FRI0313 DIFFERENTIAL PERFORMANCE OF NAILFOLD VIDEO CAPILLAROSCOPIC PARAMETERES IN THE DIAGNOSIS AND PROGNOSIS OF SYSTEMIC SCLEROSIS

Abstract

Background The role of Nailfold Video Capillaroscopy (NVC) in the identification of patients with Raynaud phenomenon (RP) at risk to develop systemic sclerosis (SSc) is well established. However, it is not clear if certain capillaroscopic indices perform better than others at predicting SSc development and which NVC parameters have a prognostic value in established SSc. Objectives To comparatively assess the performance of different NVC parameters in predicting development of SSc, very early diagnosis of SSc (VEDOSS), or mixed connective tissue disease (MCTD) in patients with RP. Also, to longitudinally examine the consistency of clinical correlations of NVC parameters in SSc patients at two different time points and evaluate the prognostic capacity of NVC in SSc. Methods Consecutive RP patients referred to our department for NVC (138 with SSc, 12 with VEDOSS, 6 with MCTD, 36 with primary RP, and 50 with non-SSc secondary RP) were evaluated at baseline, both clinically and capillaroscopically; 175 were reevaluated after a mean±SD of 3.34±1.48 years. Sixty-two healthy volunteers served as controls. Qualitative assessment of NVC images permitted categorization of patients to a normal, early, active or late capillaroscopic pattern. Capillary loss, dilated, giant or ramified capillaries and micro-hemorrhages were evaluated by a semi-quantitative score (0-3), derived as the mean of three 1mm fields in each of the 2nd, 3rd, 4th and 5th finger of both hands. FVC and DLCO were recorded, if performed within 6 months of NVC. FVC and DLCO deterioration were considered clinically significant if >10% and >15%, respectively. Skin thickening was measured using the modified Rodnan Skin Score (mRSS). MRSS deterioration was considered clinically significant if >3.5. First occurrence of digital ulcers in patients with no prior such history and vital status were also recorded at follow-up. Results Capillary loss score had the highest diagnostic accuracy at discriminating patients with an SSc-spectrum disorder from patients with RP of different etiology and from controls, as defined by ROC curve analysis [AUC (95% CI)=0.925 (0.893-0.956)], followed by dilatation score [AUC (95% CI)=0.904 (0.807- 0.938)] and giant score [AUC (95% CI)=0.856 (0.810-0.902)]. By contrast, micro-hemorrhages [AUC (95% CI)=0.727 (0.669-0.786)] and ramification scores [AUC (95% CI)=0.588 (0.521-0.654)] did not perform equally well. Notably, clinical correlations of capillaroscopic parameters in SSc were found not to be consistent over time, when longitudinally assessed at two different time points by univariate and multivariate analysis. Binary logistic regression analysis indicated that baseline capillaroscopic pattern could predict occurrence of a combined adverse disease outcome (FVC deterioration>10% and/or DLCO deterioration>15% and/or mRSS deterioration>3.5 and/or first occurrence of digital ulcers and/or death), after a mean±SD follow up of 3.28±1.45 years in 94 SSc patients with available follow-up data (OR=3.43, p=0.031 for active versus early pattern, OR=8.778, p=0.007 for late versus early pattern). Conclusion Dilatation score performs best of all semi-quantitative NVC parameters in diagnosing SSc and although clinical correlations of capillaroscopic findings change over time, an active or late capillaroscopy pattern at baseline is associated with an adverse prognosis. Disclosure of Interests Vasiliki-Kalliopi Bournia: None declared, Konstantinos Kottas: None declared, Stylianos Panopoulos: None declared, George Konstantonis: None declared, Alexios Iliopoulos: None declared, Athanasios Tzioufas Grant/research support from: ABBVIE, PFIZER, AMGEN, NOVARTIS, GSK, Petros Sfikakis: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared