Spectre étiologique des hypogammaglobulinémies dans le service de médecine interne du CHU d'amiens (à propos de 467 cas)

INTRODUCTION La découverte d une hypogammaglobulinémie est un problème relativement fréquent en médecine et impose de déterminer son origine primitive ou secondaire avant de poser la question de son étiologie. Le but de cette étude est de déterminer les étiologies des hypogammaglobulinémies au sein d un service de Médecine Interne et d étudier les complications infectieuses associées à cette anomalie biologique pour appréhender l impact du traitement substitutif par immunoglobulines polyvalentes. PATIENTS ET METHODES Il s agit d une étude rétrospective monocentrique menée dans le service de Médecine Interne du CHU d Amiens étudiant toutes les électrophorèses des protéines (EPP) ayant mis en évidence une hypogammaglobulinémies < 6g/l entre le 1er janvier 2004 et le 31 décembre 2012. RESULTATS 467 patients ont été inclus. La moyenne d âge était de 67,3 ans, le sex ratio de 0,72. 159 patients, ont été exclus des analyses car nous ne disposions pas d EPP de contrôle. 185 patients présentaient une hypogammaglobulinémie persistante et 123 patients une hypogammaglobulinémie transitoire. Dans le groupe hypogammaglobulinémies persistantes , le taux médian de gammaglobulines était de 4,71g/l, 48,6% des patients présentaient une hémopathie lymphoproliférative, 15,7% étaient sous traitement immunosuppresseur et 13,5% présentaient un DICV avec la particularité de compter 7 patients âgés de plus de 60 ans. Pour quelques patients aucune étiologie n a pu être identifiée et enfin pour 3,8% une association avec des pathologies chroniques variées a été identifiée. Dans le groupe hypogammaglobulinémies transitoires , 46,3% des patients suivaient un traitement immunosuppresseur, 17,9% étaient dénutris. Pour 8,9% des patients, aucune étiologie n a pu être retenue, en revanche, la consommation de tabac, d alcool et la prise de statines ont été souvent retrouvées chez ces patients. Enfin, 35,1% des patients avec une hypogammaglobulinémie persistante ont présenté des complications infectieuses, qui s amendaient dès la mise en place d une substitution par immunoglobulines polyvalentes. Une seule infection opportuniste a été rapportée, il s agissait d une pneumocystose. Il n a pas pu être établi de seuil de gammaglobulines au-delà duquel il n existe plus de complications infectieuses. DISCUSSION et CONCLUSION Cette étude à l effectif conséquent nous amène au constat de la nécessité de former et d informer les cliniciens sur la prise en charge des hypogammaglobulinémies car bon nombre passent inaperçues. D autre part, ce travail a permis d identifier une entité jamais décrite à ce jour le DICV de révélation tardive qui pourrait donner lieu à de futurs travaux de recherche et a permis de mettre en évidence des associations inédites de causes toxiques et d hypogammaglobulinémies.AMIENS-BU Santé (800212102) / SudocSudocFranceF

Corticosteroids and immunosuppressive agents for idiopathic recurrent pericarditis

International audienceRecurrent pericarditis is a frequent and troublesome complication of acute pericarditis. Aspirin or non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine are the mainstay of therapy but few data is available on second-line treatment. We retrospectively analyzed 13 patients, 7 females (54%), median age 40 years, with a median of 4 (IQR 1-6) recurrences per patient despite a well conducted first-line treatment and a median follow-up of 59 months (IQR 38-70). Ten patients received corticosteroids as second-line therapy; 6 out of 10 responded to this therapy while 4 needed the addition of azathioprine. Three other patients received an immunosuppressive agent as second-line therapy (azathioprine, methotrexate, mycophenolate mofetyl). Overall, the mean frequency per month (± SD) of pericarditis recurrences was 0.69 (± 0.40) with aspirin/NSAIDs and colchicine, 0.22 (± 0.34) with corticosteroids alone and 0.01 (± 0.04) with immunosuppressive agents (p < 10-4). Immunosuppressive agents including azathioprine, methotrexate and mycophenolate mofetyl seem efficacious and well tolerated in patients with idiopathic recurrent pericarditis unresponsive to corticosteroids, corticosteroids-dependent or when corticosteroids side effects are judged unacceptable

Cryoglobulinemia Vasculitis

International audienceCryoglobulinemic vasculitis (CryoVas) is a small vessel vasculitis involving mainly the skin, the joints, the peripheral nerve system and the kidneys. Type I CryoVas are single monoclonal immunoglobulins related to an underlying B-cell lymphoproliferative disorder. Type II and III cryoglobulins, often referred to as mixed cryoglobulinemia, consist of polyclonal IgG with or without monoclonal IgM with rheumatoid factor activity. Hepatitis C virus (HCV) infection represents the main cause of mixed CryoVas. The 10-year survival rates are 63%, 65% and 87% in HCV-positive mixed CryoVas, HCV-negative mixed CryoVas and type I CryoVas patients, respectively. In HCV-positive patients, baseline poor prognostic factors include the presence of severe liver fibrosis, and central nervous system, kidney, and heart involvement. Treatment with antivirals is associated with a good prognosis whereas use of immunosuppressant (including corticosteroids) is associated with a poor outcome. In HCV-negative patients, pulmonary and gastrointestinal involvement, renal insufficiency and age>65 years are independently associated with death. Increased risk of lymphoma should also be underlined. Treatment of type I CryoVas is that of the hemopathy; specific treatment also include plasma exchange, corticosteroids, rituximab and ilomedine. In HCV-CryoVas with mild to moderate disease, an optimal antiviral treatment should be given. For HCV-CryoVas with severe vasculitis (i.e. worsening of renal function, mononeuritis multiplex, extensive skin disease, intestinal ischemia…) control of disease with rituximab, with or without plasmapheresis, is required before initiation of antiviral therapy. Other immunosuppressants should be given only in case of refractory forms of CryoVas, frequently associated with underlying B-cell lymphoma

Tocilizumab-Induced Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome in Adult-Onset Still Disease: A Case Report

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Extrahepatic manifestations of chronic hepatitis C virus infection

International audienceDuring hepatitis C virus (HCV) chronic infection, extrahepatic manifestations are frequent and polymorphous. This article reports on a large cohort of patients with HCV-related autoimmune or lymphoproliferative disorders, from mixed cryoglobulinemia vasculitis to frank lymphomas. The relationship between HCV infection and such immune-related diseases has been formally demonstrated by epidemiological, clinical, immunological and pathological data, and results of therapeutic trials. More recently, other nonliver-related HCV disorders have been reported, including cardiovascular (i.e. stroke, ischemic heart disease), renal, metabolic and central nervous system diseases. For these manifestations, most evidence comes from large epidemiological studies; there is a need for mechanistic studies and therapeutic trials for confirmation. Beyond the risk of developing liver complications, that is, cirrhosis and liver cancer, patients with HCV infection have an increased risk of morbidity and mortality related to nonliver diseases. HCV chronic infection should be analyzed as a systemic disease in which extrahepatic consequences increase the weight of its pathological burden. The need for effective viral eradication measures is underlined

Transient reduction in venous susceptibility during posterior reversible encephalopathy syndrome

International audienceWe report a case of Posterior Reversible Encephalopathy Syndrome (PRES) in whichfollowed-up MRI demonstrated a transient reduction in venous signal on initial SWANimages. The progressive normalization of venous signal on D10 and D40 imagingparalleled the progressive decrease of hyperperfusion on CBF images. Decreased venoussusceptibility has never been reported in PRES; it relates most likely to a transient BOLDeffect induced by brain hyperperfusion

Tuberculosis Risk Stratification of Psoriatic Patients Before Anti-TNF-α Treatment

Psoriasis is a skin inflammatory condition for which significant progress has been made in its management by the use of targeted biological drugs. Detection of latent M. tuberculosis infection (LTBI) is mandatory before starting biotherapy that is associated with reactivation risk. Together with evaluation of TB risk factors and chest radiographs, tuberculin skin tests (TST) and/or blood interferon-γ-release assays (IGRA), like the QuantiFERON (QFT), are usually performed to diagnose M. tuberculosis infection. Using this approach, 14/49 psoriatic patients prospectively included in this study were identified as LTBI (14 TST+, induration size ≥ 10mm, 8 QFT+), and 7/14 received prophylactic anti-TB treatment, the other 7 reporting past-treatment. As the specificity and sensitivity of these tests were challenged, we evaluated the added value of an IGRA in response to a mycobacterial antigen associated with latency, the heparin-binding haemagglutinin (HBHA). All but one TST+ patient had a positive HBHA-IGRA, indicating higher sensitivity than the QFT. The HBHA-IGRA was also positive for 12/35 TST-QFT- patients. Measurement for 15 psoriatic patients (12 with HBHA-IGRA+) of 8 chemokines in addition to IFN-γ revealed a broad array of HBHA-induced chemokines for TST+QFT- and TST-QFT- patients, compared to a more restricted pattern for TST+QFT+ patients. This allowed us to define subgroups within psoriatic patients characterized by different immune responses to M. tuberculosis antigens that may be associated to different risk levels of reactivation of the infection. This approach may help in prioritizing patients who should receive prophylactic anti-TB treatment before starting biotherapies in order to reduce their number.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Biotherapies in Uveitis

International audienceNon-infectious uveitis (NIU) represents one of the leading causes of blindness in developed countries. The therapeutic strategy aims to rapidly control intra-ocular inflammation, prevent irremediable ocular damage, allow corticosteroid sparing and save the vision, and has evolved over the last few years. Anterior NIU is mostly managed with topical treatment in adults. However, for intermediate, posterior and pan-uveitis, notably when both eyes are involved, systemic treatment is usually warranted. Biotherapies are recommended in case of inefficacy or non-tolerance of conventional immunosuppressive drugs in non-anterior NIU. Anti-tumor necrosis factor alpha (anti-TNF-α) agents are by far the most widely used, especially adalimumab (ADA) and infliximab (IFX). In case of sight-threatening uveitis in Behçet's disease or in case of risk of severe recurrences, respectively IFX and ADA may be recommended as first-line therapy. Many questions are left unanswered; how long to treat NIU, how to discontinue anti-TNF-α agents, what biologic to use in case of anti-TNF-α failure? The objective of this review is to present an updated overview of knowledge on the use of biological treatments in NIU

Neuroimaging features in Posterior Reversible Encephalopathy Syndrome: A Pictorial Review

International audiencePosterior reversible encephalopathy syndrome (PRES) is a radioclinical entity associating nonspecific neurological symptoms (headache, seizures, impairment of alertness, visual disturbances...) occurring in evocative clinical condition (hypertension, eclampsia, immunosuppressor agents, systemic lupus erythematosus...). In the acute stage, the typical imaging finding is a vasogenic edema predominant in the subcortical parietal-occipital white matter.The purpose of this pictorial review is to illustrate the different neuroimaging features of PRES and present key radiological elements to assert diagnosis. In this overview, we examine the following points: the distributions of vasogenic edema, hemorrhage, the varying patterns in diffusion and perfusion, the different types of enhancement encountered and the vascular modifications demonstrated by angiography. The cause of PRES is still unknown. Nevertheless, catheter angiography, MR angiography and MR perfusion features in PRES render further insight into its pathophysiology.Follow-up imaging shows evidence of radiologic improvement in the very large majority of cases in 1 or 2 weeks, sometimes in up to 1 month. Recurrent PRES attacks are uncommon.Atypical imaging presentation should not reject the diagnosis of PRES in a compatible clinical situation