Introduction.-In systemic lupus erythematosus, hemostasis disorders are mainly thrombotic, but more rarely hemorrhagic. Case report.-A 25-year-old man presented with a macrophagic activation syndrome revealing a systemic lupus erythematosus, secondarily complicated by a hemorrhagic syndrome; biological investigations revealed an increase thrombin time and an activated partial thromboplastin time, normalized by protamin neutralization in vitro, thus confirming the presence of a heparin-like anticoagulant. The hemostasis balance normalized after the specific treatment of lupus. Conclusion.-This rare anomaly of hemostasis balance has been described in blood cancers and solid cancers. This is the first description of a case associated with an autoimmune connective tissue disorder such as lupus. After one year of follow-up, no diagnosis of blood or solid cancer was made. (C) 2018 Societe Nationale Francaise de Medecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Panton-Valentine leucocidin is a major virulence factor produced by some strains of Staphylococcus aureus (SA-PVL). The best-described invasive infection is a necrotizing haemorrhagic pneumonia. Pleural effusion is not uncommon but is always associated with a parenchymal lesion. Here, we report a case of haemorrhagic pleurisy attributable to isolated SA-PVL.

Background Giant cell arteritis (GCA) is a vasculitis of large and medium sized arteries affecting people older than 50 years. Glucocorticosteroids (GC) are the mainstay of therapy but relapses are common, resulting in prolonged treatment course and adverse events. IL-6 correlates with disease activity of GCA and temporal artery biopsy samples show enhanced IL-6 production. Cases series and open label studies reported the efficacy of tocilizumab (TCZ) on symptoms and inflamamtory markers in GCA. Objectives To report the French experience on the efficacy and safety of TCZ in patients with GCA and the evolution after treatment withdrawal. Methods A retrospective multicenter study on patients treated with TCZ for their GCA was conducted. Treatment efficacy was evaluated at a clinical and biological level. Side effects and evolution after treatment withdrawal were also recorded. Results Thirty-four patients (27 women and 7 males) aged 70.5±8.2 (mean±SD) were included. Diagnosis of GCA was based on the ACR criteria (30 patients) and/or on imaging abnormalities suggestive of GCA (8 patients). Giant cell disease was treated by GC and another immunosuppressant was added before TCZ introduction in 20/34 patients. Tocilizumab (8 mg/kg monthly) was introduced after a mean disease evolution of 18 months (0–107). It was efficient in all but 6 patients who still had mild symptoms while CRP was reduced from 40.4 mg/L to 1.5 mg/L (p<0,0001) and GC were tapered from 26.3 to 10.3 mg/day (p<0.0001). One patient died from a septic shock and 3 patients had to stop TCZ for adverse events. Among the 23 patients who stopped treatment (planned medical decision in 20 cases and side effects in 3 cases) eight patients experienced relapses that occurred after a mean of 3.5±1.3 months. Conclusions TCZ therapy leads to rapid and maintained improvement in patients with refractory GCA. However, side effects should be kept in mind in this population. Questions remain regarding the suspensive nature of this treatment and this should be specifically studied in future studies. Disclosure of Interest None declared

La sclérodermie systémique se complique fréquemment d'ulcères digitaux, qui sont source de plaies, de douleur, et de limitation fonctionnelle, aboutissant dans certains cas à des amputations. Le retentissement dans la vie quotidienne et sur la qualité de vie est majeur et souvent sous-estimé. Actuellement, le traitement des ulcères digitaux de la sclérodermie systémique repose sur l'association de traitements locaux et généraux (Ilomédine®, sildénafil) et de mesures préventives (éviction des facteurs de risque, inhibiteurs calciques, bosentan). Nous rapportons le cas d'une femme de 46 ans présentant des ulcères digitaux récidivants, résistant aux traitements usuels (bosentan, Ilomédine®, sildénafil et soins locaux adaptés) depuis plusieurs années. Un traitement par rituximab 1 g j1-j15 était mis en place en novembre 2013, devant l'apparition d'une atteinte systémique associant une atteinte pulmonaire interstitielle et une cardiomyopathie. L'évolution clinique des ulcères digitaux était initialement favorable. À 11 mois, le bilan d'évaluation mettait en évidence une aggravation de la fonction respiratoire ainsi que l'apparition de nouveaux ulcères digitaux. Le bilan biologique trouvait une normalisation du taux de CD 19. La patiente recevait alors une nouvelle cure de rituximab 1 g j1–j15, permettant une disparition complète des ulcères digitaux ; le bénéfice persistait à 6 mois du traitement. De récentes études, notamment l'étude Eustar, ont démontré l'efficacité du rituximab sur l'atteinte pulmonaire et la sclérose cutanée. Nous rapportons le cas d'une efficacité prometteuse du rituximab sur les ulcères digitaux réfractaires. Le rituximab pourrait être une option thérapeutique dans le traitement des ulcères digitaux réfractaires de la sclérodermie systémique mais son efficacité reste à confirmer par des études plus larges.

Background Extracranial involvement of large vessels in giant-cell arteritis (GCA) is probably underdiagnosed. Aortic complications (dilatation and dissection) are a prominent cause of death. [18]F-fluorodeoxyglucose positron-emission tomography ([18F]FDG-PET) is an imaging tool that can demonstrate the inflammation of large vessels. Objectives To assess the value of PET in the diagnosis, the extent of disease9s activity and the follow-up of patients with GCA. Methods Patients were enrolled if they satisfied two criteria: (1) diagnosis of GCA was established fulfilling the American College of Rheumatology criteria (including patients with two criteria and extra-temporal biopsy-proven giant-cell vasculitis); and (2) at least one PET had been performed, at diagnosis (before or in the first 10 days of corticosteroid treatment) or during the follow-up. Patients9 charts were retrospectively reviewed. Clinical symptoms were divided into cephalic and extra-cephalic manifestations. Positivity of PET was defined as a FDG vascular uptake superior to the liver on at least one of the eight following vascular segments: thoracic, abdominal aorta, subclavian, axillary, carotidian, iliac/femoral, and upper and lower limb arteries. Isolated uptakes from the iliac/femoral arteries were not considered as a positive PET. Results 133 patients were enrolled (88 women [66%], median age 70 [50–86]). GCA was biopsy-proven in 78 patients (59%), including 14 positive temporal-artery biopsies (TAB) in patients without any cephalic symptoms. PET was performed at diagnosis in 67 patients and during the follow-up in 66 patients. PET results were positive in 68 (51%) patients and a median of 4 [1–8] vascular areas were involved. The thoracic aorta was involved in 79% of cases. Patients with a positive PET had significantly more extra-cephalic manifestations (59% vs. 37%, p=0.001) and less cephalic symptoms (71% vs. 94%, p=0.0005) than patients with a negative PET. No difference was noted between the 2 groups regarding the TAB status, inflammatory parameters, or cardiovascular risk factors. With a median follow-up of 35 months [6–263], 76 (57%) patients relapsed, and PET results were not clinically useful in 24/26 patients in whom another PET was performed. Aortic dilatation occurred in 14 (11%) patients (of which, 11 [16%] had a positive PET, p=0.03) and aortic dissection in three patients with a positive PET (all with a known dilatation). In univariable analyses, occurrence of aortic complications was associated with the positivity of PET and the absence of cephalic manifestations (hazards ratio (HR) 3.96 [95% confidence interval 1.1–14.26] and 0.27 [95% CI 0.09–0.85]). Conclusions To the best of our knowledge, this is the most extensive study on the use of PET in GCA. Half of the assessed patients had an extra-cephalic involvement of GCA. Large-vessel involvement demonstrated on a PET is associated with a higher risk of aortic complications. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1985

Halophytes have evolved unique molecular strategies to overcome high soil salinity but we still know very little about the main mechanisms that these plants use to complete their lifecycle under salinity stress. One useful approach to further our understanding in this area is to directly compare the response to salinity of two closely related species which show diverse levels of salt tolerance. Here we present a comparative proteomic study using DIGE of leaf microsomal proteins to identify salt-responsive membrane associated proteins in Arabidopsis thaliana (a glycophyte) and Thellungiella salsuginea (a halophyte). While a small number of distinct protein abundance changes were observed upon salt stress in both species, the most notable differences were observed between species and specifically, in untreated plants with a total of 36 proteins displaying significant abundance changes. Gene ontology (GO) term enrichment analysis showed that the majority of these proteins were distributed into two functional categories; transport (31%) and carbohydrate metabolism (17%). Results identify several novel salt responsive proteins in this system and support the theory that T. salsuginea shows a high degree of salt-tolerance because molecular mechanisms are primed to deal with the stress. This intrinsic ability to anticipate salinity stress distinguishes it from the glycophyte A. thaliana.There is significant interest in understanding the molecular mechanisms that plants use to tolerate salinity as soil salinization is becoming an increasing concern for agriculture with high soil Na+ levels leading to reduced yields and economic loss. Much of our knowledge on the molecular mechanisms employed by plants to combat salinity stress has come from work on salt-sensitive plants, but studies on naturally occurring highly salt-resistant plants, halophytes, and direct comparisons between closely related glycophytes and halophytes, could help to further our understanding of salinity tolerance mechanisms. In this study, employing two closely related species which differ markedly in their salt-tolerance, we carried out a quantitative proteomic approach using 2D-DIGE to identify salt-responsive proteins and compare and contrast the differences between the two plant species. Our work complements a previous study using iTRAQ technology (34) and highlights the benefits of using alternative technologies and approaches to gain a broader representation of the salt-responsive proteome in these species.This article is part of a Special Issue entitled: Proteomics, mass spectrometry and peptidomics, Cancun 2013. Guest Editors: César López-Camarillo, Victoria Pando-Robles and Bronwyn Jane Barkla.

Purpose. - About forty percent of the patients with primary Sjogren's syndrome (pSS) experience chronic neuropathic pain with normal electrodiagnostic studies. Two previous studies suggest that chronic neuropathic pain in pSS is due to small fiber neuropathy (SFN). Quantification of epidermal nerve fiber density after skin biopsy has been validated to diagnose small fiber neuropathy.Methods. - Skin biopsy was performed in 14 consecutive pSS patients (satisfying the american-european classification criteria) with chronic neuropathic pain and normal electrodiagnostic studies suggesting SFN.Results. - Fourteen female pSS patients exhibited chronic neuropathic pain [burning sensation (n = 14), prickling (n = 4), dysesthesia (n = 8)] with paroxystic exacerbations (n = 10) and allodynia (n = 13), for a mean period of 18.4 +/- 12.4 months. Neuropathic pain involved mostly hands and feet (n = 13), with a distal (n = 9) and leg (n = 4) predominant distribution. Neurological examination disclosed normal deep tendon responses and absence of motor weakness (n = 14). Small fiber neuropathy was confirmed by skin biopsy in 13 cases. Epidermal nerve fiber density was decreased in distal [(n = 12), mean 3.5 +/- 1.7 fibers/mm (N > 6.9)] and proximal site of biopsy [(n = 9), mean 7.04 +/- 2.63 fibers/mm (N > 9.3)].Conclusion. - Small fiber neuropathy is commonly responsible of chronic neuropathic pain in pSS. Prevalence, physiopathology and neurological evolution of such neuropathies still remain unknown. (c) 2010 Societe nationale francaise de medecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Prolonged fever of unknown origin (FUO) identifies a pattern of fever that defined in 1961. The identification of the cause of FUO is a challenge in clinical practice despite recent advances in diagnostic techniques. No standardized diagnostic strategy could be determined. The diagnostic process should be guided by the potential diagnostic clues (PDCs) emerging from the history, meticulous physical examination and baseline tests. A standardized flow chart can be applied only in absence of PDCs or when the PDCs are contradictory. In the absence of clues, a staged diagnostic protocol was used to search elements contributing to the diagnosis (CT scan, scintigraphies, endoscopies and systematic biopsies). When diagnosis was not established and patient deteriorated, empiric therapeutic trial were started to presumptive diagnoses. Recently, the role of 18-FDG-PET scan as been intensively evaluated as a second-step investigation technique, as a part of structured diagnostic protocol, early after the initial clinical work-up and baseline biology, radiology and ultrasonography. This approach is based on the fact that reaching a diagnosis is extremely difficult in patients with FUO and that this tracer accumulates in infectious, neoplastic and non-infectious inflammatory disorders. (C) 2009 Elsevier Masson SAS. All rights reserved.

Objective: To determine whether there were any clinical and biological differences between male and female patients with primary Sjogren's syndrome (pSS) in a large bicentric series of patient.Methods: We studied 419 consecutive patients (mean age at onset 53.6 years, mean disease outcome 73 months) with pSS according to American-European criteria, attending two different Departments of Internal Medicine in France. The 42 (9%) male patients in this cohort comprised the male group described in this study.Results: Extraglandular manifestations during the course of the disease were present in 37 (89%) of our male patients with pSS. The extraglandular manifestations were similar among the two groups except that the male patients showed a lower frequency of depression or asthaenia (5% vs. 20%, p=0.014) compared with the females. A significantly greater percentage of women reported lymphopaenia (26% vs. 8%, p=0.02) and leucopaenia (18% vs. 3%, p=0.015) at onset, but thrombopaenia was more common in the male patients (21% vs. 6%, p=0.001). Lymphoma development was slightly more common in the male patients, but with no statistical significance (10% vs. 3%, p=0.06), and occurred earlier after the SS diagnosis (log rank test p=0.04).Conclusion: Although pSS is typically a disease affecting women, clinicians should be aware that it may be diagnosed in male patients. Except for haematological presentation, we could not find any notable differences in clinical and immunological characteristics between male and female patients with pSS.