Pupillary hippus in nonconvulsive status epilepticus.

We report the video recording of a patient who experienced pupillary hippus during an episode of nonconvulsive status epilepticus. [Published with video sequences].

Lupus anticoagulant-hypoprothrombinemia syndrome: report of 8 cases and review of the literature.

The lupus anticoagulant-hypoprothrombinemia syndrome (LAHS)--the association of acquired factor II deficiency and lupus anticoagulant--is a rare disease drastically different from antiphospholipid syndrome in that it may cause predisposition not only to thrombosis but also to severe bleeding. We performed a retrospective study of 8 patients with LAHS referred to 6 French tertiary care centers between January 2003 and February 2011, and a literature review retrieving all related articles published between 1960 and April 2011. Including our 8 new cases, LAHS has been reported in 74 cases.

High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses.

Histiocytoses are rare disorders of unknown origin with highly heterogeneous prognosis. BRAF mutations have been observed in Langerhans cell histiocytosis (LCH). We investigated the frequency of BRAF mutations in several types of histiocytoses.

Evaluating cellular polyfunctionality with a novel polyfunctionality index.

Functional evaluation of naturally occurring or vaccination-induced T cell responses in mice, men and monkeys has in recent years advanced from single-parameter (e.g. IFN-γ-secretion) to much more complex multidimensional measurements.

LupusQoL-FR is valid to assess quality of life in patients with systemic lupus erythematosus.

Objective: To cross-culturally adapt the LupusQoL into French, to test its measurement properties and to further investigate its domain structure.

Methods: The cultural adaptation process according to guidelines and pre-testing resulted in the LupusQoL-FR. SLE patients completed the LupusQoL-FR at baseline, 15 days, 3 months and 6 months.

The Relapsing Polychondritis Disease Activity Index: development of a disease activity score for relapsing polychondritis.

Objective: The rarity of relapsing polychondritis (RP) has hindered the development of standardized tools for clinical assessment. Here, we describe the development of a preliminary score for disease assessing activity in RP, the Relapsing Polychondritis Disease Activity Index (RPDAI).

Methods: Twenty-seven RP experts participated in an international collaboration.

Activated and resting regulatory T cell exhaustion concurs with high levels of interleukin-22 expression in systemic sclerosis lesions.

Objective: Transforming growth factor-β is considered to play a key role in the process of fibrosis in systemic sclerosis (SSc) and in the development of regulatory T cells (Treg) and pro-inflammatory Th17 T cells producing interleukin 17 (IL-17) and IL-22. The authors therefore postulated that SSc could be characterised by a marked Treg/Th17 imbalance. Previous works did not distinguish between the different subsets of Treg and the non-regulatory FoxP3(+) cells leading to inconsistent results.

Radiation hardening techniques for Er/Yb doped optical fibers and amplifiers for space application.

We investigated the efficiencies of two different approaches to increase the radiation hardness of optical amplifiers through development of improved rare-earth (RE) doped optical fibers. We demonstrated the efficiency of codoping with Cerium the core of Erbium/Ytterbium doped optical fibers to improve their radiation tolerance. We compared the γ-rays induced degradation of two amplifiers with comparable pre-irradiation characteristics (~19 dB gain for an input power of ~10 dBm): first one is made with the standard core composition whereas the second one is Ce codoped.

High-sensitivity flow cytometry provides access to standardized measurement of small-size microparticles--brief report.

Objective: Cellular microparticles (MP) are promising biomarkers in many pathological situations. Although flow cytometry (FCM) is widely used for their measurement, it has raised controversies because the smallest MP size falls below the detection limit of standard FCM (sd-FCM). Following recent technological improvements leading to high sensitivity FCM (hs-FCM), our objectives were (1) to evaluate the potential of hs-FCM for extended MP detection, (2) to set up a standardized protocol for MP enumeration, and (3) to compare MP counts obtained with both sensitivity levels.

[Treatment of systemic lupus erythematosus].

Systemic lupus erythematosus is a chronic autoimmune disease. Treatment goals are to cure flares and to prevent them as well as long-term complications and drugs toxicity. Effort to improve patient knowledge and education should be a priority.

[When to think about lupus?].

Clinical and biological manifestations of systemic lupus erythematosus (SLE) is a clinically heterogeneous autoimmune disease, which is characterized by the presence of autoantibodies directed against various nuclear antigens. It is typically a relapsing and remitting multi-system disease, and patients can present in many different ways. The most common manifestations include rash, arthritis and fatigue.

Treatment of Erdheim-Chester disease with long-term high-dose interferon-α.

Objectives: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by a foamy CD68+, CD1a- histiocyte tissue infiltration. Efficacy of standard doses of interferon-α-2a (IFNα) has been suggested in a small series but with variation, depending on the organs involved. Our aim was to report our single-center experience about the use of high-dose IFNα in ECD.

Late-onset systemic lupus erythematosus: epidemiology, diagnosis and treatment.

Systemic lupus erythematosus (SLE) is usually described as a disease that most often strikes reproductive-age women. However, the onset of SLE beyond the age of 50 years is reported to occur in 3-18% of patients. This later age at onset has a strong modifying effect on the clinical presentation, disease course, response to treatment and prognosis of SLE.

Cytokine profiles in sepsis have limited relevance for stratifying patients in the emergency department: a prospective observational study.

Introduction: Morbidity, mortality and social cost of sepsis are high. Previous studies have suggested that individual cytokines levels could be used as sepsis markers. Therefore, we assessed whether the multiplex technology could identify useful cytokine profiles in Emergency Department (ED) patients.

Erdheim-Chester disease.

Purpose Of Review: Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis first described in 1930 with a wide range of manifestations. The number of new cases has dramatically increased over the past 10 years because of the better recognition of this condition. The natural evolution is variable, but the spontaneous prognosis is severe.

Exhausted cytotoxic control of Epstein-Barr virus in human lupus.

Systemic Lupus Erythematosus (SLE) pathology has long been associated with an increased Epstein-Barr Virus (EBV) seropositivity, viremia and cross-reactive serum antibodies specific for both virus and self. It has therefore been postulated that EBV triggers SLE immunopathology, although the mechanism remains elusive. Here, we investigate whether frequent peaks of EBV viral load in SLE patients are a consequence of dysfunctional anti-EBV CD8+ T cell responses.

Pathogenesis of Takayasu's arteritis: a 2011 update.

While our knowledge of the pathogenesis of Takayasu's arteritis (TA) has considerably improved during the last decade, the exact pathogenic sequence remains to be elucidated. It is now hypothesised that an unknown stimulus triggers the expression of the 65kDa Heat-shock protein in the aortic tissue which, in turn, induces the Major Histocompatibility Class I Chain-Related A (MICA) on vascular cells. The γδ T cells and NK cells expressing NKG2D receptors recognize MICA on vascular smooth muscle cells and release perforin, resulting in acute vascular inflammation.

Multiparameter grouping delineates heterogeneous populations of human IL-17 and/or IL-22 T-cell producers that share antigen specificities with other T-cell subsets.

The ontogenic relationship between pro-inflammatory populations of interleukin-17 (IL-17A)- and/or IL-22-producing T cells and other T-cell subsets is currently unclear in humans. To appreciate T helper cell-lineage commitment, we combined cytokine production profiles of in vitro expanded T-cell clones with T-cell receptor (TCR) clonotypic signatures. Moreover, ex vivo cytokine production profiles at the single-cell level were analyzed using an original approach based on the hierarchical cluster analysis of multiparametric flow cytometry data.

The importance of assessing medication exposure to the definition of refractory disease in systemic lupus erythematosus.

Treatment of patients with Systemic Lupus Erythematosus (SLE) who have active disease refractory to current therapeutic strategies continues to be a real challenge. Here, we propose that the classic definition of refractory SLE patients - failure to achieve adequate response to the standard of care - should be further refined to incorporate the dimension of adequate drug exposure. Inter-individual pharmacokinetic variability may induce insufficient exposure to many drugs used in SLE, leading to both apparent inefficacy of treatments and inappropriate therapeutic escalation.