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Anti-cholesterol antibody levels in hereditary angioedema.

Varga L, Bíró A, Széplaki G, Tóth L, Horváth A, Füst G, Farkas H - J. Cell. Mol. Med. (2007 Nov-Dec)

Bottom Line: Serum ACHA levels were significantly higher in HAE patients, compared to healthy blood donors (P<0.0001).Longterm danazol prophylaxis had no effect on serum ACHA levels in HAE patients.However, we found a significant, negative correlation between ACHA levels and serum total cholesterol (r=-0.4033, P=0.0200), LDL (r=-0.4565, P=0.0076) and triglyceride (r=-0.4230, P=0.0121) levels only in danazol-treated patients, but not in HAE patients who did not receive long-term prophylaxis.

View Article: PubMed Central - PubMed

Affiliation: Third Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary.

ABSTRACT
Hereditary angioedema (HAE) is a rare disorder caused by the deficiency of the C1-inhibitor gene (C1INH) and characterized by recurrent bouts of angioedema. Autoimmune disorders frequently occur in HAE. Previously we found, that danazol has an adverse effect on serum lipid profile: reduced high-density lipoprotein (HDL) and elevated low-density lipoprotein (LDL) cholesterol levels are associated with long-term prophylactic use, whereas total cholesterol levels are unchanged. Our aim was to study the anti-cholesterol antibody (ACHA) production in HAE patients and compare it with those of healthy blood donors, and to investigate the possible associations between ACHA levels and serum lipid profile alterations caused by danazol. Anti-cholesterol IgG levels were measured by ELISA and their correlation with serum concentrations of total cholesterol, HDL, LDL, triglycerides was determined in HAE patients receiving/not receiving danazol. Serum ACHA levels were significantly higher in HAE patients, compared to healthy blood donors (P<0.0001). Longterm danazol prophylaxis had no effect on serum ACHA levels in HAE patients. However, we found a significant, negative correlation between ACHA levels and serum total cholesterol (r=-0.4033, P=0.0200), LDL (r=-0.4565, P=0.0076) and triglyceride (r=-0.4230, P=0.0121) levels only in danazol-treated patients, but not in HAE patients who did not receive long-term prophylaxis. Patients with HAE have higher baseline ACHA levels compared to healthy subjects, and this might reflect polyclonal B-cell activation. The latter would be a potential explanation for the lack of an increased incidence of infectious diseases in HAE patients, but might lead to increased autoimmunity.

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ACHA titres in HAE patients and healthy controls. Serum ACHA titres were significantly higher in patients with HAE, than in healthy controls. Mann–Whitney's non-parametric test.
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fig01: ACHA titres in HAE patients and healthy controls. Serum ACHA titres were significantly higher in patients with HAE, than in healthy controls. Mann–Whitney's non-parametric test.

Mentions: Serum ACHA levels of HAE patients were compared to those of healthy controls (Fig. 1). As the two study groups did not differ by age or gender, univariate analysis with Mann–Whitney's non-parametric test was performed. We found that HAE patients had significantly higher ACHA levels compared to healthy controls (54.41 [37.96–72.79]versus 25.99 [18.98–40.39] AU/ml; P < 0.0001). The frequency of high ACHA levels (defined as ACHA levels exceeding the 90th percentile of controls) was significantly higher in HAE patients: 10/59 HAE patients compared to only 2/66 healthy controls had high ACHA levels (P = 0.0084, chi-square test).


Anti-cholesterol antibody levels in hereditary angioedema.

Varga L, Bíró A, Széplaki G, Tóth L, Horváth A, Füst G, Farkas H - J. Cell. Mol. Med. (2007 Nov-Dec)

ACHA titres in HAE patients and healthy controls. Serum ACHA titres were significantly higher in patients with HAE, than in healthy controls. Mann–Whitney's non-parametric test.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4401298&req=5

fig01: ACHA titres in HAE patients and healthy controls. Serum ACHA titres were significantly higher in patients with HAE, than in healthy controls. Mann–Whitney's non-parametric test.
Mentions: Serum ACHA levels of HAE patients were compared to those of healthy controls (Fig. 1). As the two study groups did not differ by age or gender, univariate analysis with Mann–Whitney's non-parametric test was performed. We found that HAE patients had significantly higher ACHA levels compared to healthy controls (54.41 [37.96–72.79]versus 25.99 [18.98–40.39] AU/ml; P < 0.0001). The frequency of high ACHA levels (defined as ACHA levels exceeding the 90th percentile of controls) was significantly higher in HAE patients: 10/59 HAE patients compared to only 2/66 healthy controls had high ACHA levels (P = 0.0084, chi-square test).

Bottom Line: Serum ACHA levels were significantly higher in HAE patients, compared to healthy blood donors (P<0.0001).Longterm danazol prophylaxis had no effect on serum ACHA levels in HAE patients.However, we found a significant, negative correlation between ACHA levels and serum total cholesterol (r=-0.4033, P=0.0200), LDL (r=-0.4565, P=0.0076) and triglyceride (r=-0.4230, P=0.0121) levels only in danazol-treated patients, but not in HAE patients who did not receive long-term prophylaxis.

View Article: PubMed Central - PubMed

Affiliation: Third Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary.

ABSTRACT
Hereditary angioedema (HAE) is a rare disorder caused by the deficiency of the C1-inhibitor gene (C1INH) and characterized by recurrent bouts of angioedema. Autoimmune disorders frequently occur in HAE. Previously we found, that danazol has an adverse effect on serum lipid profile: reduced high-density lipoprotein (HDL) and elevated low-density lipoprotein (LDL) cholesterol levels are associated with long-term prophylactic use, whereas total cholesterol levels are unchanged. Our aim was to study the anti-cholesterol antibody (ACHA) production in HAE patients and compare it with those of healthy blood donors, and to investigate the possible associations between ACHA levels and serum lipid profile alterations caused by danazol. Anti-cholesterol IgG levels were measured by ELISA and their correlation with serum concentrations of total cholesterol, HDL, LDL, triglycerides was determined in HAE patients receiving/not receiving danazol. Serum ACHA levels were significantly higher in HAE patients, compared to healthy blood donors (P<0.0001). Longterm danazol prophylaxis had no effect on serum ACHA levels in HAE patients. However, we found a significant, negative correlation between ACHA levels and serum total cholesterol (r=-0.4033, P=0.0200), LDL (r=-0.4565, P=0.0076) and triglyceride (r=-0.4230, P=0.0121) levels only in danazol-treated patients, but not in HAE patients who did not receive long-term prophylaxis. Patients with HAE have higher baseline ACHA levels compared to healthy subjects, and this might reflect polyclonal B-cell activation. The latter would be a potential explanation for the lack of an increased incidence of infectious diseases in HAE patients, but might lead to increased autoimmunity.

Show MeSH
Related in: MedlinePlus