 1.06, 5.65) and 3.66 (1.18, 11.4), respectively] and systemic signs at baseline [HR 2.01 (95% CI 1.30, 3.11)] were significantly associated with relapse. The cumulative incidence of treatment discontinuation and relapse were similar in TNF-α antagonists and tocilizumab. Fifty-eight (20%) adverse effects occurred on biologic targeted therapies [37 (21%) on TNF-α antagonists and 21 (17%) on tocilizumab (P = 0.4), respectively]. Conclusion This large multicentre study shows high efficacy of biologic targeted treatments in refractory TAK. Efficacy, relapse and drug retention rate were equivalent with TNF-α antagonists and tocilizumab.

Background: Few studies of daily practice for patients with giant cell arteritis (GCA) are available. This French study aimed to describe the characteristics and management of GCA in a real-life setting.Methods: Cross-sectional, non-interventional, multicenter study of patients ≥50 years old who consulted hospital-based specialists for GCA and were under treatment. Patient characteristics and journey, diagnostic methods and treatments were collected. Descriptive analyses were performed.Results: In total, 306 patients (67% females, mean age 74 ± 8 years old) were recruited by 69 physicians (internists: 85%, rheumatologists: 15%); 13% of patients had newly diagnosed GCA (diagnosis-to-visit interval 18FDG-PET, and aortic angio-CT, respectively. All patients received glucocorticoids, which were ongoing for 89%; 29% also received adjunct medication(s) (methotrexate: 19%, tocilizumab: 15%). A total of 40% had relapse(s); the median time to the first relapse was 10 months. Also, 37% had comorbidity(ies) related to or aggravated by glucocorticoids therapy.Conclusion: This large observational study provides insight into current medical practices for GCA. More than one third of patients had comorbidities related to glucocorticoid therapy for a median disease duration of 13 months. Methotrexate and tocilizumab were the most common adjunct medications.

Glucocorticoids (GCs) are the mainstay of treatment for patients with polymyalgia rheumatica (PMR).1 Despite their efficacy, GCs are associated with well-known adverse events and a substantial proportion of patients with PMR do not respond adequately, or are refractory, to initial GC treatment. GC-sparing agents in PMR are limited to methotrexate (MTX).1.

An association of systemic sclerosis (SSc) with cryoglobulin and/or cryofibrinogenemia has been described. However, clinical, biological, morphological and prognostic implications are unknown. The objective of this study was to describe the phenotype and evaluate the prognosis of cryoglobulinemia and/or cryofibrinogenemia in the progression of SSc.Patients were included from the Systemic Scleroderma Toulouse Cohort (SSTC), between June 1, 2005 and May 31, 2018, and underwent a measurement of a cryoglobulin and/or cryofibrinogen in immunology laboratory at the Toulouse University Hospital Center. Patients with and without cryoglobulinemia50 mg/l and patients with and without cryofibrinogenemia were compared to identified the impact of cryoprcipitate on the phenotype. Mortality based on cryoprecipitate was explored.166 patients were included in the study. 43.3% and 46.6% had a cryoglobulinemia50 mg/l and cryofibrinogenemia, respectively. Cryoglobulin50 mg was not associated with microvascular damage. Cryoglobulin does not influence the phenotype. 5-and 10-years survival were 97.6% and 88.8% respectively in patients with cryoglobulinemia50 mg/l versus 91.9% and 78.4% in patients without cryoglobulin50 mg/l. 10-years survival was better for patients with cryoglobulinemia50 mg/l (log-rank 0.0363). Cryofibrinogenemia was not associated with neoplasia, any clinical (in particular ischemic damage), biological or morphological features. Cryofibrinogenemia had no influence on the mortality of these patients.Cryoglobulinemia and cryofibrinogenemia are frequent in SSc. The presence of cryoprecipitate (cryoglobulin or cryofibrinogen) not influence the phenotype and has not associated with a poor survival.

Classification criteria for systemic sclerosis evolved over the last three decades, allowing an earlier classification. In the late 2000s, the EULAR Scleroderma Trials and Research Group validated very early and early systemic sclerosis criteria. Raynaud phenomenon, anti-nuclear antibody positivity and the puffy fingers are "Red flags" that must lead to refer the patient to a specialist and benefit from a capillaroscopy and the specific autoantibodies. At the stage of very early systemic sclerosis, pulmonary, cardiac and digestive involvements may be present and must be screened. Herein, we detail very early and early systemic sclerosis criteria, as well as the predictive factors of evolution towards a systemic sclerosis.

Summary Background Various observations have suggested that the course of COVID-19 might be less favourable in patients with inflammatory rheumatic and musculoskeletal diseases receiving rituximab compared with those not receiving rituximab. We aimed to investigate whether treatment with rituximab is associated with severe COVID-19 outcomes in patients with inflammatory rheumatic and musculoskeletal diseases. Methods In this cohort study, we analysed data from the French RMD COVID-19 cohort, which included patients aged 18 years or older with inflammatory rheumatic and musculoskeletal diseases and highly suspected or confirmed COVID-19. The primary endpoint was the severity of COVID-19 in patients treated with rituximab (rituximab group) compared with patients who did not receive rituximab (no rituximab group). Severe disease was defined as that requiring admission to an intensive care unit or leading to death. Secondary objectives were to analyse deaths and duration of hospital stay. The inverse probability of treatment weighting propensity score method was used to adjust for potential confounding factors (age, sex, arterial hypertension, diabetes, smoking status, body-mass index, interstitial lung disease, cardiovascular diseases, cancer, corticosteroid use, chronic renal failure, and the underlying disease [rheumatoid arthritis vs others]). Odds ratios and hazard ratios and their 95% CIs were calculated as effect size, by dividing the two population mean differences by their SD. This study is registered with ClinicalTrials.gov, NCT04353609. Findings Between April 15, 2020, and Nov 20, 2020, data were collected for 1090 patients (mean age 55·2 years [SD 16·4]); 734 (67%) were female and 356 (33%) were male. Of the 1090 patients, 137 (13%) developed severe COVID-19 and 89 (8%) died. After adjusting for potential confounding factors, severe disease was observed more frequently (effect size 3·26, 95% CI 1·66–6·40, p=0·0006) and the duration of hospital stay was markedly longer (0·62, 0·46–0·85, p=0·0024) in the 63 patients in the rituximab group than in the 1027 patients in the no rituximab group. 13 (21%) of 63 patients in the rituximab group died compared with 76 (7%) of 1027 patients in the no rituximab group, but the adjusted risk of death was not significantly increased in the rituximab group (effect size 1·32, 95% CI 0·55–3·19, p=0·53). Interpretation Rituximab therapy is associated with more severe COVID-19. Rituximab will have to be prescribed with particular caution in patients with inflammatory rheumatic and musculoskeletal diseases. Funding None.

Objective The purpose of the present study was to investigate the hypothesis of a nychthemeral variation in the tolerance to ischemia and reperfusion injury in adult cardiac surgeries. Design Retrospective cohort study. Setting A single academic center. Participants All patients undergoing nonemergent aortic valve replacement (AVR) ± coronary artery bypass graft between January 2012 and May 2018 were included. They were divided into two groups (morning and afternoon) according to the time of the day at the beginning of surgery. Propensity score matching estimated by multivariate logistic regression with a 1:1 matching ratio was performed to ensure that the two groups were comparable. This allowed obtaining 269 pairs, for a total of 538 patients. Intervention The objective of the study was to assess whether there were differences in perioperative and postoperative outcomes between the morning and the afternoon groups. Results There was no between-group difference in the primary composite endpoints, namely the occurrence of death, myocardial infarction, low cardiac output, and stroke during the 30 days following the surgery. Regarding cardiac biomarkers, there were no between-group differences for both postoperative evolution of troponin T plasma levels and the maximum postoperative troponin T plasma level. Conclusion These results did not support the hypothesis that the timing of the surgery could influence the tolerance to ischemia and reperfusion injury, at least in patients undergoing nonemergent AVR or a combined AVR with coronary artery bypass graft.

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The most prevalent etiologies of splenomegaly (SM) are easily detected in routine clinical practice. However, after these first-line diagnoses have been ruled out, many cases remain unexplained. Frequently, signs and symptoms are non-specific hiding rare etiologies such as Lysosomal Storage Diseases (LSD) (e.g., Gaucher disease (GD), Niemann-Pick disease type B). SplenoMegaly Study (SMS) aims to estimate the prevalence of different etiologies in unexplained SM and to describe their diagnostic work-up. We present here the final results of the study. SMS is a prospective, observational, multi-center, longitudinal study performed in France. All patients with unexplained SM, aged ≥15 years, consulting to the French hematology or internal medicine departments were invited to take part of the study. SM was confirmed by echography or CT-scan with a craniocaudal length ≥13 cm; unexplained was defined as negative clinical and biological routine workup. All conducted exams and tests for diagnosis purpose were under each physician's responsibility. For each patient, last visit took place when an etiology was identified or up to 12 months after inclusion. From Sept-2015 to Apr-2020, 505 patients were enrolled by 87 centers (secondary centers [71.3%] and tertiary centers [28.7%]). Physicians were mainly internists (52.9%) or hematologists (46.0%). Patients characteristics at inclusion were as follows: male gender, 61.2%; median age, 51.0 years old (16-94 years old); median spleen length, 15.0 cm (13-30 cm); heterogeneous spleen structure, 11.1%. A total of 501 patients completed the study. After a median duration of 6.6 months, 232 patients (46.3%) received a diagnosis. When compared to patients without diagnosis, patients with diagnosis were older (median age 59.5 vs 45.0; p In SMS, patients at inclusion had unexplained SM despite routine clinical and biologic analysis. The diagnostic yield improved after further medical evaluations reaching an etiologic diagnosis in 46% of patients. Patients with an established diagnosis were older, had a larger spleen and altered biological parameters compared to patients without diagnosis. Interestingly, non-malignant diseases accounted for 61.3% of diagnoses. It is noteworthy the diversity of LSD etiologies obtained: 10 patients (4%) were diagnosed with LSD, including 4 GD1 patients. This study provides useful new information to optimize the diagnostic strategy of splenomegaly poorly explained by routine workup. SMS is sponsored by Sanofi-Genzyme. Figure 1 Figure 1. Disclosures Denis: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Terriou: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Séné: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Costello: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Michaud: Sanofi: Honoraria, Research Funding. Hellé: Sanofi: Current Employment. Lagadec: Sanofi: Current Employment. Bauduer: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Sanhes: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Urbanski: Sanofi: Honoraria, Research Funding. Berger: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding.

Introduction La plupart des etiologies courantes de splenomegalies (SMG) sont facilement identifiees en pratique clinique courante. Cependant, une fois ces diagnostics de premiere intention ecartes, la demarche diagnostique est alors un vrai defi, la SMG pouvant etre associee a des etiologies plus rares comme les maladies de surcharge lysosomale (MSL). L’etude SMS (SplenoMegaly Study) est une etude francaise prospective, observationnelle, multicentrique et longitudinale, qui a pour objectif d’estimer la prevalence des differentes etiologies de SMG inexpliquees. Les resultats intermediaires presentes ici portent sur les caracteristiques des patients et les tests et diagnostics etablis lors du suivi. Patients et methodes Depuis 2015, tout patient âge de 15 ans et plus et adresse vers un service d’hematologie ou de medecine interne des 118 hopitaux participants, avec une SMG d’etiologie inexpliquee (definie comme d’origine inconnue apres exclusion des diagnostics de premiere intention sur la base d’un interrogatoire, d’un examen clinique et d’un bilan biologique de routine) ont ete inclus. Pour chaque patient, la derniere visite a lieu lorsqu’une etiologie est identifiee ou jusqu’a 12 mois apres l’inclusion. Comme il n’existe pas de definition precise de SMG, l’application Splenocalc [Chow and al., 2016], a ete utilisee pour confirmer la SMG. L’objectif est d’inclure 500 patients. Resultats En septembre 2019, 374 patients (60 % d’hommes) ont ete inclus, avec un âge median de 53 ans (16–94 ans), une longueur mediane de la rate de 15 cm (13–30 cm) et une structure homogene de la rate pour 88 % d’entre eux. Une echographie et un scanner ont ete realises pour confirmer la SMG chez respectivement 53 % et 46 % des patients. Au total, 276 patients (74 %) ont termine l’etude, dont 138 (50 %) ont eu un diagnostic etabli. Par rapport aux patients restes sans diagnostic, les patients avec un diagnostic sont plus âges (âge median 62,0 ans contre 45,5 ans ; p Conclusion La SMG inexpliquee semble etre un challenge diagnostique pour lequel l’interniste et l’hematologue ont un role cle. Apres 12 mois d’investigations regulieres, 50 % des patients sont restes sans diagnostic. Les patients avec un diagnostic etabli, en particulier ceux avec des hemopathies malignes, sont plus âges et ont une rate de taille plus importante que les patients restes sans diagnostic. La BOM semble etre un examen utile dans la demarche diagnostique. Les pathologies non malignes representent 65 % des diagnostics. Ces resultats intermediaires doivent etre interpretes avec prudence car la population cible n’a pas encore ete atteinte. Les resultats definitifs sont attendus en 2021. Cette etude SMS est sponsorisee par Sanofi Genzyme.

Introduction La pericardite est une pathologie frequente. Elle est responsable de 5 % des hospitalisations pour douleurs thoraciques. La tuberculose est la cause principale dans les pays ou cette infection est epidemique. Dans les pays industrialises, les pericardites sont dans 80 a 90 % des cas « idiopathiques ». L’enjeu reste de ne pas meconnaitre les 10 a 20 % de pericardites en lien par exemple avec des pathologies inflammatoires systemiques ou neoplasiques. Le TEP scanner a deja fait la preuve de son interet dans la strategie diagnostique de ces affections. L’interet diagnostique du TEP scanner dans le bilan etiologique d’une pericardite n’a jamais ete etudie. Materiels et methodes Nous avons realise une etude retrospective bicentrique a partir du centre TEP du CHU de Toulouse. Les patients inclus etaient des patients de plus de 18 ans, beneficie d’un TEP scanner a visee diagnostique dans le cadre du bilan etiologique d’une pericardite. Resultats Vingt neuf patients ont ete inclus (14 hommes et 15 femmes). L’âge moyen au diagnostic de pericardite etait de 51 ± 18 ans. Concernant les caracteristiques des pericardites, la douleur etait presente dans 93 % des cas, un frottement pericardique auscultatoire dans 17 % des cas, des anomalies ECG dans 31 % des cas, un epanchement pericardique dans 76 % des cas. Sept patients avaient une tamponnade. Treize patients (45 %) avaient une pleuresie associee. Onze patients (38 %) etaient febriles, 7 avaient une alteration de l’etat general (24 %). Cinq patients avaient un ATCD de maladie auto-immune et 4 de cancer. Les pericardites etaient aigues dans 10 cas et chroniques dans 19 cas. Le nombre moyen de pericardites par patient etait de 1,7 ± 1,4 (de 1 a 5). Le delai median entre l’episode de pericardite et la realisation du TEP scanner etait de 22 ± 23 jours. La CRP moyenne etait de 142 ± 104 mg/L (de 5 a 369 mg/L). Une hyperfixation pericardique au TEP scanner etait signalee dans 19 cas (65 %). Chez ces patients, une anomalie au scanner thoraco-abdomino-pelvien avait ete retrouvee chez 17 d’entre eux (90 %). Finalement, le TEP scanner a permis un apport dans la demarche diagnostique dans 3 cas de pericardite (10 %). Il s’agissait toujours de pericardites chroniques. Un patient ayant deja beneficie de biopsies ganglionnaires sans apport diagnostique a beneficie d’un TEP scanner, qui a permis de cibler la biopsie ganglionnaire et aboutir au diagnostic de sarcoidose. Chez deux patients, le TEP scanner a mis en evidence une hyperfixation ganglionnaire. Chez une patiente la biopsie aspecifique n’a pas permis le diagnostic et la patiente a beneficie d’un traitement d’epreuve par quadritherapie antituberculeuse et corticotherapie. Dans l’autre cas, une biopsie ganglionnaire etait preconisee chez un patient ayant un ATCD de cancer bronchique mais le patient a ete perdu de vue. Discussion L’interet du TEP scanner au cours du bilan etiologique des pericardites n’a, a notre connaissance, jamais ete etudie. L’incidence des pericardites aigues est de 27,7 cas pour 100 000 personnes par an et la mortalite est faible (1,1 %), principalement en cas de pathologie infectieuse associee et chez les personnes âgees. L’apport diagnostique dans notre etude est faible car le scanner thoraco-abdomino-pelvien apportait deja des informations importantes. L’interet diagnostique principal du TEP scanner est de rechercher l’hyperfixation d’une lesion visualisee au scanner afin de guider une biopsie. Une etude a evalue l’apport du TEP scanner pour differencier les pericardites tuberculeuses et les pericardites idiopathiques. La fixation pericardique etait plus importante au cours de pericardites tuberculeuses et la TEP scanner permettait de mettre en evidence des hyperfixations ganglionnaires associees. L’interet pronostique du TEP scanner au cours d’un premier episode de pericardite a ete evalue dans une etude et l’intensite de la fixation scintigraphique du pericarde etait associee au risque de recurrence de la pericardite. Au cours des pericardites neoplasiques, le TEP scanner est peu utile car concordant avec les donnees de l’echographie. Conclusion La rentabilite diagnostique du TEP scanner au cours des pericardites est faible, principalement car il n’apporte pas d’information supplementaire par rapport au scanner thoraco-abdomino-pelvien. L’indication du TEP scanner au cours des pericardites doit concerner les pericardites chroniques, et etre discutee au cas par cas afin d’apporter des arguments supplementaires en cas suspicion diagnostique.

Introduction L’arterite de Takayasu est une maladie tres rare (2 a 3 cas par an par million d’habitants) touchant principalement les femmes de moins de 40 ans. Il s’agit d’une vascularite des gros vaisseaux touchant l’aorte et ses branches, au premier rang desquelles les arteres sous-clavieres et les carotides. La presentation clinique initiale sous forme de surdite bilaterale est exceptionnelle. Observation Une jeune femme de 18 ans, d’origine algerienne, consulte en ORL en raison de la survenue d’une surdite partielle bilaterale asymetrique, rapidement progressive en quelques semaines. Le bilan retrouve une surdite mixte predominant a droite. Un scanner est realise, retrouvant des opacites inflammatoire de la caisse des deux tympans au niveau des recessus posterieurs et des fenetres. A droite, il existe egalement des signes d’osteoarthrite incudo-malleaire. Pas d’anomalie labyrinthique. A l’interrogatoire, il n’y a pas d’alteration de l’etat general ni de syndrome febrile. La patiente rapporte la presence d’aphtes buccaux repetes depuis quelques mois sans aphtose genitale associee ainsi que l’apparition de crampes dans les mollets a la marche. A l’examen clinique, les pouls peripheriques des 4 membres sont ne sont pas percus et l’auscultation retrouve un souffle carotidien gauche et des souffles femoraux bilateraux. Il n’y a jamais eu d’atteinte oculaire. Le bilan sanguin retrouve une anemie microcytaire a 9,4 g/dL (VGM 71,3). La CRP est a 47 mg/L, le fibrinogene a 7,23 g/L. Les serologies syphilis, hepatites B et C, VIH sont negatives. Le bilan immunologique est negatif en dehors de la presence d’ANCA type c-ANCA, a titre faible et sans specificite anti-PR3 ou anti-MPO. Le QuantiFERON est negatif. Le scanner thoraco-abdomino-pelvien retrouve des anomalies vasculaires avec un important epaississement circonferentiel regulier de l’ensemble des parois aortiques, des troncs supra-aortiques ainsi que des arteres digestives et renales. Il existe un aspect de double contour au niveau de la crosse de l’aorte, mesure au maximum a 6,5 mm ainsi qu’une ectasie de 42 mm de diametre a ce niveau. Aucune stenose n’est visualisee. L’angioscanner des troncs supra-aortiques rapportent un epaississement parietal circonferentiel des carotides primitives et un epaississement parietal de l’artere sous-claviere droite, avec une stenose longue et irreguliere. Le TEP scanner au 18-FDG retrouve un hypermetabolisme parietal circonferentiel et homogene de l’aorte thoracique ascendante, de la crosse et de l’aorte thoracique descendante. L’echocardiographie retrouve une fuite aortique sans dilatation aortique. Le diagnostic d’arterite de Takayasu est pose, de type V, avec un score NIH initial de 3 sur 4. Un traitement car corticoides (1 mg/kg) et methotrexate (25 mg/semaine) est debute avec une amelioration modeste des parametres inflammatoires. Un traitement par infliximab 5 mg/kg est donc ajoute, avec une amelioration biologique et radiologique rapide (score NIH a 0 a 6 mois). Discussion La physiopathologie exacte de l’atteinte auditive dans la maladie de Takayasu n’est pas connue. Il s’agit probablement d’un processus mixte inflammatoire et ischemique. Cette atteinte est importante a connaitre car il peut s’agir de la seule plainte rapportee par le patient. La palpation des pouls peripheriques et l’auscultation des axes arteriels etaient donc des elements diagnostiques cliniques primordiaux. Notre cas montre egalement la discordance majeure qu’il peut exister entre les symptomes evoques et la diffusion de l’atteinte radiologique. Le diagnostic differentiel principal chez notre patient etait le syndrome de Cogan, qui presente une atteinte audio-vestibulaire et possiblement une aortique. Neanmoins, l’absence d’atteinte oculaire, sous forme de keratite interstitielle non syphilitique, etait un element contre ce diagnostic. Conclusion La survenue d’une surdite chez une femme jeune doit faire rechercher une arterite de Takayasu, notamment en cas de lesions inflammatoires de l’oreille moyenne a l’imagerie ou de syndrome inflammatoire biologique.

Introduction Une aggravation paradoxale peut survenir a l’initiation d’un traitement antituberculeux au cours d’une infection VIH non traitee. Il s’agit d’un syndrome inflammatoire de restauration immune (IRIS). Nous rapportons le cas d’un patient presentant un IRIS tuberculeux severe, resistant a la corticotherapie et ameliore par la mise en place d’un traitement par infliximab. Observation Un patient de 43 ans est hospitalise pour la prise en charge d’adenopathies cervicales. Il a ete diagnostique un an plus tot au Nigeria d’une infection par le VIH et a recu pendant un traitement antituberculeux. A l’entree, il est traite avec une bonne observance par abacavir/lamivudine (600 mg/300 mg), RITONAVIR 100 mg, darunavir 800 mg et sulfamethoxazole/trimethoprime 800 mg par jour. Il presente depuis le debut du traitement antiretroviral des adenopathies cervicales bilaterales douloureuses, renitentes, sensibles a la palpation ainsi que des adenopathies axillaires droites centimetriques. La ponction a l’aiguille d’une adenopathie ramene un liquide caseeux avec une PCR BK positive. Le scanner retrouve un emphyseme bulleux diffus, une sequestration lobaire inferieure gauche, ainsi que des ganglions intra-abdominaux necrotiques. Concernant l’infection VIH, la charge virale est indetectable et les CD4 sont a 381/mm3. Une quadritherapie classique par rifampicine, isoniazide, ethambutol et pyrazinamide est donc instauree en aout 2018. Deux mois plus tard les adenopathies sont toujours majeures, le controle du scanner retrouve une aggravation avec une volumineuse coulee ganglionnaire depuis l’espace infra-mediastinal posterieur jusqu’au pelvis, s’etendant a la racine du mesentere et au petit epiploon. Ces coulees ganglionnaires realisent une gangue circonferentielle autour des vaisseaux digestifs. Apparaissent des lesions des psoas et un osteolyse de L1. Une corticotherapie a 0,5 mg/kg est donc debutee, sans efficacite. A visee symptomatique une chirurgie d’adenectomies cervicales est realisee en decembre 2018. L’etat general s’aggrave encore, avec la persistance d’une fievre et de sueurs. Le PET-scanner au 18FDG met en evidence des hypermetabolismes intenses et multiples, ganglionnaires sus- et sous-diaphragmatiques, mais egalement de la lesion osteolytique de L1, de la peripherie des lesions des muscles psoas et ilio-psoas. Une masse du testicule droit est apparue, dont l’examen anatomopathologique est en faveur d’une origine tuberculeuse. De nouveaux prelevements ganglionnaires retrouvent des sensibilites conservees aux antituberculeux classiques et les dosages pharmacologiques sont en faveur de la bonne observance du patient. Finalement, un traitement par infliximab 5 mg/kg est initie en juin 2019 selon un protocole classique (j1, j15, j45 puis toutes les 6 semaines). La CRP passe en deux semaines de 41 mg/L a 6 mg/L, la fievre et les sueurs disparaissent. Le poids passe de 70 a 72 kg en 2 mois. Les adenopathies diminuent progressivement de taille cliniquement et le scanner de reevaluation effectue a un mois de l’introduction de l’infliximab est stable. La visite de dernieres nouvelles du patient a eu lieu a deux mois de l’initiation de l’anti-TNF. Discussion L’initiation du traitement antiretroviral (ARV) au cours d’une tuberculose associee au VIH ameliore la survie des patients. Il existe neanmoins un risque d’aggravation initiale de la tuberculose. Cet IRIS tuberculeux, present dans 8 a 54 % des cas, est source d’une morbi-mortalite importante, en particulier chez les patients les plus immunodeprimes en cas d’initiation trop precoce des ARV. Sur le plan immunologique, l’IRIS est secondaire a la presence de taux tres eleves de cytokines pro-inflammatoires. La mise en place du traitement ARV entraine une augmentation rapide des taux de lymphocytes T4 specifiques diriges contre le bacille tuberculeux. Ceci amplifie la reponse inflammatoire et l’augmentation des taux d’interferon gamma, d’interleukines 2 et 6, ainsi que de TNF alpha. Les criteres diagnostiques majeurs d’IRIS tuberculeux sont l’aggravation ou l’apparition sous traitement d’atteinte tissulaire focalisee, de signes radiologiques, d’une atteinte du systeme nerveux central ou d’epanchement d’une sereuse. Les criteres mineurs sont l’apparition ou l’aggravation des signes generaux, respiratoires ou digestifs. Il convient d’exclure la possibilite d’un diagnostic differentiel, d’un echec du traitement (mauvaise observance, resistance aux antibiotiques). Sa prise en charge repose sur la corticotherapie. Les anti-TNF peuvent etre discutes dans les exceptionnelles formes resistantes. Conclusion Nous rapportons, a notre connaissance, le premier cas de traitement par anti-TNF d’un IRIS tuberculeux resistant a la corticotherapie. L’evolution du patient est favorable mais la duree de traitement est pour l’instant inconnue.

Background Tocilizumab (TCZ) is a recombinant humanized anti-interleukin-6 receptor monoclonal antibody. There is increasing evidence of TCZ efficacy in refractory auto-immune diseases. Objectives To describe off-label use, efficacy and tolerance of TCZ use in Internal Medicine Departments. Methods This is a retrospective, descriptive and multicenter study from 9 departments of Internal Medicine. Data were reported using a standardized case report file in January 2019. Results Fifty one patients were included (19 men, 32 women). Mean age was 55.6 ± 17 years (range 23 - 80). TCZ was used in: - 12 connective tissue diseases (23.5%): relapsing polychondritis (n=6), systemic sclerosis (n=3), anti-synthetase syndrome (n=1), and unclassified connective tissue disease (n=3). - 10 vasculitis (19%): Takayasu arteritis (n=7), Cogan disease (n=1), panarteritis nodosa (n=1), unclassified vasculitis (n=1). - 10 ophthalmologic conditions (19%): non infectious posterior uveitis (n=8), sympathetic ophtalmia (n=1), Basedow orbitopathy (n=1). - 8 adult-onset Still’s diseases (16%). - 5 cases of polymyalgia rheumatica (10%) - 3 miscellaneous diseases (6%): idiopathic AA amyloidosis, multicentric non HHV8 Castelmann disease, Erdheim Chester disease (1 case each). Mean disease duration was 7.5 ± 6.4 years. In 44 cases (86%) TCZ was administered for refractory disease to corticosteroids and immunosuppressive drugs. Previous therapies included corticosteroids (83%), methotrexate (66%), TNF inhibitor drug (44%), azathioprine (20.8%), mycophenolate (12%), cyclophosphamide (8%), rituximab (10%), hydroxychloroquine (6%), anakinra in 2 patients and interferon, dapsone, etoposide, leflunomide, abatacept, salazopyrin or intra-venous immunoglobulin in 1 patient each. TCZ was initiated as first-ligne therapy (15.5%), second-line therapy (17.5%), third-line therapy (31%), fourth-line therapy (19%), fifth-line therapy (14%), sixth-line therapy (12%) or as seventh line therapy in one case. TCZ was associated with methotrexate in 3 cases (6%). Treatment route was intravenous (96%). At the end of the follow up, 41 patients (80%) were still using TCZ, with a mean follow up period of 22 ± 23 months (range 1-90). In these patients, daily corticosteroid use significantly decrease from 16.5 ± 18 mg to 5.7 ± 13.7 mg (p TCZ was interrupted in 10 patients (19%), because of treatment failure (n=2), loss of efficacy (n=2) or side effect (=6). Side effects were infection (2 pneumonias, zona, sinus infection), pruritus (n=1), urticaria (n=1), dyslipidemia (n=1), high blood pressure (n=2), infusion-related reaction (n=1), bullous dermatitis (n=1), acute renal failure (n=1), angioedema (n=1), mouth ulcers (n=1). Conclusion TCZ is used in various autoimmune diseases. TCZ allowed a significant corticosteroids reduction and short term efficacy was 93% in patients using TCZ for more than 6 months. Nevertheless TCZ was interrupted in 19% of the patients. TCZ use will probably be more common in the future to treat refractory autoimmune diseases. Disclosure of Interests None declared

We read with interest the last 2019 update of the European League Against Rheumatism recommendations for the management of systemic lupus erythematosus (SLE).1 We agree with the comment by Gabriel Figueroa-Parra et al concerning the lack of mention of chloroquine as major treatment in SLE.2 Concerning survival, the LUMINA study group also showed that use of hydroxychloroquine (HCQ) was associated with a greater survival rate in 608 patients with SLE.3 Ruiz-Irastorza et al confirmed …

Introduction La decouverte d’une splenomegalie (SMG) est un motif frequent de consultation. Un examen clinique et un bilan biologique simple permettent le plus souvent de confirmer ou d’orienter le diagnostic. En Europe, les etiologies les plus frequentes relevent de causes hematologiques (hemopathie maligne ou non), hepatiques [hypertensions portales (HTP)] auto-immunes ou infectieuses. Neanmoins, apres exclusion de ces diagnostics, certaines SMG restent inexpliquees. La demarche diagnostique est alors un vrai defi, la SMG pouvant etre associee a des etiologies rares comme les maladies de surcharge lysosomales (MSL) (maladie de Gaucher, maladies de Niemann Pick). L’etude SMS (SplenoMegaly Study) est prospective, observationnelle, multi-centrique et longitudinale, et a pour objectif d’estimer la prevalence des differentes etiologies de SMG inexpliquees a l’echelle de la France. Nous presentons ici les donnees descriptives preliminaires. Patients et methodes Depuis septembre 2015, tout patient ≥ 15 ans, refere a un hematologue ou un interniste des 117 hopitaux participant, avec une SMG inexpliquee (longueur craniocaudale ≥ 13 cm a l’imagerie abdominale) a ete invite a participer. Une SMG inexpliquee est definie si le premier bilan clinique base sur l’interrogatoire, l’examen clinique et les analyses biologiques de routine (NFS, frottis sanguin, reticulocytes, enzymes hepatiques) est negatif. L’inclusion cible est de 500 patients. La derniere visite est prevue 12 mois apres l’inclusion ou avant si une cause est identifiee. Comme il n’existe pas de definition precise de SMG, l’application Splenocalc [1] , a ete utilisee pour confirmer la SMG. Resultats En mai 2018, 190 patients (58 % d’homme) ont ete inclus avec un âge median de 53 ans (16–94 ans), une longueur mediane de rate de 15 cm (13–30 cm), une structure splenique en majorite homogene (88.6 %). Trois patients avec un diagnostic evident suite au bilan initial (n = 2, leucemie lymphoide chronique, n = 1, sarcoidose) n’auraient pas du etre inclus. Un total de 129 patients (68 %) a complete l’etude. Parmi eux, 72 patients (56 %) ont un diagnostic pose. Cinq categories ont ete identifiees : hemopathies malignes lymphoides (n = 20; 27.8 %; âge median : 66) ; maladies auto-immunes (n = 15 ; 20.8 % ; âge median : 48) ; hemopathies malignes myeloides (n = 10 ; 13.9 % ; âge median : 72) ; hypertensions portales (n = 10 ; 13.9 % ; âge median : 62) et autres diagnostics comprenant les MSL (n = 17 ; 23.6 % ; âge median : 40). Les patients avec un diagnostic pose sont significativement plus âges (âge median : 61.5 ans vs. 45.0 ans, p = 0.002) et la SMG est plus grande (longueur moyenne : 16.7 ± 3.2 vs. 15.6 ± 2.3 cm ; p = 0.05) que ceux sans diagnostic. En utilisant l’application Splenocalc® (ajustement sur la taille et le sexe), 87.7 % ont une SMG confirmee, 9.5 % ont des resultats non interpretables (taille ou poids en dehors des abaques) et 8 patients (4.5 %) n’ont pas de SMG confirmee dont 2 patients avec un diagnostic etabli (lymphome et tuberculose). Conclusion L’âge plus eleve et une rate plus massive semblent etre associes a un diagnostic final de certitude. Cependant, sur ces donnees preliminaires, 44 % des patients avec une SMG inexpliquee demeurent sans diagnostic a 12 mois. Le seuil de ≥ 13 cm de longueur de rate semble fiable 9 cas sur 10 ont ete confirmes par l’application Splenocalc®. L’analyse finale sur une population totale de 500 patients permettra d’estimer la prevalence de chaque etiologie de SMG inexpliquee, de meme que les examens et tests effectues pour aboutir au diagnostic.

Introduction Analyser l’efficacite et la tolerance des biotherapies (TNFa et tocilizumab) chez les patients avec une maladie de TAKAYASU. Patients et methodes Etude retrospective europeenne de 2017-2019, incluant les patients ayant au moins 3 mois de biotherapie. La reponse au traitement etait definie en cas de NIH Resultats Deux cents trois patients ont ete inclus, avec un âge 29 ans [7–62], et 180 (89 %) de sexe feminin. Selon NUMANO, 20 patients (10 %) etait de type I, 68 (33 %) de type II, 14 (7 %) de type III, 8 (4 %) IV et 90 (44 %) de type V. Les biotherapies etaient des anti-TNF-α (n = 130 ; 63 % avec au total 173 lignes de differents types anti-TNF-α) ou tocilizumab (n = 75 ; 37 % avec 122 lignes de tocilizumab SC ou IV). Des anti-TNF-α etaient l’infliximab (n = 109), l’adalimumab (n = 45), l’etanercept (n = 5), golimumab (n = 8), certolizumab (n = 5) et parmi les 122 lignes de tocilizumab, IV dans 96 et SC dans 26 cas. Une reponse complete (NIH Conclusion Cette serie europeenne permet de decrire les profils de reponse et de tolerance des differentes biotherapies au cours de la maladie de TAKAYASU.

Background:Nervous system involvement is considered to be rare in systemic sclerosis (SSc). Its prevalence is highly variable in SSc cohort studies and its prognosis is not well established.Objectives:To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) and central nervous system (CNS) disease in a cohort of systemic sclerosis patients.Methods:We have carried out a retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital, south west France. We included patients who met the following inclusion criteria: being over 18 years of age on diagnosis, meeting the ACR /EULAR 2013 classification criteria, being diagnosed after 01/01/1966 and before 31/12/2018, at least 12 months of follow-up. Patients were followed until 31/12/2019. Nervous system involvement associated with SSc was included when there was involvement on or after diagnosis and after exclusion of all other causes. Only symptomatic clinical involvement was included. Ischemic or hemorrhagic strokes were excluded. We calculated the incidence of CNS and/or PNS disease during the follow-up period per 1,000 person-years. Kaplan-Meier curves were plotted to determine the cumulative incidence of nervous system disease. We evaluated associated factors of CNS and/or PNS disease using multivariable Cox regression.Results:Of 447 SSc patients, 79.8% were female, 68 (15%) were diffuse cutaneous SSc, 342 (77%) were limited cutaneous SSc and 37 (8%) were sine scleroderma SSc. The mean ± SD age at diagnosis was 52.9 ± 14.3 years.During the study period, 82 (18%) patients experienced a PNS disease, 29 (6%) a CNS disease. The incidence was 28 per 1,000 patient-years of any nervous system disease, with 22 per 1,000 patient-years and 6 per 1,000 patient-years of PNS disease and CNS disease, respectively. The most frequent were carpal tunnel syndrome (63%) and polyneuropathies (12%) for PNS disease, and headache (45%) and seizures (10%) for CNS disease.Three significant independent associated factors with PNS disease occurrence were identified using multivariable Cox regression: BMI>23.1kg/m2(HR = 1.06 [1.01-1.12]), joint involvement (HR = 2.7 [1.3-5.5]), and an alteration in the left ventricular ejection fraction (HR = 3.8 [1.4-10.3]).Four significant independent associated factors with CNS disease occurrence were identified: age > 54 years (HR = 2.5 [1.1-6.0]), positive anti-PmScl testing (HR = 6.4 [1.5-28.2]), Caucasian origin (HR = 0.2 [0.1-0.5]) and hemoglobin < 12g/dl (HR = 0.2 [0.04-0.8]).Nervous system disease occurrence did not appear to have a negative impact on the survival of SSc patients (log-rank p=0.56).Conclusion:This study shows that specific nervous system disease in SSc is not uncommon and does not appear to increase mortality, but it could have an impact on functional prognosis and needs to be monitored.Disclosure of Interest:None declared

Introduction Cette etude francaise retrospective multicentrique a eu comme objectif d’analyser l’atteinte articulaire au cours de la sarcoidose aigue et chronique afin de mieux caracteriser l’attitude therapeutique et comparer l’efficacite de differents traitements. Patients et methodes Tout patient (≥ 18 ans) avec un diagnostic de sarcoidose prouvee cliniquement, radiologiquement et selon les criteres histologiques et avec une atteinte articulaire avec presence d’au moins une synovite clinique et/ou echographique a ete inclus. Resultats Un total de 39 patients avec arthropathie sarcoidosique a ete inclus, dont 20 patients avec une forme aigue (syndrome de Lofgren). La presentation de l’atteinte articulaire ainsi que le DAS28-CRP, utilise comme marqueur d’activite de l’atteinte articulaire, n’etaient pas significativement differents dans les deux formes cliniques aigue et chronique. L’atteinte oculaire etait significativement plus frequente chez les patients avec une forme chronique a difference des atteintes cutanees plus frequentes en cours de sarcoidose aigue. Il n’y avait pas de difference des taux de CRP et de ECA dans les deux groupes (mediane 27 mg/L [1–180] versus 15 mg/L [1–271] ; p-value = 0,2). Pour les 39 patients, 89 lignes therapeutiques ont ete utilisees pendant un suivi median de 18 [3–264] mois. La premiere ligne therapeutique a consiste en steroides seuls (18 %) ou avec hydroxychloroquine (12 %), methotrexate (9 %), ou autres strategies therapeutiques immunosuppressives (3 %). Les corticoides, l’hydroxychloroquine, le methotrexate et infliximab etaient associes avec une reduction significative du nombre d’articulations gonflees et douloureuses et avec un effet significatif d’epargne cortisonique. Aucune difference n’a ete retrouvee sur la reponse articulaire en utilisant un score de propension concernant les differents regimes therapeutiques. Conclusion L’atteinte articulaire dans la sarcoidose peut avoir deux presentations phenotypiques differentes, aigue et chronique. Dans la forme chronique ou dans les formes corticoresistantes ou corticodependantes de sarcoidose avec atteinte articulaire, des traitements immunosuppresseurs comme le methotrexate ou l’hydroxycloroquine semblent avoir une efficacite similaire.