Background Provisional major criteria for the diagnosis of very early systemic sclerosis (VESSc) proposed of EULAR Scleroderma Trial and Research Group (EUSTAR) are Raynaud’s phenomenon (RP), positive autoantibodies (antinuclear, anticentromere-ACA, antitopoisomerase I- ATA) and scleroderma pattern of nailfold capillary changes (SPNCC)1. However, reports about prognostic value of this antibodies in diagnosis of VESSc are inconclusive.
Objectives To assess the prognostic value of SPNCC, ACA and ATA for the development of VESSc in patients (pts) with primary RP.
Methods From the group of 3029 pts with primary RP who were prospectively followed from 1996 to 20061, separated a group of 497 pts in whom were identified ACA and ATA by indirect imunofluorescence. Results ACA i ATA were categorized as positive or negative. Nailfold capillary changes were classified as normal, nonspecific and scleroderma pattern. Sensitivity (Sn), specificity (Sp), positive predictive value (PPV), negative predictive value (NPV),Odds ratio (OR) and area under the ROC curve (AU-ROC) of SPNCC, ACA i ATA testing for the diagnosis of VESSc was assesed.
Results At the end of follow up period 20.72% pts have still the primary RP, 13.3% had suspected secondary RP, 31,9% developed SSc, 15.3% undifferentiated CTD (connective tissue disease), 5.8% Sjögren’s syndrome, 4.6% overlap syndrome, 3.0% systemic lupus erythematosus, 1.8% rheumatoid arthritis, 1.8% mixed CTD, less of 1% pts developed vasculitides and polymyositis. The SPNCC had 95% pts with SSc, and 23% pts with other CTDs. SPNCC were significantly associated with future development of SSc (p=0.0000) with Sn 95%, Sp 77%, PPV 66%, NPV 97%, OR 63, AU-ROC 0.819. ACA were detected in 41% pts with SSc, and 34% pts with other CTDs. ACA were not significantly associated with future development of SSc (p=0.187) with Sn 41%, Sp 66%, PPV 36%, NPV 70%, OR 1.32, AU-ROC 0.538. ATA were detected in 36% pts with SSc, and 4% pts with other CTDs. ATA significantly associated with future development of SSc (p=0.0000) with Sn 36%, Sp 96%, PPV 81%, NPV 76%, OR 13.9, AU-ROC 0.783. Both parameters together SPNCC-ACA and SPNCC-ATA were significantly associated with future development of SSc (p=0,0001, Sn 40%, Sp 86%, PPV 58%, NPV 75%, OR 4.2, AU-ROC 0.674 v.s. P=0,00001, Sn 33%, Sp 97%, PPV 88%, NPV 76%, OR 24, AU-ROC 0.826 respectively).
Conclusions Scleroderma pattern of capillary abnormalities or/and ATA were good predictor of future development of SSc, and important test in pts with RP in order to identify those at higher risk of developing SSc. ACA in patients with RP are not consistently associated with SSc. However, both parameters together (SPNCC and ACA) sugest future development of SSc.
Pavlov-Dolijanovic S, Damjanov N, Stojanovic R, Vujasinovic Stupar N, Stanisavljevic D.Scleroderma pattern of nailfold capillary changes as predictivevalue for the development of a connective tissue disease:a follow-up study of 3,029 patients with primary Raynaud’sphenomenon. Rheumatol Int 2011 DOI 10.1007/s00296-011-2109-2.
Disclosure of Interest None Declared
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