Quality of life in systemic lupus erythematosus: description in a cohort of French patients and association with blood hydroxychloroquine levels.

OBJECTIVES: Benefits of hydroxychloroquine (HCQ) use on physician reported outcomes are well documented in systemic lupus erythematosus (SLE). We assess for the first time the association and predictive value of blood HCQ levels towards health-related quality of life (HRQOL) in SLE. METHODS: Data from the PLUS study (a randomized, double-blind, placebo-controlled, multicentre study) were utilized. Blood HCQ levels were quantified by high-performance liquid chromatography along with HRQOL assessments (Medical Outcomes Study-SF-36) at baseline (V1) and month 7 (V2). RESULTS: 166 SLE patients' data were analysed. Mean (SD) age and disease duration were 44.4 (10.7) and 9.3 (6.8) years. Eighty-seven per cent were women. Mean (SD, median, IQR) HCQ concentrations in the blood at V1 were 660 (314, 615, 424) ng/ml and increased to 1020 (632, 906, 781) ng/ml at V2 (mean difference 366 units, 95% confidence interval -472 to -260, p < 0.001). No significant correlations between HCQ concentrations with HRQOL domains at V1 or V2 were noted. There were no differences in HRQOL stratified by HCQ concentrations. HCQ concentrations at V1 or changes in HCQ concentration (V2-V1) were not predictive of HRQOL at V2 or changes in HRQOL (V2-V1). CONCLUSIONS: No association of HCQ concentrations with current or longitudinal HRQOL were found in SLE.

[Adult kwashiorkor: a rare complication of bariatric surgery]. / Kwashiorkor chez l'adulte : une complication rare de la chirurgie bariatrique.

INTRODUCTION: Kwashiorkor is a clinical manifestation of global protein-calorie malnutrition that is described mainly in children in developing countries initially presenting eczema-like skin disease associated with hydrops. OBSERVATION: We report the case of a 44-year-old woman with dark skin who had undergone bariatric surgery for treatment of stage-III obesity without regular monitoring and who was hospitalized in our dermatology department four years later. She had diffuse oedema and asteatotic eczema-like cutaneous lesions on pigmented skin that had developed for two months despite daily application of topical corticosteroids. Clinical signs, the collapse of serum albumin and histopathological analysis of skin tissue were evocative of kwashiorkor. Her symptoms subsided after several weeks of a nutritional programme. DISCUSSION: Kwashiorkor is a clinical entity described chiefly in children in developing countries. Cutaneous involvement comprising asteatotic eczema-like skin associated with diffuse oedema is prominent. Treatment with protein supplementation alone is effective and avoids many complications such as infections and haemodynamic disorders. Obesity is a serious and common disease in Western countries affecting one third of the world's population, and for which treatment with bariatric surgery may be indicated. Without regular monitoring, this surgery can cause severe protein deficiency. Consequently, in the future there will seemingly be a greater likelihood of kwashiorkor in adults as an iatrogenic complication of obesity treatment. Its semiology needs to be underlined because of the simplicity and efficacy of treatment.

Direct observation of long-lived isomers in 212Bi.

Long-lived isomers in (212)Bi have been studied following (238)U projectile fragmentation at 670 MeV per nucleon. The fragmentation products were injected as highly charged ions into a storage ring, giving access to masses and half-lives. While the excitation energy of the first isomer of (212)Bi was confirmed, the second isomer was observed at 1478(30) keV, in contrast to the previously accepted value of >1910 keV. It was also found to have an extended Lorentz-corrected in-ring half-life >30 min, compared to 7.0(3) min for the neutral atom. Both the energy and half-life differences can be understood as being due a substantial, though previously unrecognized, internal decay branch for neutral atoms. Earlier shell-model calculations are now found to give good agreement with the isomer excitation energy. Furthermore, these and new calculations predict the existence of states at slightly higher energy that could facilitate isomer deexcitation studies.

[Symptomatic adrenal insufficiency secondary to the use of cutaneous topical steroids for skin-bleaching]. / Insuffisance surrénalienne haute symptomatique compliquant l'usage de dermocorticoïdes pour dépigmentation volontaire.

In black population, the skin-bleaching with cutaneous topical corticosteroids on a large body area is a widespread practice and is associated with numerous cutaneous complications. We report a 25-year-old Congolese woman who was admitted for weakness, arthralgias and abdominal pain. The association of a relative hyperpigmentation of the small joints of hands and feet with clinical features of hypercorticism led to suspect a chronic use of cutaneous topical steroids for skin-bleaching. On biological tests, plasma cortisol and corticotropin levels were undetectable and the short corticotropin (ACTH) stimulation test was negative, leading to the diagnosis of adrenal insufficiency complicating the chronic use of topical steroids. Clinical symptoms resolved with hydrocortisone therapy. One year later, the patient admitted a five-year continuous use of cutaneous topical steroids (betamethasone, 0.05%). Skin-bleaching through chronic use of cutaneous topical steroids, is a common practice in black women, and should be suspected in the presence of adrenal insufficiency with or without clinical features of hypercorticism, and conversely, skin-bleaching users should be tested for hypothalamo-pituitary-adrenal function.

[Cardiac sarcoidosis: Diagnosis and therapeutic challenges]. / Sarcoïdose cardiaque : avancées diagnostiques et thérapeutiques.

Sarcoidosis is a granulomatous disorder of unknown cause characterized by non-caseating granuloma in young adults. Cardiac involvement is rare and range from 2 to 75% depending on diagnostic criteria. Cardiac involvement in sarcoidosis may be asymptomatic or may manifest as rhythm/conduction troubles or congestive heart failure. The diagnosis and treatment of cardiac sarcoidosis may be challenging. However, advances have come in recent years from the use of cardiac MRI and 18FDG-TEP scanner, as well as from the stratification of the risk of ventricular tachycardia/fibrillation. Due to the rarity of the disease, there is no reliable prospective large study to guide therapeutic strategy for cardiac sarcoidosis. Corticosteroids are probably efficacious, in particular in case of atrio-ventricular block or moderate heart failure. Immunosuppressive drugs have not been largely studied but methotrexate could be helpful. In refractory forms, TNF-α antagonists have been used with success.

[Neurosarcoidosis: Diagnosis and therapeutic issues]. / Les atteintes neurologiques au cours de la sarcoïdose : diagnostic et traitement.

Neurological localizations of sarcoidosis are heterogeneous and may affect virtually every part of the central or peripheral nervous system. They are often the inaugural manifestation of sarcoidosis. The diagnosis may be difficult due to the lack of extra-neurological localization. Diagnosis may be discussed in the presence of an inflammatory neurological disease, in particular in case of suggestive radiological or biological pattern. Cerebrospinal fluid analysis shows lymphocytic pleiocytosis, often with low glucose level. The diagnosis relies on a clinical, biological and radiological presentation consistent with neurosarcoidosis, the presence of non-caseating granuloma and exclusion of differential diagnoses. Screening for other localizations of sarcoidosis, in particular cardiac disease may be obtained during neurosarcoidosis. The treatment of neurosarcoidosis relies on corticosteroids although immunosuppressive drugs are usually added because of the chronic course of this condition and to limit the side effects of steroids. Treatments and follow-up may be prolonged because of the high rate of relapses.

[Grand types of studies 2: analytical studies]. / Les grands types d'étude 2: les études analytiques.

PURPOSE: This paper describes the hierarchy of analytical studies, from the observational studies to the experimental ones. STATE OF THE ART AND MAIN POINTS: Case-control studies, the most frequently performed among analytical studies, may generate or confirm hypotheses. They are of particular use for rare diseases. Cohort studies aim mainly at confirming hypotheses already tested, and at precisely quantifying the magnitude of effect. Randomisation aims at the elimination of confounding factors, and meta-analysis, at decreasing potential selection biases and increasing power. Those goals may not always be achieved. PERSPECTIVES AND PROJETS: The study power, or the beta error, are major factors to be determined when designing a study, before any attempt of realization.

[Grand types of clinical studies]. / Les grands types d'études cliniques 1: de la description à l'analyse.

PURPOSE: This paper provides an overview of the hierarchy between the main descriptives clinical studies, with their respective weaknesses and strength. STATE OF THE ART AND MAIN POINTS: Case-reports and case-series are first described, which do not provide control groups but have been very useful in disease recognition and nosology building. Ecological studies, which are hypothesis generating at the group or population level, but which do not study individuals, and prevalence studies with one simple or a double question, which study individuals within groups but may lead to erroneous conclusions so far are risk factors are involved, are then reviewed. PERSPECTIVES AND PROJECTS: The overview of the descriptive studies leads to the notion of confounding factor, which may be better addressed by analytical studies.

[In vitro fertilization and systemic lupus erythematosus or antiphospholipid syndrome: An update]. / Fécondation in vitro au cours du lupus érythémateux systémique ou du syndrome des antiphospholipides : mise au point.

Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

[Bilateral optic neuropathy revealing systemic sarcoidosis]. / Neuropathie optique bilatérale révélatrice d'une sarcoïdose systémique.

INTRODUCTION: Although ocular involvement concerns about 25 to 60% of patients with systemic sarcoidosis, papilledema is a rare event. EXEGESIS: A 43-year-old man presented with left visual loss related to papillitis. Despite high-dose corticosteroids, visual loss became rapidly bilateral. Thoracic CT-scan showed mediastinal and parenchymal involvement. Histological study of lung, bronchial and lymphadenopathy biopsies displayed giant-cells granuloma without necrosis. Diagnosis of sarcoidosis complicated with optic neuropathy was made. Therapy combining prednisone, methotrexate and infliximab was started without significant improvement of visual acuity. CONCLUSION: This report underlines the potential severity of optic neuropathy in sarcoidosis.

[University and community health center collaboration]. / La collaboration CLSC-université.

Nurses from a CLSC in Hull, Quebec are defining a positive role for community health nurses in a multidisciplinary environment. They base their professional day-to-day practice on Orem's conceptual nursing framework. Many of these home care nurses studied in a post-RN baccalaureate nursing program at the University of Quebec in Hull, where they learned different aspects of conceptual frameworks and the nursing process. Two nurse clinicians and a nurse administrator submitted a project based on Orem's model to the administrators of their community health centre. Once it was approved, a nursing professor from the university agreed to act as a consultant and principal researcher to implement the process. The authors describe this three-year collaborative project between home care nurses and the researcher. This includes its objectives, the participants' roles, the tools developed, the process, and the repercussions on nursing care, nurses, and the multidisciplinary team.

Transport of radioactive ion beams and related safety issues: the 132Sn+ case study.

The transport of intense radioactive ion beam currents requires a careful design in order to limit the beam losses, the contamination and thus the dose rates. Some investigations based on numerical models and calculations have been performed in the framework of the SPIRAL 2 project to evaluate the performance of a low energy beam transport line located between the isotope separation on line (ISOL) production cell and the experiment areas. The paper presents the results of the transverse phase-space analysis, the beam losses assessment, the resulting contamination, and radioactivity levels. They show that reasonable beam transmission, emittance growth, and dose rates can be achieved considering the current standards.

[Cardiovascular involvement in Behçet's disease]. / Atteintes cardiovasculaires de la maladie de Behçet.

Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial.

[Acute airway obstruction mimicking angioedema and revealing spontaneous compressive haematoma of thyroid goitre]. / Détresse respiratoire secondaire à un hématome spontané compressif sur goitre rétrosternal, initialement considérée comme un angiœdème.

Substernal thyroid goitre is the most common cause of superior mediastinal masses. Respiratory symptoms are commonly present, in up to 90% of reported cases. However, these symptoms are rarely acute and life threatening. We report a case of acute airway obstruction, initially misdiagnosed as angioedema, revealing a spontaneous compressive substernal goitre haematoma, in a patient under anticoagulant. This life-threatening complication in patients with goitre and anticoagulant should be known.

[Ocular manifestations of Behçet's disease]. / Manifestations oculaires de la maladie de Behçet.

Eye involvement in Behçet's disease (BD) is frequent and an important cause of morbidity. The mean age at onset of uveitis is between 20 and 30 years in male and 30 years in female patients. Ocular involvement includes anterior, posterior or panuveitis. Uveitis may be the presenting manifestation of the disease in 20% of cases or may appear 2 or 3 years after the disease onset. The estimated risk of blindness at 5 years ranges from 15 to 25%. The main goals in the management of patients with BD uveitis are the rapid control of intraocular inflammation, the prevention of recurrent attacks, the achievement of complete remission, and preservation of vision. The medical treatment of patients with severe uveitis relies on the use of systemically administered drugs, including corticosteroids and cytotoxic agents. Anti-TNF agents and interferon-alpha seem to be efficient and well-tolerated alternative therapeutic options. Controlled clinical trials are mandatory to define the place of these new immunomodulatory agents in the therapeutic strategy, and especially their use as first-line therapy.