Local hyperhemia to heating is impaired in secondary Raynaud's phenomenon

Accurate and sensitive measurement techniques are a key issue in the quantification of the microvascular and endothelial dysfunction in systemic sclerosis (SSc). Thermal hyperhemia comprises two separate mechanisms: an initial peak that is axon reflex mediated; and a sustained plateau phase that is nitric oxide dependent. The main objective of our study was to test whether thermal hyperhemia in patients with SSc differed from that in patients with primary Raynaud's phenomenon (RP) and healthy controls. In a first study, we enrolled 20 patients suffering from SSc, 20 patients with primary RP and 20 healthy volunteers. All subjects were in a fasting state. Post-occlusive hyperhemia, 0.4 mg sublingual nitroglycerin challenge and thermal hyperhemia were performed using laser Doppler flowmetry on the distal pad of the third left finger. In a second study, thermal hyperhemia was performed in 10 patients with rheumatoid arthritis and 10 patients with primary RP. The thermal hyperhemia was dramatically altered in terms of amplitude and kinetics in patients with SSc. Whereas 19 healthy volunteers and 18 patients with primary RP exhibited the classic response, including an initial peak within the first 10 minutes followed by a nadir and a second peak, this occurred only in four of the SSc patients (p < 0.0001). The 10 minutes thermal peak was 43.4 (23.2 to 63), 42.6 (31 to 80.7) and 27 (14.7 to 51.4) mV/mm Hg in the healthy volunteers, primary RP and SSc groups, respectively (p = 0.01), while the 44°C thermal peak was 43.1 (21.3 to 62.1), 42.6 (31.6 to 74.3) and 25.4 (15 to 52.4) mV/mm Hg, respectively (p = 0.01). Thermal hyperhemia was more sensitive and specific than post-occlusive hyperhemia for differentiating SSc from primary RP. In patients with rheumatoid arthritis, thermal hyperhemia was also altered in terms of amplitude. Thermal hyperhemia is dramatically altered in patients with secondary RP in comparison with subjects with primary RP. Further studies are required to determine the mechanisms of this altered response, and whether it may provide additional information in a clinical setting

Vaccination antigrippale saisonnière au cours du lupus systémique (analyse de la couverture vaccinale et pistes d'amélioration à partir d'une étude observationnelle de soixante cas)

Le lupus érythémateux systémique (LES) est une maladie inflammatoire dysimmunitaire systémique. Le risque infectieux, à l origine d environ 25% des décès chez ces patients, est favorisé par l immunodépression induite par la maladie et les traitements nécessaires à sa prise en charge. Certaines de ces infections, la grippe en particulier, pourraient être prévenues par la vaccination. L immunisation par la vaccination peut théoriquement être associée à la (ré)activation des mécanismes auto-immuns chez les patients atteints de LES et fait l objet de discussions concernant son efficacité ou sa tolérance. Une étude rétrospective observationnelle a été menée afin d analyser la couverture vaccinale antigrippale saisonnière au sein d une cohorte de patients atteints de LES défini selon les critères de l ACR (n=60). Ce travail a montré une faible couverture vaccinale (38,3%) chez des patients lupiques. Une analyse univariée a objectivé une vaccination plus fréquente chez les patients âgés, de sexe masculin ou recevant une corticothérapie orale. Une analyse multivariée a mis en évidence une meilleure couverture vaccinale chez les hommes et chez les patients à qui la vaccination était proposée par le médecin spécialiste. Cette faible couverture vaccinale s explique principalement par le manque d incitation à la vaccination liée à la réticence des médecins mais également à l inquiétude des patients. Au vu de ces résultats, conformes aux données de la littérature, il paraît primordial de renforcer l éducation des patients afin d améliorer la couverture vaccinale des patients atteints de lupus, particulièrement à risque de développer des infections potentiellement graves.GRENOBLE1-BU Médecine pharm. (385162101) / SudocSudocFranceF

Traitement du lupus érythémateux disséminé par chlorambucil (étude rétrospective de 32 cas)

TOURS-BU Médecine (372612103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Immunoglobulins: Benefits and risks from the patient's point of view

International audienc

Immunoglobulins: Benefits and risks from the patient's point of view

International audienc

Hepatitis E and neuralgic amyotrophy: Five cases and review of literature

International audienceHepatitis E virus infection - mainly genotype 3 - is increasingly common in industrialized countries. Infection is usually asymptomatic, but cases of central or peripheral neurological symptoms with hepatitis E have been described. The most frequent is Guillain-Barre but somes cases of neuralgic amyotrophy have been described. In our center, since 2010, we have identified five cases of neuralgic amyotrophy associated with acute hepatitis E in immunocompetent patients. For all these patients, neuralgic amyotrophy was diagnosed with electromyogram and positive IgM for hepatitis E, and detectable HEV RNA in 4 of the cases. Including our patients, we count 26 cases in literature. The mean age of the patients was 44 years old, with a large predominance of males (88%). The disorder is bilateral and asymmetric in 69% of cases. Peripheral nerves other than the brachial plexus were affected in 6 patients (23%). In industrialized countries, any neuralgic amyotrophy, particularly if there is bilateral, asymmetric associated with involvement of nerves outside the brachial plexus, should lead physicians to consider a diagnosis of acute hepatitis E

Letter to the Editor: Protein phosphatase 1 subunit Ppp1r15a/GADD34 is overexpressed in systemic lupus erythematosus and related to the expression of type I interferon response genes

International audienc

Potentially stress-induced acute splanchnic segmental arterial mediolysis with a favorable spontaneous outcome

A 62-year-old woman presented with hemithoracic anesthesia and acute abdominal pain following a violent psychological stress. Magnetic resonance imaging showed a thoracic hematoma with arachnoiditis of the spinal cord. Tomography revealed a typical aspect of segmental arterial mediolysis with multiple aneurysms and stenoses of the splanchnic arteries, confirmed by abdominal arteriography. There was no argument for hereditary, traumatic, atherosclerotic, infectious, or inflammatory arterial disease. Segmental arterial mediolysis was diagnosed on the basis of the radiologic data and probably involved both medullary and splanchnic arteries. The patient spontaneously recovered and was in good health 18 months later

Autoantibodies targeting ficolin-2 in systemic lupus erythematosus patients with active nephritis

International audienceObjective: Systemic lupus erythematosus (SLE) is a multi‐system inflammatory disease characterized by production of various autoantibodies. The aim of this study was to investigate the presence of anti‐ficolin‐2 antibodies in SLE patients and to evaluate the association between the levels of these autoantibodies, clinical manifestations, and disease activity.Methods: This is a comparative study using a cohort of 165 SLE patients and 48 healthy subjects. SLE patients were further divided into two groups, with “low disease activity” (SLEDAI score ≤ 4, n = 88) and with “high disease activity” (SLEDAI score > 4, n = 77). Clinical manifestations were defined according to the physician in charge. Active lupus nephritis (LN) was documented by kidney biopsy. Detection of anti‐ficolin‐2 antibodies was performed by ELISA.Results: Levels of the anti‐ficolin‐2 autoantibodies were significantly higher in SLE patients as compared to healthy subjects and associated with the SLEDAI score. They were found positive in 61/165 (37%) SLE patients. Presence of anti‐ficolin‐2 antibodies was significantly related only to renal involvement, with a very high prevalence (86%) of anti‐ficolin‐2 antibodies in SLE patients with active LN. Patients with active proliferative LN had significantly more positive anti‐ficolin‐2 antibodies than those with non‐proliferative LN. The combination of anti‐ficolin‐2, anti‐ficolin‐3 and anti‐C1q demonstrated a very high specificity (98%) for the diagnosis of active LN.Conclusion: Our results support the usefulness of anti‐ficolin‐2 as a complementary serological biomarker for the diagnosis of active lupus with renal manifestatio