H. Maillard-Lefebvre

Soluble receptor for advanced glycation end products: a new biomarker in diagnosis and prognosis of chronic inflammatory diseases

The formation of advanced glycation end products (AGEs) is a result of the non-enzymatic reaction between sugars and free amino groups of proteins. AGEs, through interacting with their specific receptor for AGEs (RAGE), result in activation of pro-inflammatory states and are involved in numerous pathologic situations. The soluble form of RAGE (sRAGE) is able to act as a decoy to avoid interaction of RAGE with its pro-inflammatory ligands (AGEs, HMGB1, S100 proteins). sRAGE levels have been found to be decreased in chronic inflammatory diseases including atherosclerosis, diabetes, renal failure and the aging process. The use of measuring circulating sRAGEs may prove to be a valuable vascular biomarker and in this review, we describe the implications of sRAGE in inflammation and propose that this molecule may represent a future therapeutic target in chronic inflammatory diseases.

Graft-versus-host disease-related polymyositis

Chronic graft-versus-host disease (GVHD) sometimes mimics autoimmune diseases. We report the case of a 39-year-old patient who presented atypical polymyositis without elevated creatinine phosphokinase, related to a chronic GVHD following interruption of immunosuppressive treatment. Treatment with cyclosporine and corticosteroids resulted in complete and sustained remission of the polymyositis. The symptoms of chronic GVHD-related polymyositis are indistinguishable from those of idiopathic polymyositis. The context of transplantation and a decrease or interruption of prophylaxis suggest the diagnosis of GVHD-related polymyositis, especially if other manifestations of GVHD are associated. A suitably adapted treatment (association of corticotherapy and cyclosporine) improves polymyositis, and in most cases, a normal clinical state is achieved even if the symptoms were severe.

Hallucinations visuelles et maladie de Horton : à propos du syndrome de Charles Bonnet

In patients with visual hallucinations, diagnostic strategy is unclearly codified. In patients known to have giant cell arteritis, the main diagnostic assumption is disease relapse. Indeed, this should lead to rapid corticosteroid therapy. However, the Charles Bonnet syndrome, that is a poorly known etiology of visual hallucinations usually observed in elderly people, should be part of the differential diagnosis. We report a 87-year-old woman, with a 2-year history of giant cell arteritis who was admitted with an acute onset of visual hallucinations and who met all the criteria for Charles Bonnet syndrome.

Recurrent limb and facial oedema with R92Q mutation: A non-febrile late-onset tumour necrosis factor receptor-associated periodic syndrome (TRAPS)?

© 2018 Elsevier Mas rrent limb and facial ema with R92Q mutation: n-febrile late-onset tumour osis factor receptorheterozygous mutation, disclosing probable non-febrile lateonset TRAPS. TRAPS usually begins during the first two decades [1]. Some cases of late-onset TRAPS have been described, with a median age of 32 years [2]. However, only few patients with an onset after 45 years have been described [2,3]. These phenotype tio e en yp of tio Le tt e rs to th e e d i

Faut-il craquer pour du pop-corn ou pour Tony ?

Une jeune femme de 22 ans présentait des douleurs peliennes sans fièvre en décembre 2006. L’examen clinique tait normal en dehors d’une adénopathie axillaire gauche. Un remier bilan, réalisé en ville, retrouvait un syndrome inflammaoire modéré avec CRP à 16 mg/l et une discrète augmentation u CA 125. L’échographie abdominopelvienne montrait une ame liquidienne rétro-utérine. Un scanner abdominopelvien onfirmait l’ascite, surtout pelvienne, et notait une hépatoméalie homogène modérée. Au niveau thoracique, il existait un anglion de la loge de Baréty et quelques ganglions infracenimétriques de la fenêtre aortopulmonaire. Il était décidé de éaliser une cœlioscopie exploratrice qui retrouvait 250 ml de iquide sérohématique dont l’analyse révélait une cellularité aible à prédominance lymphocytaire (70 %) ; il n’y avait pas de ellules anormales ; l’examen direct et la culture étaient négatifs e même que la recherche de BK (direct et culture). Le reste de a cœlioscopie était normal. Nous étions alors en février 2007 et l’état général de la atiente s’altérait. Elle présentait maintenant des pics fébriles ccompagnés de douleurs abdominales ainsi qu’un amaigrisement de 3 kg. Il était donc réalisé une biopsie-exérèse de ’adénopathie axillaire gauche. L’anatomopathologiste mettait n évidence une transformation progressive des centres germiatifs avec architecture nodulaire ; la présence de cellules de ype popcorn cell évoquait un paragranulome hodgkinien nodu-