Background In Systemic Sclerosis (SSc), before the onset of clinical signs of fibrosis, puffy fingers, specific autoantibodies and microcirculatory modifications are present and are identified in the VEDOSS criteria for “(very) early” disease. No large scale studies are available indicating the prevalence of nailfold videocapillaroscopic (NVC) SSc patterns or quantitated capillaroscopic characteristics in a “(very) early” cohort.
Objectives Evaluation of the prevalence of SSc patterns and quantitated capillaroscopic characteristics in a “(very) early” crossectional SSc cohort.
Methods Multicentre observational cohort study of patients with two strata (Figure): 1.Raynaud's phenomenon (RP), Anti Nuclear Antibody (ANA) positive (+), 2. RP+, ANA negative (-). “Target” (RP+, ANA+, puffy finger [PF] +/-, SSc- antibody (SSc-AB)+, NVC+) and “control” (RP+, ANA-, SSc-AB-, NVC-) subsets were described. NVC patterns (normal/non-specific alterations; non-specific abnormalities; SSc-patterns (“Early”, “Active”;“Late”; “Sclerodermalike”) and quantitative (“absent (=“none” or “rare”) and “present” (=“moderate” or “extensive”) capillaroscopic alterations for giants, haemorrhages, capillary loss and abnormally shaped (=bushy capillaries) were evaluated). Generalized Estimating Equations (GEE) were used to assess differences in prevalences of NVC patterns between strata, taking clusters of patients within centers into account.
Results 1085 RP patients from 40 centres (median number [n°] of pt 12 (minimum [min] 1-maximum [max] 393) per centre were enrolled in the VEDOSS online database. Due to erroneous included/missing data (e.g. erroneous: absence/missing information on RP, presence of former ACR criteria for SSc, skin involvement at baseline; missing info on: ANA positivity, PF, Cap, SSc-AB) 750 patients (median n° of 7 (min:1-max:271) were retained for the analysis. SSc nailfold videocapillaroscopic (NVC) patterns (“Early”, “Active”, “Late”, “Scleroderma like” were present in 79%,13%,0%, 8%) as well as presence of “moderate” or “extensive” giants (49%), hemorrhages (32%), capillary loss (11%) and abnormal shapes (11%) of “target” patients and per definition in 0% of “controls”. Estimated distribution of SSc patterns differed in the ANA+/- stratum: 49%/15% (p≤0.001) (“Early” 40/13%; “Active” 5/2%; “Late” 0/0%; “Scleroderma like” 3/0%). For the quantitative capillaroscopic characteristics a statistically significant difference in the presence of “moderate” or “extensive” giants (23/5%, p=0.027) between the ANA+/- stratum was found, hemorrhages (18/9%, p=0.200), capillary loss (5/2%, p=0.798) and abnormal (ramified) shapes (7/2%,p=0.445).
Conclusions The VEDOSS cohort presents predominantly an “Early” NVC SSc pattern. Notably the prevalence of NVC SSc patterns was higher in the ANA+ than ANA- stratum. This evidence further reflects the pivotal role of NVC in the very early diagnosis of SSc.
Disclosure of Interest None declared
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