[Cryofibrinogenemia: a single-center study at the University Hospital of Toulouse, France]. / Cryofibrinogénémie : étude monocentrique au CHU de Toulouse.

PURPOSE: Cryofibrinogenemia is an unknown disorder and studies dedicated to it are limited. The aim of our study was to report on the incidence, clinical manifestations and associated diseases in patients with isolated cryofibrinogenemia. METHODS: This is a retrospective single-center study. Patients included in this study had a positive and isolated detection of cryofibrinogen between January 1st, 2011 and December 31st, 2012. Identification was possible through the database of the laboratory of immunology. RESULTS: Two hundred and eighty-one consecutive orders of cryofibrinogenemia were identified. Seventy-three patients had a positive detection of cryofibrinogenemia. Among them, 12 had an isolated cryofibrinogenemia and sixty-one patients (84%) had concomitant cryofibrinogenemia and cryoglobulinemia. The mean age was 59±19years. Seven patients were female (58%). Cutaneous manifestations were present in half case. Peripheral nerve involvement was present in 5 cases (42%) and rheumatic manifestations in 4 patients (33%). A thrombotic event was reported in 7 patients (58%). Renal impairment was present in 7 patients. The median cryofibrinogen concentration was 254±304mg/L. Five patients had a secondary cryofibrinogenemia. The most often prescribed treatment was corticosteroids. CONCLUSION: Cryofibrinogenemia is an unknown disorder. Testing for cryoglobulinemia is more frequent than for cryofibrinogenemia whereas clinical manifestations are similar. Detection of cryofibrinogen is positive in most of the cases, with an important prevalence of thrombotic events in this population. This study confirms the importance of conducting prospective studies on cryofibrinogenemia.

[100 essential drugs. An internal medicine approach]. / Les 100 médicaments essentiels. Une approche de médecine interne.

PURPOSE: Up to 4600 drugs in about 15,000 pharmaceutical forms are available in France which may be a source of misuse with increased occurrence of side effects and costs. While the World Health Organization is encouraging each developed country to work out its own list of essential drugs. The list provided in 2008 by the French Office for the safety of health products has had so far limited impact on practice, so it became obvious to a group of internists to work out a "wise list" of 100 essential medicines covering 95% of the disorders observed in France. METHODS: In June 2011, 10 internists agreed to each provide a list of 100 essential medicines, according to individual experience. In December 2011, a meeting of the participants provided a list as initial consensus and mandated five among them to make proposals for those areas neglected by too many participants or in which needless dispersion of medicines was stated. After internet-facilitated exchanges, an additional list was validated in mild-January 2012. RESULTS: Fifty-four drugs were included in the list of initial consensus (including nine selected by all 10 participants), and 46 in the additional list. So the final "wise list" included 100 drugs. In June 2012, 56 of these drugs were available as generics. This list was compared to those lists set out by five countries in the European Union. CONCLUSION: Generating such a list is feasible. Undoubtedly still non-comprehensive, this list will benefit from the expertise of 14 general practitioners who are currently working out a similar list across France. The final list will be submitted for validation by the French associations of generalist teachers and Internists.

[Systemic glucocorticoid therapy: associated measures]. / Corticothérapie systémique : les mesures d'accompagnement.

Long-term treatment with glucocorticoids results in many adverse effects. Prevention of osteoporosis is well codified, but prevention of other adverse effects is not. If there is some consensus on the prevention of glucocorticoid-induced adverse events, there are also many habits since interventional studies are lacking. A low caloric and low carbohydrate diet as well as a regular physical training are certainly necessary to avoid lipodystrophy, weight gain and diabetes mellitus. Some patients benefit from the repeated intervention of a dietetic or nutrition specialist. Physical training is often neglected though it is efficacious to limit severity of glucocorticoid-induced myopathy and probably to reduce vascular risk. Low sodium intake has no effect on lipodystrophy and its efficacy to prevent hypertension is doubtful. Benzodiazepines may be useful against anxiety, insomnia and nervousness when these symptoms are cumbersome. Anti-ulcer drugs are generally not indicated because glucocorticoids are not ulcerogenic. Hypokaliemia rarely occurs, so we prefer controlling serum potassium level 1 and 3 months after glucocorticoid initiation rather than systematically prescribe potassium supplementation. Patients on glucocorticoids are at increased risk for cardiovascular events. Due to the lack of studies specific to patients on long-term glucocorticoid therapy, the rules for the prescription of statins are the same as in the general population. There is no known prevention for cutaneous atrophy. However, use of adhesive tape should be strictly avoided when skin atrophy is severe. Prevention of infections is developed elsewhere.

Bilateral choroidopathy in systemic lupus erythematosus.

We report a case of bilateral choroidopathy in a 35-year-old woman with systemic lupus erythematosus (SLE) diagnosed 3 years previously, and treated with hydroxychloroquine and steroids that ceased 6 months before ocular signs. She complained about rapid bilateral blurred vision with a severe loss of visual acuity. Fluorescein angiography found multiple leakage points in the posterior pole of the pigment epithelium. Ocular coherence tomography (OCT) and fundoscopy showed bilateral retinal detachments. Lupus choroidopathy was diagnosed and high steroids were given intravenously and allowed a rapid improvement. Visual acuity, fundoscopy, retinal angiography and OCT were normalized at 2 months. Choroidopathy is rarely reported in lupus and only about 30 patients are found in the literature.

[Frequency and risk factors of biological adrenal insufficiency screened by the 250 microg Synacthene stimulation test after a prolonged course of systemic glucocorticoid therapy. A study of 100 patients]. / Fréquence et déterminants de l'insuffisance surrénalienne biologique dépistée par le test au Synacthène à 250mug lors du sevrage d'une corticothérapie prolongée. Etude chez 100 patients.

PURPOSE: The frequency of adrenal insufficiency after a prolonged, continuous course of oral high-dose corticosteroids is poorly documented. We evaluated it retrospectively in our internal medicine department. METHODS: The patients were included between February 2000 and June 2007 and were administered a Synacthene 250 microg test (ST250) before tapering prednisone dose below 5mg per day. A non-responsive test was defined by a cortisol increase below 18 microg/dL, 60 min after stimulation. We also studied the risk factors associated with biological adrenal insufficiency by a multivariate logistic regression analysis. RESULTS: Hundred patients were included (mean age: 61.5+/-16.3 years). Mean initial dose of corticosteroids was 65.5+/-112 mg/d. Forty-five patients failed to respond to the ST250. A normal ST250 was negatively associated with a duration of corticosteroids therapy longer than 19.5 months (OR=0.38 [0.15-0.94]; p=0.04) and positively with an age over 63.5 years (OR=2.5 [1.1-6.4]; p=0.05). Two patients experienced a clinical adrenal insufficiency crisis. CONCLUSION: Biological adrenal insufficiency is very common after a prolonged course of oral high-dose corticosteroids. The risk does not seem to increase with age. The clinical benefit of a systematic ST250 at the time of corticosteroids withdrawal followed by hydrocortisone substitution if the test is non responsive remains unknown, and this practice is still a matter of debate.

[Pericarditis as the presenting manifestation of giant cell arteritis]. / Péricardite inaugurale d'une maladie de Horton.

INTRODUCTION: Pericarditis is rarely the presenting manifestation of giant cell arteritis. We report two additional patients. CASE REPORTS: Two patients aged over 70 years presented with acute chest pain. Echocardiography evidenced a pericarditis. Laboratory features showed increased level of acute phase reactants. On questioning, both patients had cephalic symptoms related to giant cell arteritis. Temporal artery biopsy histopathology was characteristic of giant cell arteritis in both. Oral prednisone therapy (20mg/D) led to a complete remission with no clinical relapse of pericarditis after a follow-up of one year and three years, respectively. CONCLUSION: Giant cell arteritis must be evoked in elderly patients with pericarditis because corticosteroids are necessary to avoid ischemic complications of the disease. However, fortuitous association can also be considered. No severe complication of pericarditis has been reported in the literature on corticosteroid therapy.

Vaccination in adults with auto-immune disease and/or drug related immune deficiency: results of the GEVACCIM Delphi survey.

INTRODUCTION: There are insufficient data regarding the efficacy and safety of vaccination in patients with auto-immune disease (AID) and/or drug-related immune deficiency (DRID). The objective of this study was to obtain professional agreement on vaccine practices in these patients. METHODS: A Delphi survey was carried out with physicians recognised for their expertise in vaccinology and/or the caring for adult patients with AID and/or DRID. For each proposed vaccination practice, the experts' opinion and level of agreement were evaluated. RESULTS: The proposals relating to patients with AID specified: the absence of risk of AID relapse following vaccination; the possibility of administering live virus vaccines (LVV) to patients not receiving immunosuppressants; the pertinence of determining protective antibody titre before vaccination; the absence of need for specific monitoring following the vaccination. The proposals relating to patients with DRID specified that a 3-6 month delay is needed between the end of these treatments and the vaccination with LVV. There is no contraindication to administering LVV in patients receiving systemic corticosteroids prescribed for less than two weeks, regardless of their dose, or at a daily dose not exceeding 10mg of prednisone, if this involves prolonged treatment. Out of 14 proposals, the level of agreement between the experts was "very good" for eleven, and "good" for the remaining three. CONCLUSION: Proposals for vaccine practices in patients with AID and/or DRID should aid with decision-making in daily medical practice and provide better vaccine coverage for these patients.

Increased exposure to statins in patients developing chronic muscle diseases: a 2-year retrospective study.

OBJECTIVE: Case reports have suggested that lipid-lowering drugs (LLDs), especially statins, could induce or reveal chronic muscle diseases. We conducted a study to evaluate the association between chronic muscle diseases and prior exposure to LLDs. METHOD: This was a retrospective study of chronic primary muscle disease cases newly diagnosed at the Toulouse University Hospitals between January 2003 and December 2004 among patients living in the Midi-Pyrénées area, France. All patients remained symptomatic for more than 1 year after drug withdrawal, or required drugs for inflammatory myopathy. Data on the patient's exposure to LLDs and to other drugs were compared with that of matched controls (5/1) selected through the Midi-Pyrénées Health Insurance System database. RESULTS: A total of 37 patients were included in the study. Of those, 21 (56.8%) suffered from dermatomyositis (DM) or polymyositis (PM), 12 (32.4%) from genetic myopathy, and 4 (10.8%) from an unclassified disease. The prevalence of exposure to statins was 40.5% in patients and 20% in controls (odds ratio (OR) 2.73, 95% confidence interval (CI) 1.21-6.14; p<0.01). There was a significant positive interaction between statins and proton pump inhibitors exposure (weighted OR 3.3, 95% CI 1.37-7.54; p = 0.02). Statin exposure rate was 47.6% among patients with DM/PM (OR 3.86, 95% CI 1.30-11.57; p<0.01). There was no difference between patients and controls for exposure to fibrates. CONCLUSION: Patients who developed chronic muscle diseases after the age of 50, including DM/PM, had a higher than expected frequency of prior exposure to statins. Further studies are needed to confirm this association and the role of proton pump inhibitors.

[Fulminant alveolar hemorrhage: evolution of giant cell arteritis to ANCA-positive vasculitis?]. / Hémorragie intra-alvéolaire fulminante : transformation d'une maladie de Horton en vascularite ANCA-positive?

INTRODUCTION: Giant cell arteritis (GCA) is a granulomatous vasculitis of the large and medium size vessels with a remarkable sensitivity to corticosteroids, although it may be dependent to therapy. In rare cases, a vasculitis of the medium or small-size vessels may mimic, be associated to, or follow GCA. We report a case of GCA dependent to corticosteroids that was followed five years after diagnosis by an alveolar hemorrhage leading to the diagnosis of a possible Wegener's granulomatosis. EXEGESIS: A 70-year-old man had a diagnosis of GCA fulfilling the ACR criteria in 1999. Temporal artery biopsy revealed a typical histological pattern. The initial response to corticosteroids was excellent, but the patient became dependent to corticosteroids, so he was given methotrexate from 2002. Severe alveolar haemorrhage occurred in December 2004, leading to the diagnosis of possible ANCA positive, anti-proteinase 3 positive Wegener's granulomatosis. CONCLUSION: ANCA-positive vasculitis may complicate the course of GCA. This evolution should be rapidly recognized, because its treatment differs to that of GCA.