V. Veit

Igg4-related Systemic Disease: Features and Treatment Response in a French Cohort

AbstractIgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagnosis criteria. We report the clinical, laboratory, and histologic characteristics of 25 patients from a French nationwide cohort. We also report the treatment outcome and show that despite the efficacy of corticosteroids, a second-line treatment is frequently necessary. The clinical findings in our patients are not different from the results of previous reports from Eastern countries. Our laboratory and histologic findings, however, suggest, at least in some patients, a more broad polyclonal B cell activation than the skewed IgG4 switch previously reported. These observations strongly suggest the implication of a T-cell dependent B-cell polyclonal activation in IgG4-related systemic disease, probably at least in part under the control of T helper follicular cells.

Prospective investigation of a large outbreak of meningitis due to echovirus 30 during summer 2000 in marseilles, france.

Abstract: Enteroviruses (EVs) are responsible for an array of clinical diseases affecting different systems of the organism. Many cases are asymptomatic; the most severe clinical syndromes caused by EVs are due to infection of the central nervous system and present as aseptic meningitis or encephalitis. We report here a large outbreak of enteroviral meningitis that spread in Marseilles, France, during the year 2000. The dominant strain of the outbreak was genetically identified as a human echovirus 30. The study was conducted prospectively from May to December 2000, with an investigative protocol recording epidemiologic, clinical, and laboratory data. A total of 250 patients with febrile neurologic manifestations were included between May 15 and December 30, 2000. A total of 195 cerebrospinal fluid (CSF) samples, 114 throat swabs, and 85 stool specimens were processed through viral culture and resulted in respectively 117 (60%), 61 (54%), and 58 (68%) cultures positive for EV; 69/106 (65%) CSF samples tested positive for the presence of EV RNA. None of the throat swab cultures but 5 of the stool cultures in control patients were positive. One hundred thirty-nine (55.6%) patients were considered confirmed cases because they had positive culture or reverse transcription polymerase chain reaction (RT-PCR) in CSF, and 38 (15.2%) patients were considered probable cases because they had a positive throat and/or stool culture and a negative (or not performed) procedure in CSF. The 177 confirmed and probable cases were not significantly different from the remaining 73 patients in terms of age distribution and epidemiologic, clinical, and biologic characteristics. The median age was 18.4 years (range, 15 d to 84 yr), and 92% of patients were younger than 40 years old. The male:female sex ratio was 1.8:1. We found no evidence of cases spread in nosocomial, household, or institutional settings, or limited community spread. All patients were immunocompetent except 4 adults. Meningoencephalitis represented 5.6% of cases. All but 3 of the 177 patients had a good outcome without sequelae. Two immunocompetent adults with meningoencephalitis had neurologic sequelae and an immunosuppressed adult had a fatal outcome. Upper respiratory symptoms were noted in 18.5% of patients, diarrhea in 11.5%, various types of rash in 4.5%, and myalgia in 3.8%. In CSF, white cell count was elevated in 90% of cases, with a percentage of neutrophils >50% in 55% of cases. Protein level was increased in 43% of cases. In blood, C-reactive protein was elevated in 67% of cases. Other blood parameters were unremarkable. Clinical and laboratory features did not differ from those related to other pathogens that caused meningitis and meningoencephalitis. Hence, unnecessary treatment for other infections is frequently instituted during EV infections. Virologic diagnosis is important to distinguish between EV and other treatable bacterial and viral diseases. Abbreviations: CSF = cerebrospinal fluid; ECV-30 = Echovirus 30; EV = enterovirus.

Subcutaneous immunoglobulin administration: an alternative to intravenous infusion as adjuvant treatment for dermatomyositis?

Monthly high-dose intravenous administration of human polyclonal immunoglobulins (IVIG) has been shown to be effective as an adjuvant treatment for dermatomyositis. We report a patient with dermatomyositis treated with high doses of immunoglobulins by subcutaneous infusion (SCIG). SCIG was used because of the lack of peripheral and central vein access to continue effective IVIG therapy. The treatment was administered at home, was well tolerated, and was associated to the stabilization of the disease after a 1-year follow-up. Thus, our report suggests that SCIG could be an alternative to IVIG in the treatment of dermatomyositis.

Agglutinines froides, circonstances de découverte chez l’adulte et signification en pratique clinique : analyse rétrospective à propos de 58 patients

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.

Article originalAgglutinines froides, circonstances de découverte chez l’adulte et signification en pratique clinique : analyse rétrospective à propos de 58 patientsCold agglutinins, clinical presentation and significance: retrospective analysis of 58 patients

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.

Manifestations cardiaques au cours la granulomatose de Wegener: à propos de quatre observations et revue de la littérature

INTRODUCTION Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegeners 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 Wegeners granulomatosis and had a severe disease course. CONCLUSION Cardiac events in Wegeners 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 Wegeners granulomatosis.

Neuromyocardite aiguë du béribéri par régime alimentaire exclusif.Une observation

Introduction. – Thiamine deficiency can be determined by various clinical signs; some of these symptoms may be acute, and require an urgent diagnosis. In countries such as ours with a high standard of living, this disorder is more commonly observed in cases of severe alcoholism, and cases of diet-associated thiamine deficiency are rare, and therefore not easily recognized. The metabolic disorders resulting from vitamin B1 deficiency are responsible for the well-known central or peripheral neurological symptoms, and also for the less common and often more acute cardiovascular reactions. Immediate thiamine/vitamin B1 supplementation is of major importance. The rapid reversal of symptoms following this treatment is often considered as a diagnostic index. Exegesis. – In this study, an original case of diet-associated thiamine deficiency has been reported, with clinical symptoms including myocarditis and subacute peripheral nerve involvement in a young adult. The disorder was the result of a thiamine deficiency in the diet, which was exclusively based on milled rice. Conclusion. – The present report is interesting both as regards its clinical aspects and its etiology, and it emphasizes the importance in the differential diagnosis of a given case of taking the possibility of diet-related thiamine deficiency into account, although this is an uncommon etiology in developed countries.

Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association

AbstractThe association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported.We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients.Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series.This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse.

Syndrome d’encéphalopathie postérieure réversible induit par un décongestionnant nasal contenant de la pseudo-éphédrine

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.

Étude de l'association hépatite auto-immune et antiphospholipides

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.