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2.
Clin Rheumatol ; 2019 Nov 23.
Artigo em Inglês | MEDLINE | ID: mdl-31760541

RESUMO

Transfusion-related acute lung injury (TRALI), defined as the onset of acute respiratory distress after blood transfusion, is a rare complication which is a leading cause of transfusion related-mortality. In this retrospective study, we report the French nationwide experience of intravenous immunoglobulin (IVIG)-related TRALI, with a literature review and analysis of management and outcome of this rare condition. With the pharmacovigilance services, we conducted a retrospective multicenter study in the French network of intensive care units with TRALI concomitant to IVIG use and pooled with data from a literature review. Overall, 17 cases have been included in this case-series, our case report, seven personal cases and nine cases from the literature review. The median age was 55 years [2-79] with 10/17 (59%) male subjects. The underlying diseases motivating IVIG infusion were neurologic diseases in 35% of cases (Guillain Barre syndrome = 2, peripheral neuropathy = 2, neurolupus = 1, myasthenia = 1), multiple myeloma with hypogammaglobulinemia (n = 2; 12%), primary hypogammaglobulinemia (n = 2; 12%), autoimmune cytopenias (n = 2; 12%), graft versus host cutaneous disease after allogeneic hematopoietic stem cell transplantation for acute myeloid leukaemia (n = 1), anti-HLA antibodies after lung transplant (n = 1), cancer-associated thrombotic thrombocytopenic purpura-haemolytic uremic syndrome (n = 1), Kawasaki disease (n = 1) and in experimental essay (n = 1). TRALI symptoms begin either after the start or during the infusion (n = 7; 41%), or after the infusion (n = 10; 59%, 10 min to 24 h). Besides respiratory distress, it was also noted shock (33%), fever (18 %), cough (18%), nausea/vomiting (18 %), chills (12%) and agitation (12%). The X-ray showed mainly bilateral alveolar opacities (n = 15; 88%). Mechanical ventilation was needed in nine cases (53%), with median 1-day duration [1-4]. Four patients (24%) died during hospitalisation in the intensive care unit. Given the increasing use of intravenous immunoglobulins, TRALI must now be discussed in cases of respiratory distress occurring during or immediately following the infusion even if this side effect remains rare.Key Points• TRALI must now be discussed in cases of respiratory distress occurring during or immediately following an infusion of intravenous immunoglobulins.

4.
Am J Hematol ; 94(12): 1314-1324, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31489694

RESUMO

Rituximab is a second-line option in adults with immune thrombocytopenia (ITP), but the estimated 5-year response rate, only based on pooled retrospective data, is about 20%, and no studies have focused on long-term safety. We conducted a prospective multicenter registry of 248 adults with ITP treated with rituximab with 5 years of follow-up to assess its long-term safety and efficacy. The median follow-up was 68.4 [53.7-78.5] months. The incidence of severe infections was only 2/100 patient-years. Profound hypogammaglobulinemia (<5 g/L) developed in five patients at 15 to 31 months after the last rituximab infusion. In total, 25 patients died at a median age of 80 [69.5-83.9] years, corresponding to a mortality rate of 2.3/100 patient-years. Only three deaths related to infection that occurred 12 to 14 months after rituximab infusions could be due in part to rituximab. At 60 months of follow-up, 73 (29.4%) patients had a sustained response. On univariate and multivariate analysis, the only factor significantly associated with sustained response was a previous transient response to corticosteroids (P = .022). Overall, 24 patients with an initial response and then relapse received retreatment with rituximab, which gave a response in 92%, with a higher duration of response in 54%. As a result of its safety profile and its sustained response rate, rituximab remains an important option in the current therapeutic armamentarium for adult ITP. Retreatment could be an effective and safe option.

5.
BMJ Open ; 9(8): e027431, 2019 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-31439599

RESUMO

INTRODUCTION: This study protocol describes a trial designed to investigate whether antihistamine alone in patients with acute urticaria does not increase the 7-day Urticaria Activity Score (UAS7) in comparison with an association of antihistamine and glucocorticoids and reduces short-term relapses and chronic-induced urticaria. METHODS AND ANALYSIS: This is a prospective, double-blind, parallel-group, multicentre non-inferiority randomised controlled trial. Two-hundred and forty patients with acute urticaria admitted to emergency department will be randomised in a 1:1 ratio to receive levocetirizine or an association of levocetirizine and prednisone. Randomisation will be stratified by centre. The primary outcome will be the UAS7 at day 7. The secondary outcomes will encompass recurrence of hives and/or itch at day 7; occurrence of spontaneous hives or itch for >6 weeks; patients with angioedema at day 7, and 2, 6, 12 and 24 weeks; new emergency visits for acute urticaria recurrences at days 7 and 14, and 3 months; Dermatology Life Quality Index at days 7 and 14, and 3 and 6 months; and Chronic Urticaria Quality of Life Questionnaire at 6 weeks. ETHICS AND DISSEMINATION: The protocol has been approved by the Comité de Protection des Personnes Sud-Méditerranée II and will be carried out in accordance with the Declaration of Helsinki and Good Clinical Practice guidelines. A steering committee will oversee the progress of the study. Findings will be disseminated through national and international scientific conferences and publication in peer-reviewed journals. TRIAL REGISTRATION NUMBER: NCT03545464.

6.
Sci Rep ; 9(1): 12388, 2019 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-31455785

RESUMO

Diagnosis of large vessel vasculitis (LVV) and evaluation of its inflammatory activity can be challenging. Our aim was to investigate the value of hybrid positron-emission tomography/magnetic resonance imaging (PET/MRI) in LVV. All consecutive patients with LVV from the Department of Internal Medicine who underwent PET/MRI were included. Three PET/MRI patterns were defined: (i) "inflammatory," with positive PET (>liver uptake) and abnormal MRI (stenosis and/or wall thickening); (ii) "fibrous", negative PET (≤liver uptake) and abnormal MRI; and (iii) "normal". Thirteen patients (10 female; median age: 67-years [range: 23-87]) underwent 18 PET/MRI scans. PET/MRI was performed at diagnosis (n = 4), at relapse (n = 7), or during remission (n = 7). Among the 18 scans, eight (44%) showed an inflammatory pattern and three (17%) a fibrous pattern; the other seven were normal. The distribution of the three patterns did not differ between patients with Takayasu arteritis (TA, n = 10 scans) and those with giant cell arteritis (GCA, n = 8 scans). PET/MRI findings were normal in 2/10 (20%) TA scans vs. 5/8 (62%) GCA scans (p = 0.3). Median SUVmax was 4.7 [2.1-8.6] vs. 2 [1.8-2.6] in patients with active disease vs. remission, respectively (p = 0.003). PET/MRI is a new hybrid imaging modality allowing comprehensive and multimodal analysis of vascular wall inflammation and the vascular lumen. This technique offers promising perspectives for the diagnosis and monitoring of LVV.

8.
Arch Immunol Ther Exp (Warsz) ; 67(4): 225-236, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31147723

RESUMO

We aimed to compare the proportion of peripheral blood natural killer (NK) cells (CD3-CD56+) and T-cell large granular lymphocytes (CD8+CD57+) during preconception in a homogenous group of women with unexplained well-defined recurrent miscarriage (RM) and repeated implantation failure (RIF) vs healthy controls in relation to pregnancy outcomes. This case-control study followed by a literature review and meta-analysis was conducted in three university hospitals. Patients and controls were consecutively recruited from December 2015 to October 2017. In total, 115 women were included in the study: 54 with RM, 41 with RIF and 20 healthy controls with ≥ 2 term births. Percentages of CD3-CD56+ and CD8+CD57+ cells and sub-populations of CD3-CD56+ cells did not differ between cases and controls. The results for women with subsequent miscarriage did not differ from those with live births. The meta-analysis of the literature showed higher NK-cell proportions in RM [mean difference 3.47 (95% CI 2.94-4.00); p < 0.001] and RIF [mean difference 1.64 (95% CI 0.82-2.45); p < 0.001] than controls. However, the heterogeneity between the different studies was high. The proportion of peripheral blood CD3-CD56+ and CD8+CD57+ cells in the preconception period does not reflect the risk of implantation failure or miscarriage and should not be recommended indicators for the management of RM and RIF. Further prospective large studies are needed to develop a reliable peripheral blood marker of immune deregulation.

10.
Eur J Intern Med ; 67: 42-46, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31109849

RESUMO

OBJECTIVE: Hereditary angiœdema (HAE) is a rare autosomal dominant disease characterized by recurrent, unpredictable, potentially life-threatening swelling. Objective is to assess the management of the acute HAE attacks in the real life setting through a call center in France. METHODS: A pre-specified ancillary study of SOS-HAE, a cluster-randomized prospective multicenter trial, was conducted. HAE patients were recruited from 8 participating reference centers. The outcome of interest was the rate of hospitalization. RESULTS: onerhundred patients were included. The median (quartile) age was 38 (29-53) years, and 66 (66%) were female. Eighty (80%) patients had HAE type I, 8 (8%) had HAE type II and 12 (12%) patients had FXII-HAE. Fifty-one (51%) patients had experienced at least one time the call center during the follow-up. Nine over 166 (5%) attacks for 9 different patients resulted in hospital admission to the hospital (in the short-stay unit, ie, <24 h) during the follow-up period. During 2 years, there were 166 calls to call center for 166 attacks. All attacks were treated at home after call center contact. CONCLUSIONS: Use of emergency departments and hospitalizations are reduced by the use of a coordinated national call center in HAE after therapeutic education program that promoted self-administration of specific treatment and use of call to call center. TRIAL REGISTRATION: clinicalTrials.gov identifier: NCT01679912.

15.
J Dermatol ; 46(5): 383-388, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30816579

RESUMO

The benefit of corticosteroids in acute urticaria is controversial. Our objective was to determine the factors associated with relapses in patients presenting with acute urticaria. A retrospective observational study, including all patients with acute urticaria who visited the angioedema reference center of Academic Public Hospitals - Saint-Antoine in Paris between January 2015 and June 2017, was conducted. The study inclusion criterion was a diagnosis of acute urticaria in an adult patient. The urticaria was spontaneous or inducible urticaria. The primary outcome was relapse at day 7 and the secondary outcome was relapse at week 6. A total of 184 patients with a first episode of acute urticaria were included. Most of the patients were female (66%) with a mean age of 42 ± 16 years. Corticosteroid administration for treatment of acute urticaria was used in 102 (55%) patients. Overall, 85 (46%) patients had relapses after less than 7 days whereas 168 (91%) patients had relapses after more than 6 weeks. In univariate analysis, the rate of corticosteroid administration was significantly higher in cases of relapse after less than 7 days. No difference in relapse rates after more than 6 weeks appeared. In the multivariate analysis, the independent factor associated with relapses after less than 7 days was the administration of corticosteroids as treatment of acute urticaria (odds ratio, 1.93; 95% confidence interval, 1.06-3.57; P = 0.03). The prevalence of corticosteroid administration for patients with acute urticaria was high. Corticosteroid administration was an independent risk factor associated with relapses after less than 7 days.


Assuntos
Corticosteroides/uso terapêutico , Urticária/tratamento farmacológico , Doença Aguda/terapia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
16.
Eur J Obstet Gynecol Reprod Biol ; 236: 263-264, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30872045

RESUMO

Approximately 1 to 3% of women have recurrent early miscarriages, defined as ≥3 pregnancy losses before 14 weeks of gestation. The immune deregulation and tolerance rupture could be the origin of these miscarriages in at least 30% of these women. Chronic intervillositis of unknown etiology (CIUE) is a rare placental lesion characterized by intrauterine deaths, growth restriction and high recurrence rate. In cases with recurrent obstetrical adverse events and intrauterine deaths, we previously reported the benefit of hydroxychloroquine combination to prednisone. Even few data raised the potential value of TNF antagonists in early recurrent miscarriages, these cases show its potential value in the setting of recurrent refractory chronic intervillositis and unexplained miscarriages.


Assuntos
Aborto Habitual/prevenção & controle , Adalimumab/uso terapêutico , Aspirina/uso terapêutico , Prednisona/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Anti-Inflamatórios/uso terapêutico , Quimioterapia Combinada , Feminino , Fibrinolíticos/uso terapêutico , Humanos , Gravidez , Resultado da Gravidez , Resultado do Tratamento
17.
J Am Acad Dermatol ; 2019 Feb 06.
Artigo em Inglês | MEDLINE | ID: mdl-30738125

RESUMO

BACKGROUND: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M. METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. RESULTS: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone. CONCLUSION: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.

18.
Ann Rheum Dis ; 78(4): 539-550, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30760472

RESUMO

OBJECTIVES: Systemic sclerosis (SSc) is an autoimmune disease characterised by widespread fibrosis, microangiopathy and autoantibodies. Follicular helper T (Tfh) cells CD4+CXCR5+PD-1+ cooperate with B lymphocytes to induce the differentiation of plasmocytes secreting immunoglobulins (Ig). Circulating Tfh (cTfh) cells are increased in several autoimmune diseases. However, there are no data about cTfh cells and their interaction with B cells in SSc. The aim of this study was to perform a quantitative and functional analysis of cTfh cells in SSc. METHODS: Using flow cytometry, we analysed cTfh cells from 50 patients with SSc and 32 healthy controls (HC). In vitro coculture experiments of sorted cTfh and B cells were performed for functional analysis. IgG and IgM production were measured by ELISA. RESULTS: We observed that cTfh cell numbers are increased in patients with SSc compared with HC. Furthermore, the increase in cTfh cells was more potent in patients with severe forms of SSc such as diffuse SSc and in the presence of arterial pulmonary hypertension. cTfh cells from patients with SSc present an activated Tfh phenotype, with high expression of BCL-6, increased capacity to produce IL-21 in comparison with healthy controls. In vitro, cTfh cells from patients with SSc had higher capacity to stimulate the differentiation of CD19+CD27+CD38hi B cells and their secretion of IgG and IgM through the IL-21 pathway than Tfh cells from healthy controls. Blocking IL-21R or using the JAK1/2 inhibitor ruxolitinib reduced the Tfh cells' capacity to stimulate the plasmablasts and decreased the Ig production. CONCLUSIONS: Circulating Tfh cells are increased in SSc and correlate with SSc severity. The IL-21 pathway or JAK1/2 blockade by ruxolitinib could be a promising strategy in the treatment of SSc.

19.
J Autoimmun ; 99: 48-51, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30737054

RESUMO

Acute cerebrovascular ischemic events are a rare and severe complication of giant cell arteritis (GCA). We aimed to determine the prevalence of GCA-related stroke, the overall survival and the relapse-free survival in patients with GCA. A multicentric retrospective analysis was performed on 129 patients with GCA diagnosed between September 2010 and October 2018 in two University Hospitals. Among 129 GCA patients, 18 (16%) presented an acute ischemic cerebrovascular event. Patients with stroke were older (83 [67-96] years versus 76 [58-96]; p = 0.014) and more frequently males (61% versus 30%; p = 0.014) than those without stroke. The frequency of anterior ischemic optic neuropathy was higher in patients with stroke (n = 6, 33%) than patients without stroke (n = 12, 11%)(p = 0.02). Overall survival was significantly decreased in GCA patients with stroke (4.4 months), comparatively to patients without stroke (221.7 months; log rank test = 0.006). The 3-years relapse-free survival was decreased in patients with stroke (8.42 versus78.0 months; log rank = 0.0001), as well as the time with sustained remission (78 versus 139 months; log rank test = 0.0004). This study shows the prevalence and risk factors of ischemic stroke in GCA.

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