RÉSUMÉ
A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal.
Sujet(s)
Angioedème/enzymologie , Protéines inhibitrices de la fraction C1 du complément/déficit , Vascularite leucocytoclasique cutanée/enzymologie , Adulte , Angioedème/traitement médicamenteux , Antipaludiques/usage thérapeutique , Chloroquine/usage thérapeutique , Protéines du système du complément , Femelle , Humains , Hydroxychloroquine/usage thérapeutique , Résultat thérapeutique , Vascularite leucocytoclasique cutanée/traitement médicamenteuxRÉSUMÉ
OBJECTIVES: The aim of the present study was to investigate the role of soluble adhesion molecules in the pathogenesis of multiple sclerosis (MS) and systemic lupus erythematosus (SLE) with demyelinating syndrome. METHODS: Paired cerebrospinal fluid (CSF) and serum samples were analysed by an ELISA method to determine the concentrations of sVCAM-1, sICAM-1 and sL-selectin. Intrathecal syntheses of the adhesion molecules were calculated. RESULTS: Elevated serum and CSF concentrations of sVCAM-1 were present in all patient groups. Intrathecal synthesis of sVCAM-1 was present in the relapsing-remitting and secondary progressive forms of MS. Intrathecal synthesis of sICAM-1 was observed in all clinical forms of MS. MS patients with progressive forms of the disease and SLE patients were characterised by intrathecal synthesis of sL-selectin. CONCLUSIONS: The data presented suggest that (1) blood-brain barrier damage can be assumed both in systemic disease and organ-specific disease (sVCAM-1), (2) clinical forms of MS differ from each other in respect to concentrations of adhesion molecules and (3) similar immunological events in the central nervous system of SLE patients with demyelinating syndrome and progressive forms of MS can be assumed (sL-selectin).
Sujet(s)
Maladies auto-immunes/physiopathologie , Barrière hémato-encéphalique , Protéines du liquide céphalorachidien/analyse , Molécule-1 d'adhérence intercellulaire/analyse , Sélectine L/analyse , Lupus érythémateux disséminé/physiopathologie , Sclérose en plaques/physiopathologie , Molécule-1 d'adhérence des cellules vasculaires/analyse , Adulte , Sujet âgé , Maladies auto-immunes/sang , Maladies auto-immunes/liquide cérébrospinal , Maladies auto-immunes/immunologie , Sédimentation du sang , Encéphale/anatomopathologie , Études de cohortes , Maladies démyélinisantes , Évolution de la maladie , Femelle , Humains , Molécule-1 d'adhérence intercellulaire/sang , Molécule-1 d'adhérence intercellulaire/liquide cérébrospinal , Sélectine L/sang , Sélectine L/liquide cérébrospinal , Lupus érythémateux disséminé/sang , Lupus érythémateux disséminé/liquide cérébrospinal , Lupus érythémateux disséminé/immunologie , Mâle , Adulte d'âge moyen , Sclérose en plaques/sang , Sclérose en plaques/liquide cérébrospinal , Sclérose en plaques/classification , Sclérose en plaques/immunologie , Études prospectives , Solubilité , Molécule-1 d'adhérence des cellules vasculaires/sang , Molécule-1 d'adhérence des cellules vasculaires/liquide cérébrospinalRÉSUMÉ
Soluble L-selectin (sL-selectin) concentrations were measured in paired samples of serum and cerebrospinal fluid by an ELISA method. Patients with several forms of multiple sclerosis (MS) and systemic lupus erythematosus with central nervous system involvement (SLE-CNS) were investigated. Elevated CSF sL-selectin concentrations were found in patients with SLE-CNS (7.62 +/- 3.31 ng/ml) and with relapsing-remitting form of MS (6.99 +/- 4.72 ng/ml) compared to the control group (4.00 +/- 0.95 ng/ml). The data presented suggest some similarities between inflammatory/immunological events in the central nervous system in patients with SLE-CNS and relapsing-remitting form of MS. Immunological heterogeneity in MS is suspected.
Sujet(s)
Encéphale/immunologie , Sélectine L/métabolisme , Vascularite lupique du système nerveux central/sang , Vascularite lupique du système nerveux central/liquide cérébrospinal , Sclérose en plaques récurrente-rémittente/sang , Sclérose en plaques récurrente-rémittente/liquide cérébrospinal , Adulte , Sujet âgé , Études cas-témoins , Test ELISA , Femelle , Humains , Sélectine L/sang , Sélectine L/liquide cérébrospinal , Vascularite lupique du système nerveux central/métabolisme , Mâle , Adulte d'âge moyen , Sclérose en plaques/sang , Sclérose en plaques/liquide cérébrospinal , Sclérose en plaques récurrente-rémittente/métabolisme , Statistique non paramétriqueRÉSUMÉ
The concentration of soluble vascular cell adhesion molecules (sVCAM-1) of serum and cerebrospinal fluid (CSF) was measured in clinically selected multiple sclerosis (MS) and systemic lupus erythematosus (SLE) patients, using an ELISA assay. The mean sVCAM-1 concentration in the serum of SLE patients was higher than normal. The mean CSF sVCAM-1 concentration was increased in the MS as in the SLE group. On analysis, the data suggests that there are some similarities in the immunological effects of these two different diseases of the central nervous system. A longitudinal analysis of the CSF is requested.
Sujet(s)
Lupus érythémateux disséminé/diagnostic , Sclérose en plaques/diagnostic , Molécule-1 d'adhérence des cellules vasculaires/liquide cérébrospinal , Adolescent , Adulte , Sujet âgé , Test ELISA , Femelle , Humains , Études longitudinales , Lupus érythémateux disséminé/immunologie , Mâle , Adulte d'âge moyen , Sclérose en plaques/immunologie , Sensibilité et spécificitéRÉSUMÉ
Studying the gene polymorphism of serum amyloid P a common constituent of amyloid deposits two restriction fragment length polymorphism patterns were found among Hungarian rheumatoid arthritis (RA), juvenile rheumatoid arthritis (JRA), systemic lupus erythematosus (SLE) patients and healthy controls. Although no direct connection between DNA polymorphism and the frequency of amyloidosis in three patient groups was found, a marked predominance of the heterozygosity in all studied groups has been described. Authors found close correlation between the serum levels of SAP and CRP in juvenile rheumatoid arthritis, but failed to find the same correlation in SLE and healthy controls. Moreover, the serum level of CRP of heterozygous patients were significantly higher than that of homozygous patients within the juvenile rheumatoid arthritis patient groups.
Sujet(s)
Amyloïde/sang , Arthrite juvénile/sang , Maladies auto-immunes/sang , Lupus érythémateux disséminé/sang , Arthrite juvénile/génétique , Arthrite juvénile/immunologie , Maladies auto-immunes/génétique , Maladies auto-immunes/immunologie , Protéine C-réactive/analyse , Hétérozygote , Homozygote , Humains , Lupus érythémateux disséminé/génétique , Lupus érythémateux disséminé/immunologie , Biologie moléculaire , Polymorphisme génétiqueRÉSUMÉ
(SLE) One hundred patients suffering from systemic lupus erythematosus (SLE) were examined by clinical, non invasive cardiological, radiological and laboratory methods. Valve diseases were revealed by M-mode echocardiographic examination in 17 patients. 14 patients had various conduction disturbances. Out of the remaining 83 patients not having any valve disease, systolic dysfunction of the myocardium was detectable in 17 persons and pericardial effusion in 3 persons. Spirometric alterations have been found in 64 persons and cor pulmonale has been diagnosed in 8 persons.
Sujet(s)
Cardiopathies/étiologie , Lupus érythémateux disséminé/complications , Coeur pulmonaire/étiologie , Échocardiographie , Cardiopathies/diagnostic , Valvulopathies/diagnostic , Valvulopathies/étiologie , Humains , Épanchement péricardique/diagnostic , Épanchement péricardique/étiologie , Coeur pulmonaire/diagnostic , SpirométrieRÉSUMÉ
Patients with Sjögren's syndrome were treated with vitamin A (100,000 U) daily during a two-week period. The vitamin treatment significantly elevated their ADCC and NK activity. The lymphocyte blast transformation, however was not noticeably changed. After the treatment, the retinyl-ester and retinol level of the plasma significantly increased as did the plasma level of vitamin E. The level of TBA reactive substances (malondialdehyde) in the plasma increased, whilst the glutathione peroxidase and catalase activity of erythrocytes decreased. The activity of the plasma glutathione peroxidase increased, but not significantly.