Search

The presence of autoantibodies to C1-inhibitor (C1-INH-Abs) is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence in hereditary angioedema (HAE). In a prospective study performed between 2001 and 2004 in 95 patients with Type I or II HAE, serum samples were taken one to three times a year and clinical status of the patients was registered. Serum samples were tested for total activity of the classical pathway, C1q, C3, C4 and C1-inhibitor (C1-INH) concentration and activity levels, as well as the presence of IgG, IgA and IgM type anti-C1-inhibitor antibodies (C1-INH-Ab). Fifty-four healthy age and gender matched persons served as control. Significant differences between the patients and controls in the occurrence of elevated (2S.D. higher than mean of control) C1-INH-Abs titers was found only in the case of IgM type C1-INH-Abs. Elevated (>4.22AU/ml) IgM C1-INH-Abs levels were found in 31 and 4% of the patients and controls, respectively (p

BACKGROUND Serum beta2-microglobulin (beta2m) has been established as a marker of disease activity in malignancies, autoimmune conditions and infections. Despite its important role in prognosis assessment and disease monitoring, relatively few studies are available on its expression in healthy individuals. Furthermore, interpretation of results is hampered by the variety in reference limits due to differences in methodology, sample population and statistics. METHODS Serum beta2m concentrations were measured using a microparticle-enhanced immunonephelometric method in 183 healthy blood donors aged 29-75 years. RESULTS The median beta2m concentration was 1.67 (0.88-2.75) mg/L with no difference between men and women (1.71 mg/L vs. 1.62 mg/L, p

The presence of autoantibodies to C1-inhibitor (C1-INH-Abs) is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence in hereditary angioedema (HAE). In a prospective study performed between 2001 and 2004 in 95 patients with Type I or II HAE, serum samples were taken one to three times a year and clinical status of the patients was registered. Serum samples were tested for total activity of the classical pathway, C1q, C3, C4 and C1-inhibitor (C1-INH) concentration and activity levels, as well as the presence of IgG, IgA and IgM type anti-C1-inhibitor antibodies (C1-INH-Ab). Fifty-four healthy age and gender matched persons served as control. Significant differences between the patients and controls in the occurrence of elevated (2S.D. higher than mean of control) C1-INH-Abs titers was found only in the case of IgM type C1-INH-Abs. Elevated (4.22AU/ml) IgM C1-INH-Abs levels were found in 31 and 4% of the patients and controls, respectively (p0.001). Surprisingly, high titer IgM C1-INH-Abs were present with equal frequency in the 41 HAE patients ever treated with C1-INH concentrate and in the 54 C1-INH treatment naïve patients. In the latter group, strong positive correlation between the levels of the IgM C1-INH-Abs and the most severe disease (score 1) (p=0.0021) and the yearly attack rate (p=0.0173) were obtained. In addition, the levels of the IgM C1-INH-Abs exhibited strong negative correlation to the C1-inhibitor concentration and functional activity, total classical complement pathway activity, and a positive correlation to total IgM concentration. Taken together, these data indicate that IgM type C1-INH-Abs are present with highly elevated frequency in HAE patients irrespectively of the previous treatment with C1-INH concentrate. Most probable production of these autoantibodies is the consequence of the activation of complement and other plasma enzyme systems during HAE attacks. Determination of IgM C1-INH-Abs can be used as an activity marker in HAE.

Recurrent spontaneous abortion (RSA) is diagnosed if three or more spontaneous abortions follow each other typically in the first trimester. The root cause of miscarriages often can not be found. A significant proportion of this unexplained RSA cases may be caused by immunopathological failure.A multicentric clinical study started in 2000 to introduce an immunological screening protocol for patients suffering in idiopathic habitual abortion, and to use immunotherapy for their treatment if immunological background was defined.The general checkup of the patients was managed based upon a detailed protocol, with which non-immunopathological reasons for RSA were excluded. The unexplained RSA cases underwent an immunological checkup including cellular and humoral immunological, immunogenetic and autoimmune examinations. Based upon these parameters, the immunopathological background of RSA was certified or excluded. In the confirmed immunopathological cases intravenous immunoglobulin (IVIG) therapy was applied during their next pregnancy, with continuous monitoring of the immunological parameters.120 patients with RSA were examined, and 32 of them got IVIG therapy during their next pregnancy. In 72% of cases (23/32) IVIG treatment for RSA with immunopathological alloimmune background was successful, with the outcome of healthy newborn. Of the 9 unsuccessful cases, in 6 patients subsequently additional non-immunopathological reasons were diagnosed for their RSA. IVIG treatment of patients with clear alloimmune background was successful in 88.5% (23/26).Results show that immunopathological checkup and immunotherapy is a useful treatment in the modern medicine for the patients with unexplained RSA. However the success of this method depends on the adherence of the checkup protocol, because unsuccessful therapy of non-clear cases can reduce the efficiency.

3he antigen binding capacity and idiotypic specifity of isolated and reassociated chains of an IgG1 myeloma protein with anti-horse-alpha-2-macroglobulin activity (Fero) and of an IgM cryoglobulin with anti-IgG activity (Far), were investigated by precipitation and inhibition of precipitation methods. The binding capacity of heavy chains obtained from both proteins was about 10-fold higher than that of the light chains. The inhibitory capacity of recombinants composed of the autologous heavy chains of protein Fero was not significantly higher than that of the isolated heavy chains. Autologous light chians reassociated with heterolgous heavy chains were mnore effective in the inhibition test, than the isolated chains. Heterologous recombinants could not precipitae the antigen. The heavy chains of protein Far recombined with heterologous light chian showed a higher binding capacity than did the separated heavy chains. These recombinants had cyoprecipitation ability. Expression of idiotypic determinants of both proteins was more or less parallel with the antibody activity. The activity of H chains to form antibodies of similar specificity with series of L chains differing in V sequences is discusse.d