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Iron deficiency, without anaemia, is common in the general population and induces various symptoms. Its management consists of oral and intravenous supplementation for cases of inefficacy of or intolerance to oral iron. We assessed the efficacy of intravenous iron therapy in non-anaemic iron-deficient patients with fatigue. We prospectively evaluated the level of fatigue, using the Fatigue Severity Scale (FSS), in patients suffering from iron deficiency without anaemia, treated by intravenous iron at the moment of the perfusion (W0), after 4 weeks (W4), and 12 weeks (W12). Of 25 patients, at W0, the mean FFS was 49.3+/-13.7. There was a significant improvement in FSS at W4 (44+/-15; p = 0.01) and a sustained response at W12 with an FFS of 35.8+/-17.1 (p < 0,0001). There was no correlation between FSS and serum ferritin level at W12 (p=0.54) or between serum ferritin at W12 and difference between FSS at W0 and W12 (p=0.58). There were six mild adverse events (24%): asthenia (8%), nausea (8%), headache (4%), local pain (4%); and no serious adverse events. Our results suggest the rapid efficacy of intravenous iron in improving fatigue in iron deficiency without anaemia with a good profile of tolerance.

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AIMS: Raynaud's phenomenon is a vasospastic disorder of the extremities that can lead, in the hands, to pain, disability, ischemic ulcers and digital chronic ischemia. Medical and surgical current treatments are not fully effective while causing side effects. Recent studies have emphasized the value of botulinum toxin type A (BTX A) in the management of primary Raynaud's phenomenon. The originality of Raynaud's syndrome secondary to systemic sclerosis is to combine both arterial vasospasm and sclerosis of the arterial wall, what is supposed to reduce BTX A effects. The purpose of this work is to evaluate BTX A efficiency in patients with Raynaud's phenomenon secondary to systemic sclerosis. PATIENTS AND METHOD: We performed a prospective study for 12 months. Patients with severe Raynaud's phenomenon due to systemic sclerosis were injected with BTX A in the two hands. Evolution of ischemic ulcers, QuickDASH Score, O2 partial pressure, pain were measured before and 30 days after injection. RESULTS: We treated 18 patients. Thirty days after injection, we noticed a complete healing of ulcers, QuickDASH Score was improved from 39.4 to 20, as the O2 partial pressure from 16 to 42 mmHg and the pain from VNS from 6/10 to 2/10. CONCLUSION: BTX A appears to improve significantly Raynaud's phenomenon symptomatology in patients with systemic sclerosis despite the component of arterial sclerosis.

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INTRODUCTION: Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis. CASE REPORTS: We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded. CONCLUSION: Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.

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INTRODUCTION: Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric oedema on neuroimaging. Many pathological conditions and treatments have been associated with this syndrome. CASE REPORT: We report a 19-year-old woman, followed-up for hypocomplementemic urticarial vasculitis, who presented with a posterior reversible encephalopathy syndrome induced by the intake of an over-the-counter cold remedy containing pseudoephedrine. Clinical manifestations and radiological abnormalities resolved after anti-hypertensive therapy and withdrawal of sympathomimetic drug. CONCLUSION: The diagnosis of posterior reversible encephalopathy syndrome should be considered in patients with compatible clinical and radiological presentation because of its potential reversibility with an appropriate management. Intake of drugs, including over-the-counter cough and cold drugs, should be looked for in the history as well as autoimmune disorders.

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INTRODUCTION: Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegener's granulomatosis. CASE REPORTS: Data from four patients were studied retrospectively. Three patients had associated airway localization and three had kidney involvement. All patients had positive test for anti-PR3 antineutrophil antibodies. Two patients presented with dilated cardiomyopathy (one with terminal cardiac failure), another patient with complete atrioventricular block and pericarditis, and the remaining one with myopericarditis. One patient was asymptomatic. For three of these patients, the cardiac manifestations were contemporary of the diagnosis of Wegener's granulomatosis and had a severe disease course. CONCLUSION: Cardiac events in Wegener's granulomatosis are probably underestimated, given the various type of heart damage and the clinical presentation. Cardiac involvement seems to be associated with a poor prognosis. Thus, we recommend systematic and regular cardiac assessment in the follow-up of patients with Wegener's granulomatosis.

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PURPOSE: During bone-marrow biopsy, one third of patients score their pain as moderate or severe. Combination of analgesic and hypnotic is effective at reducing pain, but prolonged medical surveillance is necessary. The objective of the study was to assess the effectiveness and ease of use of the equimolar nitrous oxide-oxygen mixture (nitrous oxide), a short acting analgesic with little sedative effect, during bone-marrow biopsy. METHODS: As part of a non-controlled prospective observational study, patients undergoing a bone-marrow biopsy received nitrous oxide as an adjuvant to local anaesthesia. Facemask was self-maintained. A questionnaire was given after completion of the procedure to assess the pain (with a numerical-rating scale [RS] ranging from 0 to 10) as the main criterion and the ease of the procedure, the tolerance, and satisfaction, as secondary criteria. The physician noted adverse reactions. RESULTS: Nineteen women and 21 men were included. The median age was 51 years. Ninety percent of patients felt slight pain, less than 5 out of 10 on the RS. Only one patient had difficulty in keeping the mask. A patient experienced nausea and four presented a fleeting euphoria noticed by the physician. Ninety-five of patients wished to use nitrous oxide again should further bone-marrow examinations be necessary. CONCLUSION: Nitrous oxide is an effective analgesic when performing bone-marrow biopsies. Ten percent of patients feel a moderate to severe pain instead of one third. Despite some mild side effects, there is a very good appreciation by patients. Since this study, the authors routinely use nitrous oxide.

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PURPOSE: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder and early treatment is vital. Here, we review the recent advances in the understanding of the pathophysiology of TTP and its treatment. CURRENT KNOWLEDGE AND KEY POINTS: Recent advances have shown that TTP is caused by deficiency of the (ADAMTS-13) metalloprotease that cleaves von Willebrand factor multimers. Acquired TTP is associated to inhibitory antibodies directed against ADAMTS-13. This has led to assess new therapeutic approaches in refractory and relapsing forms of TTP and the use of rituximab has shown very encouraging results. FUTURE PROSPECTS AND PROJECTS: A better characterization of TTP amongst the other thrombotic microangiopathies has allowed the use of new therapeutic approaches with the use of rituximab. The encouraging results reported with rituximab in some forms of TTP challenge the classic treatment based on plasma exchanges.

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INTRODUCTION: Only few series have reported the association of autoimmune hepatitis with antiphospholipid antibodies. The aim of our study is to investigate the frequency of these antibodies in a series of autoimmune hepatitis and to search for a correlation with clinical, biological or histological characteristics. MATERIAL AND METHODS: Antiphospholipid were investigated in 24 patients with well defined autoimmune hepatitis. Characteristics were compared between antiphopholipids positive and negative patients. Characteristics of our patients were also compared toward cases collected in a literature review. RESULTS: The frequency of antiphospholipid antibodies is of 70.8% in our series. Four patients had a well defined antiphospholid syndrome. Seven patients had a systemic lupus erythematosus in the antiphospholipid group whereas none in the antiphospholipid negative group. The frequency of the different antiphopholipid antibodies was: IgG ACL (52.9%), IgM APE (52.9%), ACC (43.7%), IgG Abeta2GP1 (41.2%). We found no correlation between hypergammaglobulinemia and the presence or the isotype of antiphospholipid antibodies. Clinical presentation and outcome as biological and histological parameters were similar in both groups. CONCLUSION: Our study report a high frequency of antiphospholipids antibodies in autoimmune hepatitis patients. However we found no clinical, biological or histological correlation with the presence of antiphospholipids. Further longitudinal studies on larger cohorts should clarify the association between antiphospholipid antibodies and autoimmune hepatitis and potential therapeutic issues.

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INTRODUCTION: Familial macrothrombocytopenias are a group of rare autosomal dominant platelet disorders including many syndromes in particular the May-Hegglin anomaly. They are characterized by thrombocytopenia with giant platelets and in some cases neutrophilic inclusions in peripheral blood granulocytes. Recently these different clinical entities have been demonstrated to be linked to mutations in the same gene, MYH9. CASE REPORT: We report in a young African woman presenting as a May-Hegglin anomaly a new mutation of the MYH9 gene. In regard of this case we present a brief review of the MYH9 syndrome. CONCLUSION: The MYH9 syndrome includes now several clinical entities who share some common clinical and biological characteristics such as a thrombocytopenia with giant platelets, presence or absence of other manifestations including Dohle like bodies, nephritis, sensorineural hearing loss, cataract. We report a new case in which a new mutation of the MYH9 gene was evidenced.

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OBJECTIVE: Anaerobic deep abscesses are rare and may have unusual location leading to severe outcome due to delayed diagnosis and treatment. In order to improve their diagnosis, we report and analyse 5 new cases. METHODS: Patients were seen from 1999 to 2003 in a single department of internal medicine of the university hospital of Marseille. RESULTS: Five new cases were diagnosed consisting in 3 females and 2 males with a medium age of 56,8 years, with unusual location in 4 cases: epidural (2), psoas (1) and sub-diaphragmatic (1) or circumstances in one case of pulmonary abscess unrelated to inhalation. Predisposing conditions thought to compromise resistance to infection were found in all cases: social poverty (4/5), alcoholism (3/5), smoking (4/5), teeth and periodontal disease (4/5), neoplasia (2/5), iatrogenic disease (2/5). Symptoms were insidious (5/5) and unspecific but were always related to the abscess location. Abscesses were frequently found distant from the initial focus of infection because of frequent hematogenous spread (4/5). Drainage of the collection led to bacterial identification in all cases (4/4), although blood cultures could be positive (3/5) and helpful in one case in which drainage was not possible (1/5). The isolated organisms always corresponded to the suspected initial focus (oropharynx 4/5 and digestive 1/5). Finally, combination of surgical drainage and double prolonged antibiotherapy (penicillin+metronidazole) was the elected treatment. CONCLUSION: Since hematogenous diffusion is frequent, anaerobic infection should be suspected in any case of deep abscess affecting patients with predisposing conditions such as poverty, severe teeth disease or iatrogenic procedure.

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PURPOSE: To describe clinical, biological characteristics and associated diseases of cold agglutinins in adults. METHODS: Retrospective study in a single department of internal medicine from 1997 to 2002. The inclusion criteria were a positive direct Coombs test and a positive research for cold-reactive autoantibodies. We recorded for each patient: clinical presentation at onset and during follow-up, biological parameters of haemolysis, biological characteristics of the cold agglutinin and associated diseases. RESULTS: Fifty-eight patients (34 females, 24 males), with medium age of 58.8 were included in the study. Clinical presentation was highly variable between acute life-threatening haemolysis and absence of symptoms. Results of direct antiglobulin test were C3 (74%), IgG + C3 (22.4%), IgG (3.4%). Titer, thermal amplitude, strength and specificity of Coombs test were correlated, in all cases except 6, with cold agglutinin haemolytic activity. In 77.6% of cases cold agglutinin was secondary; related to: autoimmune disorders (n = 19), lymphoproliferative disorders (n = 11) and infections (n = 10). CONCLUSION: Clinical presentation of cold agglutinin is highly variable and not always related to the biological characteristics of the bound antibody (titer, thermal amplitude, specificity). In our single center study, diseases associated with cold agglutinin were various with the highest frequency of auto-immune disorders. Our study underlined also the high frequency of lymphoproliferative disorders and justifies a close follow-up of these patients. Finally, we reported a high frequency of hepatitis C virus infection among the infectious aetiologies.

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INTRODUCTION: Influenza virus outbreaks occur each year, in France, during autumn and winter. Influenza-associated acute encephalitis were reported during epidemics or pandemics. Sporadic cases are rarely identified probably because influenza virus is not searched among etiology of febrile encephalitis. EXEGESIS: We report a case of influenza-associated encephalitis complicated by adrenal insufficiency in a young woman. Diagnosis was based on seroconversion of serum influenza virus A antibodies (complement fixation test). Follow up of the patient showed a total recovery. CONCLUSION: Influenza must be searched for any febrile encephalitis occurring during winter. Reverse transcriptase polymerase chain reaction (RT-PCR) on cerebrospinal fluid should be assessed. It is not actually a routine technique and we do not know yet if it is accurate enough for diagnosis. So, it is important to identify influenza virus and obtain documentary evidence concerning neurological impairment. Nevertheless, a better understanding of pathogenesis and use of vaccination are needed to improve prognosis.

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INTRODUCTION: Shrinking lung syndrome usually manifest in dyspnea, decreased lung volume associated with elevated diaphragm. It reports with systemic autoimmune disease and physiopathological mechanism is controversial. EXEGESIS: We report three shrinking lung syndrome observations in which two cases were diagnosed at the time to onset of autoimmune disease. The three patients were treated with corticosteroid, two of them necessitated theophylline. Review of the literature highlight 60 cases and permit to discuss physiopathological mechanisms which remain uncertain. Diaphragmatic dysfunction (because of myositis or neuropathy) represented by abnormal transdiaphragmatic pressures is actually discussed. CONCLUSION: Shrinking lung syndrome is rare but must be considered in patient with autoimmune disease and dyspnea. The diagnosis can be difficult because of clinical, pathological and functional features which are controversial. The optimum treatment is unknown.

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