D. Vital Durand

Apport de l'IRM cerebrale dans une serie de 58 cas de malade lupique avec ou sans manifestations neuropsychiatriques

PURPOSE Central nervous (CNS) involvement in SLE is common and can be evaluated with MRI. The primary goal of this study was to evaluate with high-field MRI the CNS involvement in a series of SLE patients with or without neuropsychiatric symptoms. The secondary goal was to detect a possible relationship between MRI and clinical or biological parameters in SLE. MATERIALS AND METHODS We correlated the clinical and biological parameters of 58 patients with a lupus defined according to the American College of Rheumatology criteria, including 30 with neuropsychiatric manifestations with conventional and modern MRI (including diffusion weighted-images, high-resolution 3D T1 weighted-images). The population studied was compared to a group of 18 normal controls. RESULTS In 69% of cases, MRI demonstrated involvement of the CNS both in asymptomatic patients (64.3%) and in patients with neuropsychiatric manifestations (73.3%): microembolic signals, cerebral infarctions (associated with the anti-phospholipid syndrome), atrophy, basal ganglia involvement, posterior leucoencephalopathy, subcortical calcification or hemosiderin deposits (T2*), dilated perivascular spaces. CONCLUSION MRI with adapted sequences clearly demonstrated the cerebral involvement in approximately 70% of SLE patients with or without neuropsychiatric symptoms.

Lupus érythémateux disséminé survenant après 65 ans

Resume Propos. – Le lupus erythemateux dissemine revele chez des sujets âges a ete peu etudie (une serie de 21 patients de plus de 65 ans publiee). La prise en charge de cette maladie est-elle modifiee dans cette population ? Methodes. – Dix-sept cas de lupus ayant debute apres 65 ans et hospitalises entre 1988 et 2000 sont rapportes retrospectivement. Les resultats sont compares a ceux de sujets plus jeunes. Resultats. – Le sex-ratio femme/homme est a 1,83. L’âge moyen lors de la survenue du premier signe est de 71,9 ± 3,5 ans. La duree moyenne de suivi est de 3,5 ± 2,4 ans. Les principaux signes initiaux sont une alteration de l’etat general (41 %), des manifestations articulaires (35 %), cutanees (35 %), thromboemboliques (24 %) et des pleuresies (18 %). Le rash malaire est exceptionnel (12 %). La nephropathie n’est jamais revelatrice et rarement severe au cours de l’evolution. Comme pour les atteintes neurologiques, l’etiologie doit etre discutee en tenant compte des comorbidites. Sur le plan hematologique, la lymphopenie (82 % des patients) a une specificite contestable. Les anticorps antinucleaires sont constants, avec des anti-ADN natifs dans 82 % des cas, des anticorps anti-antigenes nucleaires solubles dans 50 %, un anticoagulant circulant dans 59 %. Le pronostic est difficile a estimer sur une petite serie, mais la probabilite de survie a 5 ans est de 83 %. La corticotherapie a entraine 50 % de complications graves. Conclusions. – Cette etude permet surtout d’insister sur les manifestations revelatrices particulieres du lupus erythemateux dissemine chez le sujet âge (alteration de l’etat general, thromboses, signes cutanes atypiques), ainsi que sur les difficultes specifiques du diagnostic differentiel et du traitement.

Anticoagulants circulants en dehors de l'hémophilie: Enquête multicentrique conduite par le secrétariat à la recherche de la SNFMI à propos de 207 observations

A retrospective multicentre study, undertaken under the aegis of the French National Society of Internal Medicine, involved 200 subjects with acquired circulating anticoagulants; 130 were female and 77 were male; mean age was 45 +/- 23 years (range: 10 months to 80 years). Mean duration of follow-up was 23 months. In 130 subjects the anticoagulants were detected as a result of a systematic screening examination. The main overt clinical manifestations were haemorrhages, venous or arterial thrombosis and spontaneous abortion. Typing of the anticoagulant, performed in 166 cases, showed the presence of an antiprothrombinase in 141; this enzyme is not responsible for severe bleeding unless it is associated with other disorders of coagulation; less frequent were an anti-factor VIIIc (n = 16) and an anti-factor V (n = 2) anticoagulants. An underlying pathology was found in 172 subjects, including systemic lupus erythematosus (n = 60), induced lupus (n = 11), discoid lupus (n = 3), infection (n = 23), blood disease (n = 19), cancer (n = 15) and vasculitis (n = 15); other factors were pregnancy (n = 5) and medicines (n = 6). The anticoagulant disappeared spontaneously in 10 cases and in 33 of the 115 subjects treated. In subjects with lupus and in children under twelve years of age, an antiprothrombinase was regularly identified at typing.

Mise au pointÉlévation modérée, persistante et inexpliquée des transaminasesUnexplained, subclinical chronically elevated transaminases

Unexplained, subclinical chronically elevated transaminases is mainly a marker of non-alcoholic fatty liver disease, metabolic syndrome, alcoholism and diabetes, which are very common situations but viral hepatitis and iatrogenic origin must also be considered. Before looking for hepatic or genetic rare diseases, it is worth considering hypertransaminasemia as a clue for muscular disease, particularly in paediatric settings, and creatine phosphokinase is a specific marker. Then, patient history, examination and appropriate biologic requests can permit the identification of less frequent disorders where isolated hypertransaminasemia is possibly the unique marker of the disease for a long while: hemochromatosis, celiac disease, autoimmune hepatitis, Wilsons disease, α1-anti-trypsine deficiency, thyroid dysfunctions, Addisons disease. Liver biopsy should be performed only in patients with aspartate aminotransferases upper the normal range or alanine aminotransferases higher than twice the normal range after 6 months delay with dietetic corrections.

Élévation modérée, persistante et inexpliquée des transaminases

Unexplained, subclinical chronically elevated transaminases is mainly a marker of non-alcoholic fatty liver disease, metabolic syndrome, alcoholism and diabetes, which are very common situations but viral hepatitis and iatrogenic origin must also be considered. Before looking for hepatic or genetic rare diseases, it is worth considering hypertransaminasemia as a clue for muscular disease, particularly in paediatric settings, and creatine phosphokinase is a specific marker. Then, patient history, examination and appropriate biologic requests can permit the identification of less frequent disorders where isolated hypertransaminasemia is possibly the unique marker of the disease for a long while: hemochromatosis, celiac disease, autoimmune hepatitis, Wilsons disease, α1-anti-trypsine deficiency, thyroid dysfunctions, Addisons disease. Liver biopsy should be performed only in patients with aspartate aminotransferases upper the normal range or alanine aminotransferases higher than twice the normal range after 6 months delay with dietetic corrections.

Amyloses localisées de découverte forfuite: analyse de 12 observations

Authors report ten cases of localized amyloidosis exposed by surgery. Three patients have developped later a systemic amyloidosis. The others patients have a local disease (2 larynx, 1 heart valves, 1 urinary bladder, 1 sciatic nerve, 1 eyelid, 1 Fallopian tube) with an excellent pronostic.

La stérilité masculine par atrophie congénitale bilatérale des canaux déférents est-elle une forme clinique de mucoviscidose?

We investigated clinical data, sweat electrolytes and cystic fibrosis (CF) mutations in twelve patients with congenital bilateral aplasia of vasa deferentia (CBAVD) to debate arguments for diagnosing CF. Sweat chloride concentration was definitely raised in four patients. Three patients are CF compound heterozygotes. Six patients are CF heterozygotes. This result reinforces the hypothesis that white males with CBAVD might have a mild form of CE.

Abcès viscéraux aseptiques: une manifestation systémique inhabituelle inaugurant la maladie de Crohn (5 observations)

Aseptic abscesses affecting spleen and mesenteric lymph nodes are reported in five patients. The abscesses were diagnosed from one to three years ago before we discovered a Crohns disease. All the patients received corticosteroids successfully. A neutrophilic cellulary dysfunction is discussed.

La maladie de Whipple : analyse de 40 observations

This series of 40 case reports of Whipples disease (National Register of the SNFMI) confirms the difficulties of diagnosis, the necessity of a prolonged treatment and possible return of symptoms later. A prospective study would help to codify the therapy better, as well as the modalities of medical supervision which are still empirical.

Phénotype HLA-DR dans la polychondrite atrophiante: étude multicentrique de 53 cas

53 patients with relapsing polychondritis were studied for HLA-DR phenotype with a classicallymphocytotoxicity method. No significant association was found after comparison with HLA-DR frequencies in Caucasoid population.