Thrombose veineuse ovarienne et thrombophile (à propos de 13 cas)

Les thromboses veineuses ovariennes (TVO) sont rares et de siège atypique. Les facteurs les favorisant et la place du bilan de thrombophilie sont discutés. Cette étude rétrospective cas-témoin compare les facteurs favorisants, le BT, la survie et les récidives de patientes porteuses de TVO, avec un groupe de patientes, appariées par l'âge, présentant une thrombose veineuse profonde des membres inférieurs (TVP). 13 TVO sont incluses entre 1998 et 2008 au CHU de Clermont-Ferrand, la moyenne est de 2.07 facteurs de risque de thrombose veineuse/patientes ; le post-partum est statistiquement corréléà la survenue de ces thromboses (p=0,016). Le bilan de thrombophilie du groupe TVO n'objective qu'une anomalie majeure (syndrome des antiphospholipides) et 2 anomalies mineures (mutation hétérozygotes Leiden du facteur V) sans différence statistique avec le groupe témoin. Après un suivi moyen de 41 mois pour les TVO versus 39 mois pour les TVP ; et des médianes respectives de traitement anticoagulants de 3 et 6 mois, la mortalité et le taux de récidive sont nuls dans le groupe TVO sans différence statistique avec le groupe TVP. Les TVO sont multifactorielles et ne semblent pas être un mode d'entrée dans la maladie thromboembolique veineuse récidivante. L'absence de complications et l'évolution favorable de TVO non taitées pourraient remettre en cause leur pathogénicité.CLERMONT FD-BCIU-Santé (631132104) / SudocSudocFranceF

Venous thrombosis in patients with giant cell arteritis: Features and outcomes in a cohort study

International audienceOBJECTIVES:To describe the features and outcomes of patients with giant cell arteritis who developed venous thrombosis.METHODS:Inception cohort study including 428 newly diagnosed patients of giant cell arteritis from 1976 to 2014. Clinical and biological data and outcomes were analysed by comparing patients with and without venous thrombosis.RESULTS:Twenty-six patients (6%) developed venous thrombosis, 12 of whom presented with pulmonary embolism. The mean time between the onset of giant cell arteritis symptoms and venous thrombosis occurrence was 248.8±215.0 days. No difference was observed between the two groups in clinical or laboratory data collected at diagnosis. The mean time from the start of prednisone to venous thrombosis diagnosis was 187.7±217.0 days. The average dose of prednisone at venous thrombosis onset was 21.5mg/day. The venous thrombosis group had a higher number of glucocorticoid-related adverse effects (mean, 3.1 vs 1.1; P<0.0001), a higher mortality rate (58% vs 33%, P=0.01) and a higher proportion of deaths occurring during glucocorticoid treatment (31% vs 14%, P=0.03). Death was related to venous thrombosis in four patients.DISCUSSION:The occurrence of overt venous thrombosis is more than anecdotal among patients treated for giant cell arteritis. Venous thrombosis does not rely on the active phase of giant cell arteritis, but could be associated with long-term use of glucocorticoids. Because venous thrombosis may be associated with an increased mortality risk in patients with giant cell arteritis, a high index of suspicion should be applied in appropriate settings, especially in patients experiencing multiple glucocorticoid-related adverse effects

Active Immunological Profile Is Associated with Systemic Sjögren’s Syndrome

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T cell Polarization toward T(H)2/T(FH)2 and T(H)17/T(FH)17 in Patients with IgG4-related Disease

International audienceIgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder involving virtually every organ with a risk of organ dysfunction. Despite recent studies regarding B cell and T cell compartments, the disease's pathophysiology remains poorly understood. We examined and characterized subsets of circulating lymphocytes in untreated patients with active IgG4-RD. Twenty-eight consecutive patients with biopsy-proven IgG4-RD were included in a prospective, multicentric study. Lymphocyte's subsets were analyzed by flow cytometry, with analysis of T(H)1/T(H)2/T(H)17, T-FH cells, and cytokine release by peripheral blood mononuclear cells. Results were compared to healthy controls and to patients with primary Sjogren's syndrome. Patients with IgG4-RD showed an increase of circulating T regulatory, T(H)2, T(H)17, and CD4(+)CXCR5(+)PD1(+) TFH cell subsets. Accordingly, increased levels of IL-10 and IL-4 were measured in IgG-RD patients. TFH increase was characterized by the specific expansion of T(FH)2 (CCR6(-)CXCR3(-)), and to a lesser extent of (T(FH)17 (CCR6(+)CXCR3(-))) cells. Interestingly, CD4(+)CXCR5(+)PD1(+) TFH cells normalized under treatment. IgG4-RD is characterized by a shift of circulating T cells toward a T(H)2/T(FH)2 and T(H)17/T(FH)17 polarization. This immunological imbalance might be implicated in the disease's pathophysiology. Treatment regimens targeting such T cells warrant further evaluation

Peroral endoscopic pyloromyotomy is efficacious and safe for refractory gastroparesis: prospective trial with assessment of pyloric function

International audienceBackground Gastroparesis is a functional disorder with a variety of symptoms that is characterized by delayed gastric emptying in the absence of mechanical obstruction. A recent series of retrospective studies has demonstrated that peroral endoscopic pyloromyotomy (G-POEM) is a promising endoscopic procedure for treating patients with refractory gastroparesis. The aim of this prospective study was to evaluate the feasibility, safety, and efficacy of G-POEM.Methods 20 patients with refractory gastroparesis (10 diabetic and 10 nondiabetic) were prospectively included in the trial. Patients were treated by G-POEM after evaluation of pyloric function using an endoscopic functional luminal imaging probe. Clinical responses were evaluated using the Gastroparesis Cardinal Symptom Index (GCSI), and quality of life was assessed using the Patient Assessment of Upper Gastrointestinal Disorders – Quality of Life scale and the Gastrointestinal Quality of Life Index scores. Gastric emptying was measured using 4-hour scintigraphy before G-POEM and at 3 months.Results Feasibility of the procedure was 100 %. Compared with baseline values, G-POEM significantly improved symptoms (GCSI: 1.3 vs. 3.5; P < 0.001), quality of life, and gastric emptying (T½: 100 vs. 345 minutes, P < 0.001; %H2: 56.0 % vs. 81.5 %, P < 0.001; %H4: 15.0 % vs. 57.5 %, P = 0.003) at 3 months. The clinical success of G-POEM using the functional imaging probe inflated to 50 mL had specificity of 100 % and sensitivity of 72.2 % (P = 0.04; 95 % confidence interval 0.51 – 0.94; area under the curve 0.72) at a distensibility threshold of 9.2 mm2/mmHg.Conclusion G-POEM was efficacious and safe for treating refractory gastroparesis, especially in patients with low pyloric distensibility

Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients

Objective A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-relapsing polychondritis (VEXAS-RP).Methods Patients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP.Results Compared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, p&lt;0.001) and were older at diagnosis (66 vs 44 years, p&lt;0.001). They had a greater prevalence of fever (60% vs 10%, p&lt;0.001), of skin lesions (82% vs 20%, p&lt;0.001), of ocular involvement (57% vs 28%, p=0.01), of pulmonary infiltrates (46% vs 0%, p&lt;0.001), of heart involvement (11% vs 0%, p=0.0336) and with higher median C-reactive protein levels (64 mg/L vs 10 mg/L, p&lt;0.001). Seventy-five per cent of the patients with VEXAS-RP had myelodysplastic syndrome (MDS) versus none in I-RP group. The glucocorticoids use, and the number of steroid sparing agents were similar in both groups, but patients with VEXAS-RP had more frequent refractory disease (remission obtained in 27% vs 90%, p&lt;0001). VEXAS-RP was associated with higher risk of death: six patients (11%) died in the VEXAS-RP group after a median follow-up of 37 months and none in the I-RP group after a median follow-up of 92 months (p&lt;0.05).Conclusion We report the largest cohort of VEXAS-RP, characterised by high prevalence of male sex, fever, skin lesion, ocular involvement, pulmonary infiltration, heart involvement, older age and MDS association

Efficacy and Tolerance of Rituximab in IgG4-Related Disease: A retrospective Multicentric Study in 24 patients

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