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Abstract

80ACA = anti-centromere antibodies; aCL = anticardiolipin antibodies; AFA = antifibrillarin/anti-U3-RNP; ANA = anti-nuclear antibodies; ANCA = anti-neutrophil cytoplasmic antibodies; ANoA = anti-nucleolar antibodies; anti-RNAP = anti-RNA-polymerase antibodies; anti-Sm = anti-Smith antibodies; aPL = antiphospholipid antibodies; β 2 gp I = β 2 glycoprotein I antibodies; CENP = centromeric nucleoprotein; CIE = counterimmunoelectrophoresis; CREST = a variant of SSc defined by the presence of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangectasia; CTD = connective tissue diseases; dcSSc = diffuse cutaneous systemic sclerosis; DL CO = diffusion capacity for carbon monoxide; DM = dermatomyositis; ELISA = enzyme-linked immunosorbent assay; FVC = forced vital capacity; HLA = human leukocyte antigen; IB = immunoblotting; ID = immunodiffusion; IIF = indirect immunofluorescence; IP = immunoprecipitation; lcSSc = limited cutaneous systemic sclerosis; MCTD = mixed connective tissue disease; PFT = pulmonary function tests; PM = polymyositis; PM/SSc = myositis/scleroderma overlap; RLD = restrictive lung disease; RNP = ribonucleoprotein; SLE = systemic lupus erythematosus; snRNP = small nuclear RNP; SSc = systemic sclerosis. Arthritis Research & Therapy Vol 5 No 2 Ho and Reveille IntroductionSystemic sclerosis (scleroderma or SSc) is a heterogeneous disorder characterized by autoantibody subsets, which in turn have their own clinical associations. Much controversy resides in whether these autoantibodies contribute directly to the pathology seen in SSc or whether they are merely epiphenomena of the underlying disease process. Nevertheless, various autoantibodies found in patients with SSc carry significant value in diagnosis and in predicting clinical outcomes (Fig. 1). The autoantibodies classically associated with SSc include anti-centromere antibodies (ACA) and anti-Scl-70 (otherwise known as antitopoisomerase I or anti-topo I). In addition to these is the less commonly occurring anti-nucleolar antibody (ANoA) system, which comprises a mutually exclusive heterogeneous group of autoantibodies that produce nucleolar stain- AbstractScleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70 antibodies are very useful in distinguishing patients with systemic sclerosis (SSc) from healthy controls, from patients with other connective tissue disease, and from unaffected family members. Whereas ACA often predict a limited skin involvement and the absence of pulmonary involvement, the presence of anti-Scl-70 antibodies increases the risk for diffuse skin involvement and scleroderma lung disease. Anti-fibrillarin autoantibodies (which share significant serologic overlap with anti-U3-ribonucleoprotein antibodies) and anti-RNA-polymerase autoantibodies occur less frequently and are also predictive of diffuse skin involvement and systemic dise...

Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)

“…ATA is highly specific for SSc (90–100%), and is associated with diffuse cutaneous SSc (although still observed in limited forms), poor prognosis, and higher risk of pulmonary fibrosis [54,55,56]. Sensitivity and specificity for predicting radiographic ILD in SSc is approximately 45% and 81% respectively [57].…”

Treatment of rapidly progressive systemic sclerosis: current and futures perspectives

“…Most circulating autoantibodies have not been shown to influence directly SSc pathogenesis and have been considered to represent autoimmune epiphenomena useful as disease diagnostic and clinical subset biomarkers, although certain autoantibodies correlate with specific clinical SSc patterns. For example, the presence of anti-RNA polymerase III antibodies indicates a higher risk of scleroderma renal crisis, whereas anti-centromere antibodies (ACA) are associated with limited skin involvement, and anti-topoisomerase I (Scl-70) antibodies are associated with increased risk of diffuse skin involvement and SSc-associated ILD [68–70]. The association of various circulating autoantibodies with SSc clinical subsets and certain clinical features are shown in Table 1.…”

Synthetic Peptides: The Future of Patient Management in Systemic Rheumatic Diseases?

“…Anti-topoisomerase-I (topo-I) autoantibodies are found in 20-40% of patients with systemic sclerosis (SSc) and are directed to a chromatin associated non-histone 110 kD protein, identified as topo-I [71]. These antibodies were initially referred to as Scl-70 based on reactivity to what has turned out to be a 70 kD degradation product of the native 110 kD protein [72].…”