Efficacy of Anakinra for Various Types of Crystal-Induced Arthritis in Complex Hospitalized Patients: A Case Series and Review of the Literature

International audienceBackground. There are few data on anakinra use after failure of conventional medications for crystal-induced peripheral arthritis and/or crowned dens syndrome among complex hospitalized patients. Methods. We retrospectively analyzed the outcome of six patients affected with subacute crystal-induced arthritis who had received anakinra in second or third line therapy, including three patients with crowned dens syndrome and three others with gouty arthritis. Patients' comorbidities, reasons for anakinra use and associated drugs, and outcomes were recorded. Results. All patients presented with elevated inflammatory syndrome, systemic symptoms with poly/oligoarthritis. Except for absolute contraindications, all patients were previously treated with full or decreased dose of NSAID, colchicine, and/or glucocorticoids, with unsatisfactory response. All three gouty patients exhibited complete responses in all acute involvements under anakinra within 3 to 5 days, including one of them who needed the reintroduction of colchicine treatment that was previously unsuccessful. Crowned dens syndrome patients, including two with pseudogout and one with subacute hydroxyapatite deposition disease, needed 9 to 11 days to achieve complete response. Tolerance to anakinra was good. Conclusion. In case series of complex hospitalized patients, anakinra showed good activity in crowned dens syndrome and associated crystal-induced peripheral arthritis, with longer treatment duration than in gouty arthritis

Intra-Alveolar Haemorrhage Complicating IgA Vasculitis: A Case Report, Literature Review and Discussion of Treatment

Introduction: Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis with IgA-dominant immune deposits. IgAV frequently involves the skin, gastrointestinal tract, joints and kidneys. In contrast to other types of small-vessel vasculitis, IgAV is rarely complicated by intraalveolar haemorrhage (IAH). Methods/Results: We describe a patient with relapsing bladder cancer who presented with IAH during the course of IgAV successfully treated with corticosteroids alone. Conclusion: This case report reminds us that IgAV can manifest with IAH. There are no robust data to support the systematic use of cyclophosphamide or plasma exchange as first-line therapy for IgAV with IA

Policy and choices of plasma-derived versus recombinant products in haemophilia treatment : safety issues and the instutional, industrial, medical and psycho-social drivers

Introduction et problématiques: Les politiques et choix des produits plasmatiques (PP) ou recombinants (PR) en hémophilie divergent selon les pays en dépit de recommandations internationales consensuelles. A la différence d'autres pays, la politique française en ce sens ne fait aucune priorité de choix entre les PP versus les PR en dépit de l'avènement de variante (v) de la maladie de Creutzfeldt-Jakob (MCJ) dont le risque transfusionnel affecté à ces premiers est jugé être infinitésimal. L'adhésion des soignants et des patients à cette politique ainsi que les répercussions psychologiques des retraits de lots de PP pour le risque de vMCJ sur ces derniers, sont inconnues et suscitent des questions éthiques. Méthodologie: Des enquêtes mono et multicentriques ont été menées auprès de patients et médecins français afin d'évaluer leurs sentiments envers la politique de maintien sans restriction des PP et celle de leurs procédures de retrait de lots. Une discussion des problématiques éthiques inhérentes est conduite à partir des rapports des expertises scientifiques et de nos propres données. Résultats: L'analyse des politiques et choix des PP versus PR en hémophilie a permis d'identifier un modèle français aux côtés des trois modèles nord-américain, britannique et européen. Ce premier modèle apparaît être le moins astreignant alors que la France pointe à la haute deuxième place de la prévalence mondiale de la vMCJ après le Royaume Uni qui a tout simplement banni la prescription des PP pour les hémophiles, à la faveur des PR dénués de ce risque. Nous avons identifié nombre de tensions dans la prise en charge des patients et dans la gestion des risques de ces produits en France. Celles-ci sont principalement le fait de quatre problématiques qui correspondent à des paradoxes français de la politique de gestion de risque de ces produits, notamment: 1) multiplication injustifiée des procédures de retrait de lot de PP, essentiellement en rapport avec les formes sporadiques de la MCJ pourtant non transmissibles par ce biais. 2) absence de formalisation précise de l'organisation des procédures de retrait de lot dont les sources, les méthodes, les moyens et les délais de contact des médecins et leur relais aux patients sont divers et variés, ce qui témoigne d'une certaine improvisation et d'un cafouillage dans ce processus. 3) maintien sans restriction d'indication ni de source des PP. 4) information systématique traumatisante à posteriori des patients à l'occasion des retraits de lots pour ce risque très infime, au lieu d'une approche au cas par cas. Les problématiques éthiques induites sont: traumatisme psychologique rémanent des patients et leur famille à l'occasion de l'information, mal conduite et mal comprise, du risque de la vMCJ lors des procédures de retrait de lots de PP conduisant à leur switch massif pour des PR; inefficacité de ces procédures à éliminer les lots incriminés car souvent déjà consommés; diminution drastique, voire bannissement de la prescription des PP; vécu difficile et désapprobation majoritaire des médecins de la politique du maintien sans restriction des PP et des procédures de retrait de lot; faible respect des procédures de retrait de lots, appliquées de façon confuse et inégale; crise de confiance des patients et médecins envers cette politique; sentiments de conflits d'intérêts des autorités politiques en rapport avec un protectionnisme supposé de l'industrie nationale de fabrication des PP en l'absence de motifs solides à leur maintien sans restriction à des indications précises. Conclusion: Un recadrage institutionnel de procédures de retrait de PP en France apparait nécessaire, celles-ci étant majoritairement injustifiées car relatives aux formes sporadiques de la MCJ. La limitation de l'usage des PP à des indications spécifiques nécessite d'être discutée à l'heure de la grande disponibilité des PR. De larges enquêtes et réflexions aideraient aux futures orientations de la politique nationale.Introduction and issues: The policy and local choices of plasmatic (PP) or recombinant products (RP) in hemophilia vary according to the country, despite consensus international recommendations. In contrast to other countries, French policy in this context gives no priority in the choice between plasmatic and recombinant ones, despite the appearance of variant Creutzeldt-Jakob disease (vCJD) involving risks in transfusions, albeit miniscule, in the former. Caregivers and patient adherence to this policy and its likely psychological impact on them, are unknown and raise ethical questions. Methodology: Single- and multi-site surveys of French doctors and patients were carried out to evaluate their feelings about the French policy of unrestricted continued use of plasmatic products and their procedures of batch recall. A discussion of inherent ethical problems is conducted, based on reports of scientific expertise and our own data. Results: This analysis allowed a French model to be defined and compared to the North American, British, and European ones. This French model seems to be the least strict in terms of the use of PP, while France is in second place in terms of the worldwide prevalence of vCJD, just after U.K who have simply banned the prescription of these first products for hemophiliacs in favor of RP, which are risk-free. We identified many ethical tensions in the management of patients and in risk management of these products in France. These are mainly due to four issues that correspond to the paradoxes of French policy of hemophilia product risk management, including: 1) unjustified multiplication of recall procedures concerning batches of PP, primarily those related to sporadic forms of CJD even though incommunicable through it; 2) lack of accurate formalization concerning the organization of batch removal procedures, the sources, methods, means and time of contact for physicians and their patients that are very varied, reflecting some improvisations and a mess in the process; 3) maintaining of unrestricted indication and source of PP; 4) systematic retrospectively traumatic information for patients, especially during the batch recalls for this very small risk, rather than a case-by-case basis. The induced ethical issues are : residual psychological trauma for patients and their families on the occasion of information, poorly managed and poorly understood, the risk of vMCJ during batches removal procedures of PP leading to their massive switch RP ; ineffectiveness of these procedures to remove incriminated batches since they are often consumed; drastic reduction or even banishment of the prescription of PP; difficult experience and majority disapproval of physicians maintaining unrestricted PP and batch removal procedures; low respect for batch removal procedures, applied unevenly; crisis of confidence of patients and physicians concerning this policy; feelings of conflict of interest from political authorities in connection with an alleged protectionism of the domestic industry manufacturing PP in the absence of strong reasons to their retention without restriction to specific indications. Conclusion: An institutional reframing of PP recall in France appears to be necessary, these being mainly unjustified since relative to sporadic CJD forms. Restrictions on the use of PP at specific directions need to be discussed at this time of high availability of RP. Extensive surveys would help the future direction of national policy

Syndrome de la dent couronnée (un diagnostic à connaître)

PARIS6-Bibl. St Antoine CHU (751122104) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

An Updated Review of Cardiovascular Events in Giant Cell Arteritis

Giant cell arteritis (GCA) is a systemic vasculitis with a direct and indirect increased risk of acute and chronic vascular events, affecting large and medium vessels, and responsible for most of the morbidity and mortality of this disease. We aimed in this review to provide an updated synthesis of knowledge regarding cardiovascular events observed in GCA. By definition, GCA patients are over 50 and often over 70 years old, and subsequently also present age-related cardiovascular risk factors. In addition, the systemic and vascular inflammation as well as glucocorticoids (GC) probably contribute to an accelerated atherosclerosis and to vascular changes leading to arterial stenoses and aortic dilations and/or dissections. GCA-related ischemic complications, especially ophthalmologic events, stroke or myocardial infarcts are mostly observed within the first months after the diagnosis, being mainly linked to the vasculitic process. Conversely, aortic complications, including dilations or dissections, generally occur several months or years after the diagnosis, mainly in patients with large-vessel vasculitis. In these patients, other factors such as atherosclerosis, GC-related endothelial damage and vascular wall remodeling/healing probably contribute to the vascular events. GCA management includes the detection and treatment of these previous and newly induced cardiovascular risk factors. Hence, the use of cardiovascular treatments (e.g., aspirin, anticoagulation, statins, anti-hypertensive treatments) should be evaluated individually. Aortic structural changes require regular morphologic evaluations, especially in patients with previous aortitis. The initial or secondary addition of immunosuppressants, especially tocilizumab, an anti-IL-6 receptor antibody, is discussed in patients with GCA-related cardiovascular complications and, more consensually, to limit GC-mediated comorbidities

FDG PET-CT as a powerful tool for diagnosing and monitoring treatment outcomes of relapsing polychondritis

International audienc

Infections and AA amyloidosis: An overview

International audienceBackground: Amyloidoses are a heterogeneous group of systemic diseases characterised by extracellular accumulation of insoluble amyloid fibrils derived from unfolded proteins. Inflammatory (AA) amyloidosis can complicate various inflammatory disorders that are associated with a sustained acute phase response and serum amyloid A (SAA) protein overproduction. Chronic infections were the first recognised cause of amyloidoses. However, with the better management of underlying diseases, the frequency of AA amyloidosis is decreasing.Purpose: The aim of this overview was to discuss the several infections associated with AA amyloidosis and the relative frequency of infections as aetiological factors.Methods: A search of the literature was performed using the PubMed database using the MeSH terms "Amyloidosis" and "Infections," from inception to December 31st, 2019. Articles written in other languages than English or French were excluded.Results: The frequency of AA amyloidosis secondary to infections decreased from more than 50% to less than 20% after the 2000s, with a parallel increase in the frequency of AA amyloidosis secondary to inflammatory diseases and to an unknown cause.Conclusion: Whereas new antibiotics have been developed and sanitary conditions are better, infections still represent 5%-30% of the causes of AA amyloidosis, including in developed countries. These data argue for better screening of chronic infections to prevent AA amyloidosis and the development of new strategies to manage recurrent infections