[Prevention of infections in adults and adolescents with systemic lupus erythematosus: Guidelines for the clinical practice based on the literature and expert opinion]. / Prévention des infections au cours du lupus systémique chez l'adulte et l'adolescent : élaboration de recommandations pour la pratique clinique, à partir d'une analyse de la littérature et de l'avis d'experts.

PURPOSE: To develop French recommendations about the management of vaccinations, the screening of cervical cancer and the prevention of pneumocystis pneumonia in systemic lupus erythematosus (SLE). METHODS: Thirty-seven experts qualified in internal medicine, rheumatology, dermatology, nephrology and pediatrics have selected recommendations from a list of proposition based on available data from the literature. For each recommendation, the level of evidence and the level of agreement among the experts were specified. RESULTS: Inactivated vaccines do not cause significant harm in SLE patients. Experts recommend that lupus patient should receive vaccinations accordingly to the recommendations and the schedules for the general public. Pneumococcal vaccination is recommended for all SLE patients. Influenza vaccination is recommended for immunosuppressed SLE patients. Live attenuated vaccines should be avoided in immunosuppressed patients. Yet, recent works suggest that they can be considered in mildly immunosuppressed patients. Experts have recommended a cervical cytology every year for immunosuppressed patients. No consensus was obtained for the prevention of pneumocystis pneumonia. CONCLUSION: These recommendations can be expected to improve clinical practice uniformity and, in the longer term, to optimize the management of SLE patients.

[Screening and management of cardiovascular risk factors in systemic lupus erythematosus: Recommendations for clinical practice based on the literature and expert opinion]. / Dépistage et prise en charge du risque cardiovasculaire au cours du lupus systémique : élaboration de recommandations pour la pratique clinique, à partir d'une analyse de la littérature et de l'avis d'experts.

PURPOSE: To develop French recommendations about screening and management of cardiovascular risk factors in systemic lupus erythematosus (SLE). METHODS: Thirty-nine experts qualified in internal medicine, rheumatology and nephrology have selected recommendations from a list developed based on evidence from the literature. For each recommendation, the level of evidence and the level of agreement among the experts were specified. RESULTS: Experts recommended an annual screening of cardiovascular risk factors in SLE. Statins should be prescribed for primary prevention in SLE patients based on the level of LDL-cholesterol and the number of cardiovascular risk factors, considering SLE as an additional risk factor. For secondary prevention, experts have agreed on an LDL-cholesterol target of <0.7 g/L. Hypertension should be managed according to the 2013 European guidelines, using renin-angiotensin system blockers as first line agents in case of renal involvement. Aspirin can be prescribed in patients with high cardiovascular risk or with antiphospholipid antibodies. CONCLUSION: These recommendations about the screening and management of cardiovascular risk factors in SLE can be expected to improve clinical practice uniformity and, in the longer term, to optimize the management of SLE patients.

[Cost of systemic lupus erythematosus for adult patients with active and treated disease in France (LUCIE study)]. / Coût du lupus systémique en France des patients adultes avec une maladie active et traitée (étude LUCIE).

PURPOSE: To evaluate in France the annual direct medical cost of adult patients with active systemic lupus erythematosus (SLE) on medication and estimate the cost of a flare. METHODS: A two-year, observational, retrospective, multicenter study, carried out between December 2010 and February 2011. Patients' characteristics, SLE disease activity and severity, rate of flares, healthcare consumption (medications, hospitalisations, etc.) were evaluated. Medical costs were assessed from the national Health Insurance perspective. Cost predictors were estimated using multivariate regression models. RESULTS: Eight centres specialized in SLE management included 93 eligible patients (including 50.5% severe). The mean age was 39.9 (11.9) years and 93.5% were women. At baseline, the mean SLE duration was 9.8 (6.6) years. The mean scores of the SELENA-SLEDAI instrument and the SLICC/ACR index were higher in severe patients (9.8 vs 5.6, and 1.2 vs 0.4 respectively; P<0.001). Over the study period, 51% of patients received the combination containing at least corticosteroids or immunosuppressants. The mean annual direct medical cost of severe patients was €4660 versus €3560 for non-severe patients (non-significant difference). The cost of medications (61.8% of the annual cost) was higher in severe patients (€3214 vs €1856; P<0.05). Immunosuppressants and biologics represented 26.5% and 4.6% of the annual total cost respectively. Patients experienced on average 1.10 (0.59) flares/year, of which 0.50 were severe flare. The occurrence of a new severe flare incremented the annual cost of €1330 (P<0.05). CONCLUSION: Medications represented the major component of the annual direct medical cost. Severe flares increase significantly the cost of SLE care management.

[Positive Pneumocystis jirovecii PCR in immunocompromised patients with a systemic disease: infection or colonisation?]. / PCR Pneumocystis jirovecii positive chez les patients atteints de maladie auto-immune: pneumocystose pulmonaire ou simple colonisation?

PURPOSE: Pneumocystis pneumonia is a serious opportunistic infection that frequently occurred in HIV-seropositive patients, prior to the advent of highly active antiretroviral therapy. This infection can also occur in patients with systemic diseases. The diagnostic value of a positive Pneumocystis jirovecii PCR in patients with systemic diseases has not yet been clearly defined. METHODS: We conducted a retrospective study of patients with a systemic disease who presented clinical symptoms consistent with Pneumocystis pneumonia to assess the diagnostic value of a positive P. jirovecii PCR in respiratory samples. RESULTS: During a 10-year period, 73 patients with respiratory symptoms underwent respiratory sampling with tests for the presence of P. jirovecii. P. jirovecii PCR was positive in 20 patients: Pneumocystis pneumonia was diagnosed in nine patients and for six of these nine patients, the microscopic examination was negative. Patients with Pneumocystis pneumonia differed from those who were solely colonized in that they had a lower CD4+ T lymphocyte count, were more likely to have received immunosuppressive treatment, and were not receiving primary prophylaxis against Pneumocystis pneumonia. Chronic pulmonary involvement was more frequent among colonized patients. CONCLUSION: A positive P. jirovecii PCR does not always indicate overt infection. However, in a context of severe immunosuppression and in the absence of prophylaxis against Pneumocystis pneumonia, a specific treatment should be considered.

[Cytomegalovirus and systemic lupus: severe infection and difficult diagnosis]. / Cytomégalovirus et lupus érythémateux systémique: une infection grave de diagnostic difficile.

INTRODUCTION: There are a few reports only on cytomegalovirus (CMV) reactivation in lupus. Diagnosis of this infection is difficult and can be associated with of a poor outcome. We report three cases of infection with CMV that occurred in patients with lupus and review the literature. CASE REPORTS: The three reported patients presented with fever, polyarthritis, myocarditis and enteritis. Lupus was longstanding and the patients were receiving corticosteroids or cyclophosphamide. There was no major CD4 lymphopenia. The diagnosis was obtained with the presence of antigenemia pp65. The outcome was favorable with antiviral therapy in two patients, while the remaining patient died. In the English literature, pulmonary and intestinal involvement seem frequent, and associated with poor prognosis. CONCLUSION: In systemic lupus CMV infection is often serious and difficult to diagnose. Risk factors, treatment and prophylaxis remain to be evaluated in this population. The incidence of this infection could increase among patients receiving a biotherapy.

[Euthyroid Graves' disease: a case report]. / Le syndrome de Means: à propos d'une observation.

Euthyroid Graves' disease is defined as an ophtalmopathy without any clinical or biological signs of thyroid dysfunction. It highlights the apparent dissociation between orbitopathy and thyroid disease. Diagnosis is often too late while early treatment could really improve functional prognosis. We report a 57-year-old woman who presented with this entity and that illustrates both the diagnostic difficulty and disease course after intravenous corticosteroid therapy.

Primary Sjogren's syndrome in men.

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.

[Adult-onset Still's disease and hepatic angiosarcoma, a fortuitous association or a paraneoplastic syndrome: a case-report]. / Maladie de Still de l'adulte et angiosarcome hépatique, une association fortuite ou un syndrome paranéoplasique: à propos d'un cas.

Adult-onset Still's disease is a systemic disorder without specific histological feature. Diagnosis requires to rule out any other disorder including neoplasia. Nevertheless, patients with paraneoplastic adult-onset Still's disease have been reported. We report a patient with an adult-onset Still's disease who presented with a liver involvement at onset. Two years later, a liver angiosarcoma was diagnosed. This report underlines the difficulty of the diagnosis of the adult-onset Still's disease even in the presence of Yamaguchi et al.'s [J Rheumatol 19 (1992) 424-30] and Fautrel et al.'s [Medicine 81 (2002) 194-200] classification criteria and may suggest a link between the initial clinical picture and the discovery nearly two years later, of a liver angiosarcoma.

Stressful life events and pemphigus.

BACKGROUND: 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.

Influence of aspirin on the clinical outcomes of 103 anti-phospholipid antibodies-positive patients.

One hundred and three consecutive asymptomatic anti-phospholipid (aPL) antibody-positive carriers, taking aspirin (n=75) or not (n=28), were studied retrospectively to determine whether aspirin could provide primary prevention of anti-phospholipid syndrome (APS) symptoms. All patients positive for anti-cardiolipin antibodies (aCL; >25 UGPL or UMPL) and/or lupus anti-coagulant were followed for a mean of 64+/-24.7 months.Among aPL-positive patients, 37 had systemic lupus erythematosus (SLE), 20 had prolonged activated partial thromboplastin times, 19 had other connective tissue diseases, 16 had autoimmune thrombocytopenia (AIT), 11 had diverse diseases. Nineteen patients experienced thrombotic event(s) during follow-up. Clinical features, biological parameters and hydroxychloroquine use were comparable for the two groups, but thrombotic events differed (log-rank test; P=0.02). Four of the 10 SLE patients not taking aspirin developed thrombosis compared with 3/27 SLE patients taking aspirin (log-rank test; P=0.03). Anti-phospholipid -positive patients with AIT developed fewer thromboses while taking aspirin (log-rank test; P=0.01). In conclusion, aPL-positive SLE and AIT patients should take aspirin to prevent APS manifestations. Prospective therapeutic trials are needed to confirm aspirin's prophylactic role in such patients.

[MPO-ANCA related vasculitis presenting as chronic iron deficiency anemia due to paucisymptomatic intra-alveolar haemorrhage]. / Micropolyangéite révélée par une anémie d'évolution chronique secondaire à une hémorragie intra-alvéolaire paucisymptomatique: à propos d'un cas.

INTRODUCTION: An alveolar haemorrhage occurs in 12 to 29% during microscopic polyangeitis and can reveal this disease. EXEGESIS: We report the case of a fifteen years old female patient with a microscopic polyangeitis which was diagnosed during the investigation of a chronic anemia with chronic asymptomatic alveolar haemorrhage and extracapillary glomerulonephritis with antineutrophil cytoplasmic antibodies positive (anti-myeloperoxydase antibodies). The good tolerance of alveolar haemorrhage is usual for children, particularly in idiopathic pulmonary haemorrhage but is exeptionnal for adults. According to some studies, thirty percent of idiopathic pulmonary haemorrhage goes to auto-immune disease. CONCLUSION: This observation shows that complementary pulmonary investigations are necessary in chronic anemia when gynaecologic and digestive investigations are negative and that auto-immune investigations are judicious in the survey of idiopathic pulmonary haemorrhage.

[Assessment of plasma endothelin level measurement in systemic sclerosis]. / Intérêt du dosage plasmatique de l'endothéline au cours de la sclérodermie systémique.

PURPOSE: According to current knowledge, endothelin (ET)-1 plays an important role in the pathogenesis of systemic sclerosis (SSc). We assessed ET plasma levels in SSc patients according to the clinical presentation and the presence of complications such as pulmonary arterial hypertension (PAH). METHODS: Sixty-three consecutive patients with SSc were included. The control group included 17 healthy patients. ET plasma level was determined for all patients. Pulmonary function test and pulmonary high resolution computed tomography were performed in 44 patients and echocardiography in 51 patients, to screen for PAH, always confirmed by a right heart catheterization. RESULTS: ET plasmatic levels were higher in SSc patients than in healthy group subjects but the difference was not significant (3.72+/-1.13 vs 3.40+/-0.71 pmol/l, p=0.27). ET plasmatic levels were significantly higher in patients with PAH than in patients without PAH (4.28+/-0.65 vs 3.62+/-1.07 pmol/l, p=0.04) and in patients with anticentromere antibodies (3.96+/-1.11 vs 3.19+/-1.12 pmol/l, p=0.03). There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure (r=0.34, p=0.013). The best cut-off value for ET plasmatic level to discriminate patients affected by PAH was determined by ROC curve method: 4.1 pmol/l (sensibility 85.7%, specificity 66%). CONCLUSION: ET plasmatic levels were higher in SSc patients affected by PAH and patients with anticentromere antibodies. There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure. Assessment of ET plasmatic levels for detection and monitoring of pulmonary hypertension during SSc is warranted in larger prospective studies.

[Protein-losing enteropathy revealing an idiopathic constrictive pericarditis]. / Entéropathie exsudative révélant une péricardite chronique constrictive idiopathique.

INTRODUCTION: The cause of protein-losing enteropathy is sometimes difficult to establish. It can be rarely due to a constrictive pericarditis. EXEGESIS: We report a patient presenting a protein-losing enteropathy revealing a constrictive pericarditis. CONCLUSION: Chronic pericarditis should be evoked in case of unexplained protein-losing enteropathy. Echocardiography can sometimes be normal. Therefore, chest computed tomography scan or cardiac MRI followed by confirmation right heart catheterization should be performed in case of persistent unexplained protein-losing enteropathy.