[Polyarteritis nodosa, an alternative diagnosis of giant cell arteritis in cases of temporal arteritis]. / La périartérite noueuse, un diagnostic alternatif à l'artérite gigantocellulaire devant une artérite temporale.

INTRODUCTION: Weight loss, myalgias, neurologic manifestations and arterial hypertension are common features of polyarteritis nodosa (PAN) at diagnosis. Temporal arteritis is a rarer manifestation of PAN, more suggestive of giant cell arteritis (GCA). CASE: We report the case of a 77-year-old woman who presented with fatigue, weight loss, fever, neck pain, jaw claudication and cough, diagnosed with giant cell arteritis. Diagnosis was reconsidered in favour of a medium and small-sized vessels necrotizing vasculitis corresponding to PAN because of steroid dependence, mononeuritis and suggestive histological features. CONCLUSION: Although temporal arteritis is suggestive of GCA, other causes of temporal arteritis can be identified with temporal artery biopsy.

[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.

Pituitary-adrenal function after prolonged glucocorticoid therapy for systemic inflammatory disorders: an observational study.

CONTEXT: Glucocorticoid therapy is being used in a wide variety of systemic disorders. Reference papers, published more than 20 years ago, showed no correlation between adrenal insufficiency risk and dose or duration of glucocorticoid therapy. OBJECTIVE: Our objective was to evaluate the extent to which long-term glucocorticoid therapy damages the pituitary-adrenal axis in patients with systemic inflammatory disorders. DESIGN: We conducted a retrospective observational study from January 2011 to August 2012. SETTING: This was a monocentric study at the Department of Internal Medicine, Bichat Hospital, Paris-Diderot University, Paris, France. PARTICIPANTS: Sixty consecutive patients who were receiving long-term prednisone therapy for systemic inflammatory disorders and in whom discontinuation of glucocorticoid treatment was planned. INTERVENTION: A short Synacthen test was performed. A bolus of 0.25 mg 1-24-ACTH was injected in the morning, 24 hours after the most recent dose of prednisone. Cortisol was measured at baseline and 60 minutes after Synacthen injection. MAIN OUTCOME MEASURES: We assessed frequency and risk estimate of pituitary-adrenal dysfunction. RESULTS: Twenty-nine patients (48.3%) had adrenal insufficiency defined by a plasmatic cortisol <100 nmol/L (n = 13) at baseline (time 0) or <550 nmol/L (n = 16) 60 minutes after Synacthen injection. Cumulative dose (area under the receiver operating characteristic curve = 0.77 [95% confidence interval = 0.62-0.91], P = .007) and exposure (area under the receiver operating characteristic curve 0.80 [95% confidence interval = 0.67-0.93], P = .002) to prednisone were predictive for adrenal insufficiency based on a T0 <100 nmol/L. Prednisone was stopped in 29 of 31 patients (93.5%) showing a normal response to short Synacthen test; none of these patients required hydrocortisone replacement with a mean follow-up of 10 (± 6) months. CONCLUSION: Adrenal insufficiency is frequent in patients treated with long-term glucocorticoids for systemic inflammatory disorders and is related to duration and cumulative dose of steroids.

[Long-term follow-up of a French cohort of retroperitoneal fibrosis]. / Fibrose(s) rétropéritonéale(s) : stratégie diagnostique, pathologies associées et suivi à long terme d'une cohorte française.

PURPOSE: Retroperitoneal fibrosis (RPF) is a rare disease with an expanding etiologic spectrum. We aimed to analyze non-invasive diagnosis strategy, associated disorders, monitoring, treatment and prognosis. METHODS: Retrospective cohort study in a single tertiary center. RESULTS: Eighteen RPF cases (11 males) followed between 1996 and 2009 were reviewed. Blood CRP level was high in all cases before treatment. CT scan, associated or not with MRI or 18-FDG PET-scan, confirmed the diagnosis in 15 patients. Histological analysis of a surgical biopsy specimen was performed in only three cases. Ten patients suffered retroperitoneal fibrosis secondary to systemic vasculitis (granulomatosis with polyangeitis, n=1, Takayasu aortitis, n=2), systemic fibrosis with Riedel thyroiditis (n=1) and atheromatous periaortitis (n=6). Fifteen patients were treated with corticosteroids with a mean treatment duration of 60 months (12-228). Dependency to corticosteroids was recorded in ten patients. Patients with fibrosis related to vasculitis were younger, had a higher CRP level, more frequent corticosteroid dependency and a higher relapse rate. Relapses were successfully treated with steroids. Immunosuppressive treatment was only prescribed in the setting of systemic vasculitis. No patient died, after a 6±2 years follow-up. Late relapses could occur, sometimes years after steroid therapy cessation. CONCLUSION: In our study, RPF occurred as a secondary disorder in 60% of the cases. Disease extension, relapse rate and treatment response varied according to the underlying cause of RPF, pleading for an extensive and systematic initial assessment. Since no death or end-stage renal insufficiency was observed, RPF might be considered as a steroid-sensitive and benign disorder.

Intensified treatment of type 1 diabetes: prospective evaluation at one year of a therapeutic patient education programme.

BACKGROUND: Type 1 diabetes treatment requires not only an intensive insulin regimen, but also intensive management, which daily involves participation of the patient. Our aim was to prospectively evaluate the efficacy on patient skills and metabolic control of our routine 5-day in-patient education programme. METHODS: Over one year, each type 1 diabetic patient undertaking the programme for the first time, and able to complete a 2, 6, and 12 month follow-up, participated in the study (n=76). At baseline (T0), 61 patients had intensified insulin treatment and 15 increased from 2 to 3 daily insulin injections during the hospital stay. Using questionnaires and analysis of a glycaemia logbook, we evaluated at T0, T2, T6 and T12 patient skills related to diet, physical exercise, self-monitoring of blood glucose (SMBG), adjustment of insulin doses, and treatment of hypoglycaemia. Metabolic control was evaluated at the same time. RESULTS: At one year, the following skills improved: Intake of carbohydrates (T0: 59%, T12: 90% of patients, p<0.001) and snacks (p<0.001), appropriate physical exercise (p<0.001), frequency of SMBG (T0: 3.3 +/- 1.5/day, T12: 4.5 +/- 1/day, p<0.001), frequency of post-prandial tests (p<0.001) and adjustment of insulin doses (T0: 18%, T12: 53% of patients, p<0.001). More patients always carried sugar (T0: 61%, T12: 97%, p<0.001) and appropriately treated hypoglycaemia (T0: 48%, T12: 79%, p<0.001). Concurrently, HbA(1c) decreased (T0: 8.6 +/- 1.5%, T12: 7.7 +/- 0.9%, p<0.001), and the frequency of hypoglycaemia was reduced (p<0.001). There was a correlation between the decrease of HbA(1c) and the frequency of SMBG (p<0.001, r2=0,24). CONCLUSIONS: Education led to positive changes in patient skills, which were maintained over one year and were associated with improved metabolic control.

[Healthcare networks: involvement of various healthcare professionals]. / Les réseaux de soins: multidisciplinarité et équipe.

Diabetes is a very representative model of chronic disease requiring a partnership between various healthcare professionals. The organisation of healthcare networks allows a global care improvement. For the "Midi-Pyrénées" area, the diabetes network associates physicians and other healthcare professionals, working in their private - practice or in clinics or hospitals. The priority of this network was to develop therapeutic patient education, outdoors and indoors. The use of a specific communication system will be unavoidable, as a basis for medical files, evaluation, and permanent updates for the network members. Current projects concern a regional organization for chronic foot ulcers care, coronary heart disease and diabetes during pregnancy. These various care domains suppose the collaboration with other regional networks.

[Diabetic foot]. / Le pied diabétique.

Diabetic foot ulcers are a frequent and severe complication of diabetes mellitus. A multidisciplinary approach (in-patient and out-patient care) improves prognosis and reduces the amputation rate. Risk factors are well defined and easily identified. High-risk patients (sensory loss, vascular disease, previous ulcer, foot deformities) have to be detected. In France, comprehensive foot-care programs, including education and regular foot examination, must be developed. The reduction of the economical and human burden needs the urgent formation of more multidisciplinary teams (to take care of patients properly and very early), foot care networks, and prevention programs.