Systemic lupus erythematosus-associated acute transverse myelitis: manifestations, treatments, outcomes, and prognostic factors in 20 patients.

BACKGROUND: Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE). This retrospective multicentre study identifies the prognostic factors in a relatively large patient series. PATIENTS AND METHODS: Twenty patients fulfilled the SLE criteria of the ACR classification and the Transverse Myelitis Consortium Working Group. A severe neurological flare was defined as muscle strength grade <3/5 in more than half the muscle groups at the motor neurological level. Inability to run or another significant ambulation-unrelated disability was considered as 'unfavourable neurological outcome'. RESULTS: Myelitis was the first SLE symptom in 12 patients; in the eight others, it occurred 8.6 years (median delay) after SLE onset. Eleven patients presented severe neurological impairments. The treatment included corticosteroids in all patients associated with intravenous cyclophosphamide in 11 and/or hydroxychloroquine in 14. Unfavourable outcomes were observed in 53% of the patients at six months and in 28% at end of follow-up (median: 5.9 years). An initial severe neurological impairment and no cyclophosphamide use were associated with unfavourable neurological outcomes at six months and at end of follow-up, respectively. CONCLUSION: Transverse myelitis may reveal SLE or occur more than 10 years after SLE diagnosis. The initial severity of the neurological flare (with paraplegia) is the main prognostic marker. The study provides arguments for cyclophosphamide use.

Adult-onset Still's disease and pregnancy: about ten cases and review of the literature.

Little is known about the interactions between adult-onset Still's disease (AOSD) and pregnancy. In an attempt to clarify the link between these 2 conditions, we retrospectively analyzed patients registered as suffering from AOSD seen in our university hospital. A total of 57 patients, among them 30 women, were diagnosed. Ten pregnancies in 8 women were identified. Three cases manifested AOSD in their first trimester, all treated with prednisone. Premature births and flares occurred in 2 patients. One patient developed a monocyclic AOSD during her second pregnancy's postpartum. In the 4 other cases, AOSD was known and quiescent before pregnancy. One patient had 2 pregnancies without any flare or complication. One patient experienced her first pregnancy while under treatment and presented a late flare 8 months after delivery. The third patient developed exacerbation in the first trimester of her second pregnancy which was treated with IgIV alone. The last one presented her first pregnancy 7 years after diagnosis. A prednisone-treated systemic flare occurred during the first trimester without later complication. Based on our own experience and the analysis of only two series of the literature, including, respectively, 4 and 5 patients, we suggest that two settings could be distinguished. First, AOSD can occur during pregnancy and can be responsible for obstetrical complications. Then, in patients with known AOSD, the second trimester and postpartum appear to be periods exposing to disease recurrence. Thus, we recommend a close multidisciplinary monitoring by a rheumatologist and an obstetrician prior to, during and after pregnancies.

[Spontaneous adrenal hematomas. Retrospective analysis of 20 cases from a tertiary center]. / Hématomes surrénaliens non traumatiques : série rétrospective de 20 cas.

INTRODUCTION: Spontaneous adrenal hemorrhages (AH) are a rare condition with no consensus about their management. METHODS: Patients were identified using the Medicalization of the Information System Program database, imaging software and a call for observations to internists, intensivists and obsetricians working at our institution. Adult patients whose medical records were complete and whose diagnosis was confirmed by medical imaging were included. RESULTS: From 2000 to 2007, 20 patients were identified, including 15 were women. The clinical onset of AH was non-specific. In five cases, AH occurred during pregnancy; four of them were unilateral and right sided. The etiology of the other fifteen (bilateral adrenal hemorrhage in 11) were as follows: antiphospholipid syndrome (n=8), heparin-induced thrombocytopenia (n=4), essential thrombocythemia (n=3), spontaneous AH due to oral anticoagulants (n=1), complication of a surgical act (n=3), and sepsis (n=3). In seven cases, two causes were concomitant. The diagnosis of AH was often confirmed by abdominal CT. An anticoagulant treatment was initiated in 16 cases. Ten of the eleven patients presenting with bilateral adrenal hematomas were treated using a long-term substitute opotherapy. One patient died because of a catastrophic antiphospholipid syndrome. CONCLUSION: The clinical onset of HS is heterogeneous and non-specific. The confirmatory diagnosis is often based on abdominal CT. The search for an underlying acquired thrombophilia is essential and we found in this study etiological data comparable to the main series in the literature. Adrenal insufficiency is most of the time definitive in cases of bilateral involvement.

Spinal cord sarcoidosis: report of seven cases.

BACKGROUND: Spinal cord involvement in sarcoidosis is rare, occurring in <1% of patients with sarcoidosis. METHODS: We report seven cases of spinal cord sarcoidosis, seen in two French hospitals over a 13-year period. Presentation of disease, methods of diagnosis and response to treatment, with quantification according to the reduction of the modified Rankin scale (MRS), were noted. RESULTS: Six patients presented insidious paresthesias or weakness and one a sudden paraplegia. Average MRS at diagnosis was to 2. Spine MRI showed one or several intramedullary lesions in all cases. Diagnosis was confirmed by extra-neural tissue biopsies in all cases, including mediastinoscopy (two patients), coelioscopy (one patient), bronchoscopy (one patient), salivary gland biopsy (one patient) and skin biopsy (two patients). The average follow-up for the group was 49.4 months. All patients responded to corticosteroid therapy with a median reduction of MRS of one point. Five patients received immunosuppressive therapy: cyclophosphamide (two patients), methotrexate (two patients), azathioprine (one patient), mycophenolate mofetyl (one patient), with an inconstant benefit. Patients who received cyclophosphamide presented severe fungaemia. CONCLUSION: Based on our study and literature analysis, we propose an algorithm for treatment of spinal cord sarcoidosis.

[Thymic carcinoid tumor revealed by a Cushing's syndrome: usefulness of positron emission tomography]. / Tumeur carcinoïde du thymus révélée par un syndrome de Cushing: intérêt de la tomographie par émission de positons.

Ectopic adrenocorticotropic (ACTH) syndrome is a rare condition, generally due to lung or carcinoid tumors. 18-fluorodeoxy-glucose positron emission tomography ((18)FDG-PET) can be useful where conventional localization techniques often fail. A 50-year-old man presented with sudden diffuse oedema, hypokaliemic alkalosis, Diabetes mellitus and high serum levels of ACTH and cortisol. Ectopic ACTH syndrome was confirmed leading to ketoconazole treatment. Chest-computed tomography only revealed an aspecific anterior mediastinal nodule that was hypermetabolic on the whole body-(18)FDG-PET. A thymic tumor was suspected and the patient had a thymectomy that revealed an atypical carcinoid tumor with pleural carcinosis. The postoperative course was favorable with clinical and biochemical remission of neoplastic Cushing's syndrome.

[Thymoma and autoimmune diseases]. / Thymomes et maladies auto-immunes.

The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later. Two situations require the systematic investigation of a thymoma: the occurrence of myasthenia gravis or autoimmune erythroblastopenia. Nevertheless, the late onset of systemic lupus erythematosus or the association of several autoimmune manifestations should lead to look for a thymoma. Neither the characteristics of the patients nor the pathological data can predict the occurrence of an autoimmune disease after thymectomy. Thus, thymectomy usefulness in the course of the autoimmune disease, except myasthenia gravis, has not been demonstrated. This seems to indicate the preponderant role of self-reactive T lymphocytes distributed in the peripheral immune system prior to surgery. Given the high infectious morbidity in patients with thymoma, immunoglobulin replacement therapy should be considered in patients with hypogammaglobulinemia who receive immunosuppressive therapy, even in the absence of prior infection.

[Interleukin-1, inflammasome and autoinflammatory diseases]. / Interleukine-1, inflammasome et maladies auto-inflammatoires.

Interleukin-1 is a major cytokine of innate immunity and inflammation. It exerts various systemic effects during the inflammatory response, such as fever induction, thrombopoiesis and granulopoiesis, or leukocyte recruitment. Its involvement has been demonstrated in many inflammatory-mediated diseases, such as diabetes or gout. Moreover, interleukin-1 plays a pivotal role in some autoinflammatory diseases, such as cryopyrinopathies or familial Mediterranean fever. In these diseases, a constitutional defect of the inflammasome, a protein complex responsible for the activation of interleukin-1, explains the hypersecretion of interleukin-1. Other autoinflammatory diseases have a more complex pathophysiology involving deregulation of the interleukin-1 pathway, upstream or downstream of the inflammasome, or through more complex mechanisms. In this review, we are detailing the synthesis, the activation, the signalling, and the regulation of interleukin-1. We then describe the autoinflammatory diseases or related-diseases where the pathological role of interleukin-1 has been demonstrated.

[Sarcoidosis demonstrated by fluorodeoxyglucose positron emission tomography in a case of granulomatous myopathy]. / Apport de la tomographie par émission de positons pour le diagnostic de sarcoïdose au cours d'une myopathie granulomateuse.

INTRODUCTION: Granulomatous myositis is a rare condition that has been described in association with sarcoidosis. In the absence of sarcoidosis or other underlying disease, a diagnosis of isolated granulomatous myositis is considered. OBSERVATION: A 61-year-old African man presented with progressive limitation in running and proximal atrophy of the lower limbs for the past year. Quadricipital muscle biopsy revealed non-caseating epithelioid granulomas and multinuclear giant cells. Whole body fluorodeoxyglucose positron emission tomography ((18)FDG-PET) revealed hypermetabolic activity of salivary and lachrymal glands, and mild hypermetabolism in the mediastinal lymph nodes. Minor salivary gland biopsy was consistent with sarcoidosis. CONCLUSION: To our knowledge, this is the first reported case of sarcoid myopathy demonstrating the diagnostic usefulness of (18)FDG-PET.

[Off-label use of intravenous immunoglobulin therapy in the treatment of lupus myocarditis: Two case reports and literature review]. / Utilisation hors AMM d'immunoglobulines intraveineuses dans le traitement de la myocardite lupique : à propos de deux cas et revue de la littérature.

INTRODUCTION: Several case reports have reported the benefit of intravenous immunoglobulin therapy in many autoimmune diseases, including systemic lupus erythematosus. CASE REPORTS: Here, we report on two cases of lupus myocarditis treated with high dose of intravenous immunoglobulin. The first patient was a 42-year-old woman who presented with lupus myocarditis that was resistant to corticosteroids and cyclophosphamide, and who was finally successfully treated with a single dose of 2 g/kg of intravenous immunoglobulin. The patient displayed clinical improvement a few days later. The second case - a 43-year-old woman was diagnosed with lupus myocarditis and immunosuppressive drugs were contraindicated because of the context of a recent infective endocarditis. She was treated with repeated dose of 2 g/kg of intravenous immunoglobulin. Clinical improvement was observed and the left ventricular ejection fraction increased from 20 % to 60 % within a few days. We also report 9 similar observations identified from a literature review. CONCLUSION: The use of intravenous immunoglobulin in lupus myocarditis is not officially recognized but could be considered as an alternative when conventional therapies have failed or are contraindicated.

[Opportunistic infections and sarcoidosis]. / Les infections opportunistes au cours de la sarcoïdose.

Opportunistic infections (OI) are uncommon in sarcoidosis (1 to 10%) and mostly occur in patients with previously diagnosed disease or can rarely be the presenting manifestation. The most common OIs are, in descending order: aspergillosis, cryptococcosis, and mycobacterial infections. Treatment with corticosteroids is the most frequent risk factor for OI occurrence during sarcoidosis but immunosuppressive drugs and therapy with anti-TNFα are also risk factors. Overall, clinical presentation, treatment, and outcome are identical to that occur in other conditions complicated with the occurrence of OIs. However, some atypical presentations of OIs can mimic sarcoidosis exacerbation and misdiagnosis may lead clinicians to increase immunosuppression, causing worsening of the OI. The meticulous collection of patient's history along with factors differentiating OI from sarcoidosis exacerbation is key factor to optimally manage these patients.

[Carcinoma of unknown primary site]. / Les carcinomes de primitif inconnu.

PURPOSE: Carcinoma of unknown primary site is a common clinical syndrome, accounting for 2% of cancer patients. Diagnosis is a recurrent challenge for internists. Treatment is difficult and prognosis is still poor. This review presents one synthesis of diagnosis strategies and therapeutic trials. It envisages the interest of new molecular biology methods as well as therapeutic perspectives. CURRENT KNOWLEDGE AND KEY POINTS: Pathologic examination completed with immunohistochemical tests, and, depending on cases, with electron microscopy, cytogenetics, and molecular biology is a key-point for diagnosis. Diagnosis work-up, based on histological type and on individualization of some clinical presentation, proceeds in three steps. Positron emission tomography is recommended when a curative treatment is planed, particularly in cases of isolated metastasis. Functional status analysed using the performance status and simple biologic parameters (serum lactate dehydrogenase, serum alkaline phosphatase) permit us to assess prognosis. Chemotherapy is offered for patients with a good general health status. FUTURE PROSPECTS AND PROJECTS: Further evaluation of positron emission tomography, as well as cost-benefit analyses, is warranted. Further randomised trials are necessary to determine the optimal chemotherapy regimen in good-risk patients and the interest of chemotherapy in patients with poor-risk disease. Gene expression profiling and proteomic evaluation, as well as pharmacogenomic offer new investigation fields.

[Interest of an internist's consultation in uveitis. Comparative study in 66 cases]. / Apport de la consultation interniste dans le diagnostic étiologique des uvéites. Etude comparative portant sur 66 patients.

BACKGROUND: After exclusion of a masquerade syndrome, uveitis may be associated with infection, systemic diseases, specific ocular diseases, or may be drug-induced. In order to improve diagnostic strategy in uveitis, we performed a comparative study to assess the importance of internist and ophtalmologist's collaboration and we proposed a well-adapted diagnostic procedure. MATERIAL AND METHODS: A comparative study was performed in a tertiary centre. The population was divided in two groups. The first one was retrospective and consisted of patients treated at the Ophthalmology department of Croix Rousse Hospital from 1991 to 2002 without internist's collaboration. The second one was prospective and consisted of patients referred in the same centre in 2003 and 2004 with intervention of an internist. Patients of less than 18 years of age with pre-existent diagnosis, specific ocular diseases, toxoplasmosis infection, or with human immunodeficiency virus infection were excluded. RESULTS: Sixty-six patients were included. Anterior uveitis was the most frequent form (25 cases), followed by panuveitis (20 cases), posterior uveitis (14) and intermediate uveitis (7). The most frequent cause of uveitis was systemic disease (19 cases) followed by infection (7 cases) and neurological entities (4 cases). The rate of diagnosis was 30.3% without internist's intervention and 60.6% when patients were referred to an internist (P=0,01). The internist intervention was contributive in 75% of diagnoses. CONCLUSIONS: The internist intervention significantly enhances the rate of etiological diagnoses in uveitis. These results should further strengthen the internist/ophthalmologist collaboration for patients with uveitis.

[Aspergillosis in systemic diseases treated with steroids and/or immunosuppressive drugs: report of 9 cases and review of the literature]. / Aspergilloses au cours de maladies systémiques traitées par corticoïdes et/ou immunosuppresseurs: analyse de neuf cas et revue de la littérature.

This is a multicentric retrospective study of aspergillosis in patients treated by corticosteroids and/or immunosuppressive drugs for systemic diseases and a review of the literature. Nine patients, 5 men and 4 women, mean age of 62.8 years old were included among which Horton's diseases (3 cases), systemic lupus erythematosus (2), polymyositis (1), microscopic polyangiitis (1), idiopathic thrombocytopenic purpura (1), rheumatoid polyarthritis (1). Aspergillosis occurred in average 28.4 month after the diagnosis of systemic disease, and 28 months after the beginning of its treatment: corticosteroids in all cases, at a dose of 50.8 mg/day (equivalent prednisone) in average, cyclophosphamide (2 cases), methotrexate (1), intravenous immunoglobulins (1), leflunomide (1). All cases were invasive or chronic pulmonary aspergillosis located in the lungs (6 cases), or in the brain (3). Revealing symptoms were mild and non specific. Lymphopenia was severe in most cases, in average 472 lymphocytes/mm3 and 283 CD4+/mm3. The diagnosis was confirmed 20.75 days after the first symptoms in invasive aspergillosis, and 18.5 months in the chronic pulmonary cases, by cultures in 7 cases (broncho-alveolar lavage: 4; cerebral biopsy: 3), and direct microscopy examination of broncho-alveolar lavage in 2 cases. Specific serology was positive in 4 cases. Patients were treated by voriconazole (4 cases), itraconazole (2), amphotericin B (1), association of caspofungin and voriconazole (1), successive voriconazole and itraconazole (1). Six patients recovered from aspergillosis with 10.8 months of following time, 3 patients died a few days after confirmation of the diagnosis. Fifty-four cases of the literature are analysed.

[Lemierre syndrome variant: Hepatic abscesses and hepatic venous thrombosis due to Fusobacterium nucleatum septicemia]. / Un variant du syndrome de Lemierre: abcès hépatiques et thrombose veineuse sus-hépatique secondaire à une septicémie à Fusobacterium nucleatum.

INTRODUCTION: Like Fusobacterium necrophorum, Fusobacterium nucleatum is capable causing Lemierre's syndrome. Various locations of venous thrombosis have been described associated with Fusobacterium sp. septicemia. EXEGESIS: We describe a 43-year old alcoholic patient with F.nucleatum septicemia complicated with hepatic abscesses, middle hepatic venous thrombosis, osteomyelitis and infiltrative pneumonia. A pancreatic prosthesis was the only potentially identified infectious entrance. CONCLUSION: Our patient showed an alternative presentation of Lemierre's syndrome, a "digestive variant". To the best of our knowledge, this is the first report of Fusobacterium septicemia associated with hepatic venous thrombosis. This report is close to the cases of portal thrombosis and opens the clinical sphere of the lemierre's syndrome, whose incidence is increasing.

[Clinical efficacy of eculizumab as treatment of gemcitabine-induced thrombotic microangiopathy: A case report]. / Efficacité de l'éculizumab comme traitement de la microangiopathie thrombotique induite par la gemcitabine : à propos d'un cas.

INTRODUCTION: Gemcitabine-induced thrombotic microangiopathy is a rare event whose management is not yet consensual. The use of eculizumab could be of interest. CASE REPORT: A 68-year-old woman was treated by gemcitabine as adjuvant chemotherapy of a pancreatic adenocarcinoma. Two months later, the patient presented with mechanical hemolytic anemia, thrombocytopenia and high blood pressure that led to the diagnosis of thrombotic microangiopathy. Gemcitabine was stopped. Plasma exchange therapy was introduced since hematological and renal parameters had worsened. As clinical efficacy was insufficient, eculizumab was introduced at a dose of 900 mg per week 4 times, then 1200 mg every 2 weeks. Symptoms along with hematological and nephrological analysis were back to physiological standards after 7 intravenous injections. CONCLUSION: Eculizumab seems to be an effective treatment against gemcitabine-induced thrombotic microangiopathy in case of severe hematological and renal injuries associated with a lack of response to plasma exchange therapy.

Thymoma associated with autoimmune diseases: 85 cases and literature review.

OBJECTIVES: To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma. DESIGN: Pathological records from three university hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD. RESULTS: 47/85 (55%) cases of thymoma had AD, including myasthenia gravis (MG) (n=33), Hashimoto's thyroiditis (n=4), Isaac's syndrome (n=3), Morvan syndrome (n=2), pure red cell aplasia (n=2), systemic lupus (n=2), lichen planus (n=2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good's syndrome, pemphigus, autoimmune hepatitis, Graves' disease, limbic encephalitis, and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median duration of follow-up after surgery was 60 months (40-78 months). In 32 patients, the diagnosis of AD preceded the diagnosis of thymoma, in 9 patients, thymoma was diagnosed at the same time as the AD and 7 patients had been operated on when they developed an AD. We found a significative difference on the Masaoka stage between the MG patients and the patients who present another AD (p=0.028). No risk factor for developing an AD after thymectomy was identified. CONCLUSIONS: We describe here the long-term follow-up of a large series of AD related to thymoma. Our results confirm previous data concerning AD occurrence in patients with thymoma and suggest that preexisting autoimmunity is not a risk factor for developing autoimmune manifestations after thymectomy.

[Common variable immunodeficiency with autoimmune manifestations: study of nine cases; interest of a peripheral B-cell compartment analysis in seven patients]. / Déficit immunitaire commun variable avec manifestations auto-immunes: étude de neuf observations; intérêt d'un immunophénotypage spécifique des lymphocytes B circulants chez sept patients.

PURPOSE: Autoimmune manifestations (AIM) are associated to common variable immunodeficiency (CVI) in about 20 to 25% of the cases. This study presents the clinical, biological characteristics and the evolution of nine patients developing CVI and AIM. A peripheral B-cell compartment analysis has been performed in seven cases. METHOD: This multicenter retrospective study analyses nine patients, six men and three women, within a population of 32 CVI. RESULTS: The mean age was 27 years at the time of diagnosis of AIM and 30 years at the time of diagnosis of CVI. The diagnosis of AIM preceded the diagnosis of CVI in five cases. Thirteen AIM of different types were observed: autoimmune hemolytic anemia (AHA, 3), immune thrombocytopenic purpura (ITP, 2), Evan's syndrome (2), primary biliary cirrhosis (1), rheumatoid arthritis (1), alopecia totalis (1), myasthenia gravis (1). The peripheral B-cell compartment was investigated in seven patients: five patients with autoimmune cytopenia presented with a diminution of memory B cells (CD27+IgD-) and immature B cells (CD21-) levels; the patient with primary biliary cirrhosis and myasthenia gravis had only a diminution of memory B cells level; the last patient with ITP presented with a normal level of memory B cells. Five among the seven patients with autoimmune cytopenia required a specific treatment using corticosteroids, high dosages of intravenous immunoglobulin, then splenectomy after failure of the medical management, with severe infectious complications in one case. CONCLUSION: The association of AIM and CVI is not fortuitous. The most common AIM is autoimmune cytopenia. The peripheral B-cell compartment analyses show that a majority of patients have a defect in memory B-cells. Treatment regimens are not standardized and splenectomy increases the risk of infectious complications.

Assessment of glycosylated hemoglobin measurement with sample collection papers.

It would be advantageous to be able to measure glycosylated hemoglobin (GHb) from a sample of blood dried on paper and sent to the physician by the patient. Indeed, such a technique has been introduced by Isolab (Akron, OH). We tested the validity of the method to determine whether applying the blood to paper and immediately assaying it correlates with the same sample assayed by the usual whole-blood technique, the size of sample applied to paper affects apparent GHb results, time and temperature of storage of the sample paper affect apparent GHb results, and plasma glucose concentration of the sample affects GHb results. The GHb of samples assayed immediately after application to sample paper versus those assayed as whole blood showed very good correlation (r = .93, P less than .001). Volume of the drop of blood applied to paper (25-100 microliters) did not affect results. However, there was a dramatic, temperature-dependent increase in apparent GHb when samples were stored on sample paper, averaging 12.5, 16.3, and 19.5% when stored at room temperature for 3, 6, and 9 days, respectively. Overall, apparent GHb rose from 1.3-fold in 3 days at 4 degrees C to 3.8-fold in 9 days at 37 degrees C. The rate of GHb formation was proportional to plasma glucose concentration, but removal of free glucose by ethanol or glucose oxidase did not yield consistent results for this method. We conclude that these sample papers are not useful as an approach to collecting blood samples for GHb measurement.

Feasibility of intraperitoneal insulin therapy with programmable implantable pumps in IDDM. A multicenter study. The EVADIAC Study Group. Evaluation dans le Diabète du Traitement par Implants Actifs.

OBJECTIVE: To report the overall French experience, obtained through the collaboration of seven centers (EVADIAC [Evaluation dans le Diabète du Traitement par Implants Actifs] register), on the safety, feasibility, and efficacy of intraperitoneal insulin therapy by programmable implantable pumps, using three different devices. RESEARCH DESIGN AND METHODS: This is a multicenter prospective study involving 224 type I diabetic patients implanted with a programmable implantable pump (cumulative follow-up: 353 patient-years; mean duration: 1.5 +/- 0.9 years [mean +/- SD]. The Infusaid and the Promedos devices are equipped with a side port and refilled with U100 insulin (Hoechst 21 PH); the Minimed pump is not equipped with a side port and is refilled with U400 insulin (Hoechst 21 PH). Metabolic data and adverse events were recorded in a central register run by EVADIAC. RESULTS: A total of 29 local pump-pocket events (8/100 patient-years) and 9 pump failures (2.5/100 patient-years) occurred. The major technical problems were 1) pump flow rate reduction related to insulin aggregates, reversible after alkaline rinsing of the pump, and 2) 47 catheter obstructions requiring laparoscopic or conventional surgery. Pump therapy was abandoned in only 11 patients. HbA1c (7.4 +/- 1.8 vs. 6.8 +/- 1.0%, P < 0.001), mean glycemia (8.7 +/- 1.5 vs. 7.8 +/- 1.0 mmol/l, P < 0.001), and blood glucose SDs (3.8 +/- 0.8 vs. 3.3 +/- 0.8 mol/l, P < 0.001) decreased significantly after 6 months and remained lower than baseline thereafter. CONCLUSIONS: Intraperitoneal insulin infusion using an implantable programmable pump is a feasible and relatively safe technique that may improve metabolic control and glycemic stability. Long-term studies, however, are needed to demonstrate whether or not the improvement in glycemic control could be sustained for several years.

[Pathophysiology, subtypes, and treatments of adult-onset Still's disease: An update]. / Données actualisées sur la physiopathologie, les phénotypes et les traitements de la maladie de Still de l'adulte.

Adult-onset Still's disease is a rare and difficult to diagnose multisystemic disorder considered as a multigenic autoinflammatory syndrome. Its immunopathogenesis seems to be at the crossroads between inflammasomopathies and hemophagocytic lymphohistiocytosis, the most severe manifestation of the disease. According to recent insights in the pathophysiology and thanks to cohort studies and therapeutic trials, two phenotypes of adult-onset Still's disease may be distinguished: a systemic pattern, initially highly symptomatic and with a higher risk to exhibit life-threatening complications such as reactive hemophagocytic lymphohistiocytosis, where interleukin-1 blockade seems to be very effective, a chronic articular pattern, more indolent with arthritis in the foreground and less severe systemic manifestations, which would threat functional outcome and where interleukin-6 blockade seems to be more effective. This review focuses on these data.