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Anti-fibroblast antibodies from systemic sclerosis patients bind to -enolase and are associated with interstitial lung disease

Posted Nov 27 2009 10:03pm
By Benjamin Terrier and Colleague


To identify target antigens of anti-fibroblasts antibodies (AFAs) in systemic sclerosis (SSc) patients.

Patients and methods:

In the first part, sera from 24 SSc patients (12 with pulmonary arterial hypertension [PAH] and 12 without) and 36 idiopathic PAH (IPAH) patients, tested in pooled sera for groups of 3, were compared to a sera pool from 14 healthy controls (HCs). Serum IgG reactivity was analyzed by use of a 2-D electrophoresis and immunoblotting technique with normal human fibroblasts antigens.

In the second part, serum IgG reactivity for 2 groups - 158 SSc, 67 IPAH and 100 HCs; and 35 SSc and 50 HCs - was tested against -enolase from Saccharomyces cerevisiae (Sc) and human recombinant (rHu) -enolase, respectively, on ELISA.


In the first part, we identified -enolase as a main target antigen of AFAs from SSc patients. In the second part, 37/158 (23%) SSc patients, 6/67 (9%) IPAH patients and 4/100 (4%) HCs (p <0.0001) had anti-Sc -enolase antibodies; 12/35 (34%) SSc patients and 3/50 (6%) HCs had anti-rHu -enolase antibodies (p=0.001).

In SSc, the presence of anti-Sc -enolase antibodies was associated with interstitial lung disease (ILD), decreased total lung capacity (73.2 vs. 89.7%, p=0.0001) and diffusion capacity for carbon monoxide (47.4 vs. 62.3%, p=0.0009)], and anti-topoisomerase 1 antibodies (46 vs. 21%, p=0.005) but not anti-centromere Abs (11 vs. 34%, p=0.006). Results were similar with rHu -enolase testing.


In SSc, AFAs recognize -enolase and are associated with ILD and anti-topoisomerase antibodies.
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