Anti-nuclear autoantibodies in systemic sclerosis : News and perspectives

Abstract

Systemic sclerosis is a connective tissue disorder characterized by microvascular damage and excessive fibrosis of the skin and internal organs. One hallmark of the immunological abnormalities in systemic sclerosis is the presence of anti-nuclear antibodies, which are detected in more than 90% of patients with systemic sclerosis. Anti-centromere antibodies, anti-DNA topoisomerase I antibodies, and anti-RNA polymerase III antibodies are the predominant anti-nuclear antibodies found in systemic sclerosis patients. Other systemic sclerosis-related anti-nuclear antibodies include those targeted against U3 ribonucleoprotein, Th/To, U11/U12 ribonucleoprotein, and eukaryotic initiation factor 2B. Anti-U1 ribonucleoprotein, anti-Ku antibodies, anti-PM-Scl, and anti-RuvBL1/2 antibodies are associated with systemic sclerosis overlap syndrome. Anti-human upstream binding factor, anti-Ro52/TRIM21, anti-B23, and anti-centriole antibodies do not have specificity to systemic sclerosis, but are sometimes detected in sera from patients with systemic sclerosis. Identification of each systemic sclerosis-related antibody is useful to diagnose and predict organ involvement, since the particular type of systemic sclerosis-related antibodies is often predictive of clinical features, severity, and prognosis. The clinical phenotypes are largely influenced by ethnicity. Currently, an immunoprecipitation assay is necessary to detect most systemic sclerosis-related antibodies; therefore, the establishment of an easy, reliable, and simple screening system is warranted.

Keywords: Systemic sclerosis; anti-nuclear antibodies; autoantibodies; clinical features.

Figure 1.

Indirect immunofluorescence patterns observed on HEp-2 cells stained with (a) anti-centromere antibody (Ab), (b) anti-topoisomerase I Ab, (c) anti-U3 RNP Ab, (d) anti-Th/To Ab, (e) anti-Ku Ab, (f) anti-RuvBL1/2 Ab, (g) anti-hUBF Ab, and (h) anti-centriole Ab sera (original magnification 400×).