Prevalence and Clinical Associations of Antiphospholipid Antibodies in Systemic Sclerosis: New Data From a French Cross-Sectional Study, Systematic Review, and Meta-Analysis

Objectives: Antiphospholipid antibodies (aPL) can be present in the sera of systemic sclerosis (SSc) patients. This study aimed to determine the prevalence of aPL in a cross-sectional study of SSc patients, to assess their clinical associations, to perform a systematic review of published reports and a meta-analysis to estimate the worldwide prevalence of aPL in SSc.Methods: Two-hundred and forty-nine SSc patients were consecutively tested once for lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2glycoprotein I (anti-β2GpI) antibodies. Clinical associations with aPL positivity were studied using a logistic regression model. A systematic review of the literature was carried out in PubMed and Embase. Meta-analysis was performed using number of aPL positive (at least one of the three antibodies positive) and negative patients. Meta-regression was used to study potential factors explaining the heterogeneity between studies.Results: In our cross-sectional study, aPL positivity was found in 16 patients (prevalence 6.4%; 95%CI [3.8–10.4]). In multivariate analysis, there was a significant association between aPL positivity and venous thrombosis (VT) (OR 6.25 [1.18–33.00]; p = 0.028) and miscarriage (OR 5.43; 95%CI [1.31–22.13]; p = 0.017). Twenty-four studies were included in the meta-analysis, representing a total population of 3036 SSc patients. The overall pooled prevalence of aPL in SSc was 14% (9–20) with a high degree of heterogeneity among studies.Conclusion: This study found a prevalence of aPL positivity in our SSc population of 6.4% (3.8–10.4) and an overall worldwide pooled prevalence of 14% (9–20). In our SSc population, aPL positivity was associated with VT and miscarriage. These data provide additional insights into the role of aPL in the vasculopathy observed in SSc

The interest of gait markers in the identification of subgroups among fibromyalgia patients

<p>Abstract</p> <p>Background</p> <p>Fibromyalgia (FM) is a heterogeneous syndrome and its classification into subgroups calls for broad-based discussion. FM subgrouping, which aims to adapt treatment according to different subgroups, relies in part, on psychological and cognitive dysfunctions. Since motor control of gait is closely related to cognitive function, we hypothesized that gait markers could be of interest in the identification of FM patients' subgroups. This controlled study aimed at characterizing gait disorders in FM, and subgrouping FM patients according to gait markers such as stride frequency (SF), stride regularity (SR), and cranio-caudal power (CCP) which measures kinesia.</p> <p>Methods</p> <p>A multicentre, observational open trial enrolled patients with primary FM (44.1 ± 8.1 y), and matched controls (44.1 ± 7.3 y). Outcome measurements and gait analyses were available for 52 pairs. A 3-step statistical analysis was carried out. A preliminary single blind analysis using k-means cluster was performed as an initial validation of gait markers. Then in order to quantify FM patients according to psychometric and gait variables an open descriptive analysis comparing patients and controls were made, and correlations between gait variables and main outcomes were calculated. Finally using cluster analysis, we described subgroups for each gait variable and looked for significant differences in self-reported assessments.</p> <p>Results</p> <p>SF was the most discriminating gait variable (73% of patients and controls). SF, SR, and CCP were different between patients and controls. There was a non-significant association between SF, FIQ and physical components from Short-Form 36 (p = 0.06). SR was correlated to FIQ (p = 0.01) and catastrophizing (p = 0.05) while CCP was correlated to pain (p = 0.01). The SF cluster identified 3 subgroups with a particular one characterized by normal SF, low pain, high activity and hyperkinesia. The SR cluster identified 2 distinct subgroups: the one with a reduced SR was distinguished by high FIQ, poor coping and altered affective status.</p> <p>Conclusion</p> <p>Gait analysis may provide additional information in the identification of subgroups among fibromyalgia patients. Gait analysis provided relevant information about physical and cognitive status, and pain behavior. Further studies are needed to better understand gait analysis implications in FM.</p

INTERET DU DOSAGE DES ANTICORPS ANTI-BETA2-GLYCOPROTEINE 1 AU COURS DU SYNDROME DES ANTIPHOSPHOLIPIDES (A PROPOS DE 248 PATIENTS)

LILLE2-BU Santé-Recherche (593502101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Stressful life events and pemphigus.

International audienceBACKGROUND: Stress might be a triggering factor causing pemphigus. We studied 11 consecutive cases of pemphigus over 5 years. OBJECTIVE: Studying and looking for a link between severe life events and the history of the disease. METHODS: An epidemiological retrospective and prospective study was carried out, including an interview and a collection of the clinical history; then the life events were integrated into the clinical history with the patient blind. Two scales were used: Paykel's inventory (assessing the negative impact of life events) and the Mini International Neuropsychiatric Interview DSM-IV (MINI). RESULTS: 10 patients out of 11 were included. With the MINI, 2 patients presented anxiety. Paykel's inventory showed type 3 life events for numerous patients, life event type 4 for 7 patients and type 5 for 3 patients, happening from 1 to 6 months before the first signs or worsening of pemphigus. We found stressful life events before the start or worsening of pemphigus for all patients with no other risk factors. CONCLUSION: Stressful life events can worsen or trigger off a pemphigus. Psychological care, associated with the immunosuppressive treatment, should entail a better management of these patients

Neuralgic amyotrophy triggered by hepatitis E virus: a particular phenotype

International audienceObjective: The neuralgic amyotrophy may be of difficult diagnosis, due to phenotypic variability, with different initial presentations (upper plexus multiple mononeuropathy, lumbosacral involvement, distal reached, phrenic involvement). To date, there is little guidance on these patients’ therapeutic management, especially those for which neuralgic amyotrophy is triggered by hepatitis E virus (HEV-NA). The study aims to identify specific features that characterize patients bearing the neuralgic amyotrophy triggered by HEV. Methods: We first describe a new case report of HEV-neuralgic amyotrophy, with delayed diaphragmatic reach. Then, the literature was searched for reports of HEV-NA (n=39), and neuralgic amyotrophy with phrenic paresis (n=42) from 1999 to June 2016. Relevant data were retrieved, analyzed and compared with the parameters of idiopathic neuralgic amyotrophy (n=199) of the largest cohort, described by Van Alfen and Van Engelen in 2006. Results: Compared to the published cohort, HEV-NA patients were more likely to be men (M/F 34/5 versus 136/ 63, p=0.017), with more frequent bilateral symptoms (86.8% cases versus 28.5%, p<0.0001) as well as phrenic paresis (18.0% versus 6.6%, p=0.028). The clinical improvement is poor, with 15.6% of cases with remission only. Conclusions: A particular phenotype characteristic of the HEV-induced neuralgic amyotrophy has arisen. Our findings call for action in validating the above mentioned features that illustrate the HEV-NA cases as an early diagnosis would prevent complications, especially the phrenic damage often associated with a worse functional outcome

French recommendations for the management of Takayasu’s arteritis

International audienceThe aim of this National Diagnostic and Care Protocol (PNDS) is to explain to the professionals involved the current optimal diagnosis and therapeutic management and care approach for a patient with Takayasu's arteritis. Its purpose is to optimize and harmonize the management and follow-up of this rare disease throughout the country. It also identifies pharmaceutical specialties used in an indication not provided for in the Marketing Authorization, as well as the specialties, products or services necessary for the care of patients but not usually paid for or reimbursed