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Background: Interleukine 6 (IL-6) is the most important cytokine involved in malignant plasma cells growth and survival., Aim: To analyse bone marrow plasma cells IL6 receptor gene expression in both multiple myeloma patients at diagnosis and healthy bone marrow donors., Methods: Clinical and biological patients' features and responses to Dexamethasone-Thalidomide induction therapy were gathered. 47 patients and 16 case controls were analyzed: Bone marrow plasma cells were isolated; and IL6 receptor gene expression was quantified using Taqman quantitative PCr technology and 2-ΔCT formula., Results: Quantitative and qualitative IL6 receptor gene expression were negatively correlated with the degree of response to therapy (p= 0.02). In this study, plasma cells IL6 receptor gene expression seems to be decisive in predicting the response to treatment., Conclusion: Understanding the mechanisms involved in plasma cells IL6 receptor gene expression may offer a better appreciation of the physiopathologic and anti-oncogenic ways of drug resistance in multiple myeloma and consequently the discovery of new specific drugs.

Multiple myeloma (MM) is a still incurable adult's severe hematologic malignancy. It is characterized by deregulation of several cytokines and their receptors. Among these cytokines, Insulin growth factor 1 (IGF1) and its receptor (IGF1-R) are well documented as major factor of malignant plasma cells growth and survival in multiple myeloma. The objective of this study was to analyze the expression of IGF1-R in multiple myeloma at diagnosis in correlation with clinical and biological data. IGF1-R gene plasma cells expression was studied in 47 patients and 17 controls by Taqman technology RT-PCR. IGF1-R gene was down expressed in the malignant plasma cells of MM patients at diagnosis compared to normal plasma cells, isolated from healthy donors (p = 0.01). Expression decrease was accentuated in the disease advanced stage IIIB. A negative correlation was found between IGF1-R malignant plasma cells expression and the percentage of bone marrow invasion (p = 0.03). Bone marrow infiltration greater than 30% was significantly associated with a low level of IGF1-R gene expression (p = 0.04). Our results suggest that the decreased expression of IGF1-R by malignant plasma cells is a prognostic factor associated with severe disease. Understanding of mechanisms involved in IGF1-R expression negative regulation may contribute to the discovery of new targets therapy in myeloma. the discovery of new targets therapy in myeloma.

We report a patient with Sjögren's syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmer's test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids.

Objective: The aim of this study was to investigate mucosal expression of INF-gamma and IL-10 in patients with Crohn's disease (CD) or ulcerative colitis (UC)., Methods: Fourteen patients with CD and 11 patients with ulcerative colitis participated and 7 healthy subjects were also included. Study of the mucosal expression of INF-gamma and IL-10 was conducted using biopsies from healthy and damaged colons, using the inverse transcription and genetic amplification (RT-PCR) technique in real time (Taqman). Our results were expressed as ratio between messenger cytokine (mRNA) levels and ribosomal RNA level of a reference molecule (rRNA 18S), then multiplied by 108., Results: In the cases of Crohn's disease, the mucosa expressed increased INF-gamma and IL-10 compared with controls (respective medians: 23.03 vs. 1.87 p=0.04 and 20.61 vs. 2.13 p=0.08). A strong positive correlation was found in the mucosal expression of IL-10 and INF-gamma during CD (r=0.9 p<0.0001). In contrast, in patients with UC, the expression of INF-gamma and IL-10 were comparable to those observed in the controls (7.18 vs. 2.18 p=0.36 and 3.66 vs. 1.87 p=0.44)., Conclusion: During Crohn's disease, the expression of both IL-10 and INF-gamma was increased and strongly correlated, compared with the controls.

Purpose: To describe epidemiological and clinical characteristics of neurological involvement in Behçet's disease (BD) and to determine a subgroup of patients at high risk for this complication., Patients and Methods: The medical notes of 105 patients with BD fulfilling the criteria of the international Study Group for Behçet's disease were retrospectively reviewed. Patients were divided into two groups according to the presence (group 1) or not (group 2) of neurological and/or psychiatric involvement attributable to BD. The epidemiological, clinical and genetic (HLA B51 and MICA 6 frequency) features in the two groups were analysed and compared using the Kruskall-Wallis and the chi-square tests., Results: Twenty-seven patients (25.7%) had clinical evidence of neurological involvement. They were 20 men and 7 women. The mean age at neurological onset was 34.26 +/- 8.79 years. Nineteen patients (70.3%) had meningoparenchymal "MP" central nervous system involvement (brainstem: 9, hemispheric involvement: 6, spinal cord: 4, psychiatric involvement: 2, isolated pyramidal signs: 1, aseptic meningitis: 1). Seven patients (25.9%) had cerebral large vessels involvement that is cerebral angio-Behçet "CAB" (intracranial hypertension: 5 cases due to cerebral venous thrombosis: 3 and pseudotumor cerebri: 2, cerebral haemorrhage: 1, cerebral arterial thrombosis: 1). One patient (3.7%) had both "MP" and "CAB" involvement. Headache was significantly more frequent in non-parenchymal patients. In group 1, complete recovery or improvement with mild neurological impairment was seen in 13 cases, improvement with severe disability in 3 cases, worsening in 1 case, the course was stationary in 1 case and 3 patients died (11.2%). Arterial aneurysms were significantly more frequent in "CAB" subgroups than in subgroup 2., Conclusion: Frequency of neurological involvements in BD was high in our study. Arterial aneurysms seem to be a risk factor to these complications. Cerebral angio-Behçet appears to be a protector factor against uveitis.

Résumé: Le gluten est une protéine contenue dans le blé largement utilisée dans l'industrie alimentaire pour ses propriétés de texture et de goût. L'hypersensibilité au gluten non cœliaque (HGNC) est un syndrome défini par l'apparition de symptômes intestinaux et extra-intestinaux secondaires à l'ingestion de gluten et régressant après un régime sans. L'HGNC a une présentation clinique similaire à la maladie cœliaque mais s'en distingue par l'absence d'atteinte histologique de la muqueuse intestinale. La cause de l'HGNC est indéterminée mais une activité du système immunitaire inné et une augmentation de la perméabilité intestinale ont été observées. Le diagnostic est fait après exclusion d'une allergie au blé et d'une maladie cœliaque par la recherche d'anticorps spécifiques et des biopsies duodénales. Il peut être confirmé par un challenge au gluten, rarement effectué dans la pratique clinique. Contrairement à la maladie cœliaque, le risque de complications malignes et de malabsorption est absent et permet un régime avec une quantité de gluten adaptée aux symptômes. Malgré des traitements en cours d'étude, le régime sans gluten est le seul traitement recommandé de la maladie cœliaque et l'HGNC. La surveillance clinique, biologique et histologique préconisée pour la maladie cœliaque n'est pas nécessaire en cas d'HGNC. Gluten is a protein contained in wheat and widely used in the food industry for its texture and taste properties. It causes a spectrum of diseases that concerns 5% of the world's population. Non-celiac gluten sensitivity (NCGS) is a syndrome defined by the occurrence of intestinal and extra-intestinal symptoms in the days and weeks following intake of gluten that resolves after its discontinuation. NCGS has a clinical presentation similar to celiac disease but differs by the absence of histological involvement of the intestinal mucosa. The pathogenesis of NCGS is undetermined but innate immune system activity and increased intestinal permeability have been observed. The diagnosis is made after excluding wheat allergy and celiac disease by looking for specific antibodies and duodenal biopsies. It can be confirmed by a gluten challenge, which is rarely performed in clinical practice. Unlike celiac disease, the risk of malignant complications and malabsorption is absent in NCGS allowing a diet with a quantity of gluten adapted to symptoms. Despite treatments being studied, the gluten-free diet is the only recommended treatment for celiac disease and NCGS. The biological and histological monitoring recommended in celiac disease is not necessary in the case of NCGS. [ABSTRACT FROM AUTHOR]

Résumé: La maladie de Behçet est une vascularite systémique avec un grand polymorphisme clinique. L'atteinte vasculaire est souvent grave et peut engager le pronostic vital. Les artères et les veines de tout calibre peuvent être touchées. L'atteinte des vaisseaux digestifs est souvent grave. Nous rapportons le cas d'un jeune patient, de sexe masculin, dont la maladie de Behçet a été révélée par une ischémie digestive secondaire à une thrombose de la veine mésentérique supérieure étendue à la veine porte. Behçet's disease is a systemic vasculitis with a large clinical polymorphism. Vascular involvement is often severe and can be life-threatening. Arteries and veins of any size can be affected. The involvement of the digestive vessels is often severe. We report a case of a patient whose Behçet's disease was revealed by digestive ischemia secondary to superior mesenteric vein thrombosis extended to the portal vein. [ABSTRACT FROM AUTHOR]

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Abstract: We report a patient with Sjögren''s syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmer''s test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids. [Copyright &y& Elsevier]