C1-inhibitor autoantibodies in SLE

The presence of anti-C1-inhibitor (anti-C1-INH) autoantibodies is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence, diagnostic value, and/or significance in systemic lupus erythematosus (SLE). In a multicentre study, we determined the levels...

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Bibliographic Details
Published in:Lupus 2010-04, Vol.19 (5), p.634-638
Main Authors: Mészáros, T., Füst, G., Farkas, H., Jakab, L., Temesszentandrási, G., Nagy, G., Kiss, E., Gergely, P., Zeher, M., Griger, Z., Czirják, L., Hóbor, R., Haris, A., Polner, K., Varga, L.
Format: Article
Language:English
Subjects:
Adult
Antibodies
Autoantibodies - blood
Complement C1 Inhibitor Protein - immunology
Disease
Female
Humans
Immunology
Internal medicine
Laboratories
Lupus
Lupus Erythematosus, Systemic - blood
Male
Middle Aged
Proteins
Rheumatology
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Summary:The presence of anti-C1-inhibitor (anti-C1-INH) autoantibodies is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence, diagnostic value, and/or significance in systemic lupus erythematosus (SLE). In a multicentre study, we determined the levels of autoantibodies to C1-inhibitor in sera from 202 patients with SLE and 134 healthy controls. Additional clinical and laboratory parameters, such as organ involvement, as well as anti-C1q, anti-double-stranded DNA antibody, erythrocyte sedimentation rate, C-reactive protein, C3 and C4 serum complement levels have been studied in patients. The level of anti-C1-INH IgG was significantly higher (p = 0.034) in SLE patients, than in the controls. A high anti-C1-INH level of ≥0.4 U/ml (mean of controls + 2 SD) was found in 17% of the patients, but in only 4% of the controls (p = 0.0003). The SLEDAI score was significantly higher (p = 0.048) and the duration of SLE was significantly longer (p = 0.0004) among patients with elevated anti-C1-INH levels compared with patients without this autoantibody (median disease duration 8 vs. 17 years, respectively). Anti-C1-INH level was not correlated with any other laboratory parameter or organ manifestation of the disease. These findings indicate that the anti-C1-INH level is higher in SLE patients than in healthy controls and furthermore, the anti-C1-INH level correlates with the duration and activity of the disease. Lupus (2010) 19, 634—638.
ISSN:0961-2033
1477-0962
DOI:10.1177/0961203309357059