Prevalence, distribution and associations of the scleroderma capillaroscopic patterns: new insights from the Italian SPRING-SIR registry

Objectives: To assess the relationship between disease duration and the prevalence/distribution of nailfold videocapillaroscopy (NVC) patterns, named according to the current classification as “early”, “active”, and “late”, in a large cohort of systemic sclerosis (SSc) patients.

Methods: A cross-sectional analysis was conducted on 1,689 patients undergoing standardised NVC. Clinical-serological data and treatments were collected. Statistical comparisons and multivariable logistic regression models were applied, including analyses based on disease duration.

Results: The prevalence of NVC patterns was as follows: “early” 21.6%, “active” 47.4%, “late” 25.7%, and normal/non-specific 5.3%. The distribution by disease duration showed that the three main patterns were always present. While the “early” and “active” progressively decreased (from 30.3% and 51.9% in patients with ≤5 yrs, to 14.6% and 43.5% in those >10 yrs, p< 0.01), the “late” pattern increased from 13.2% (≤5 yrs) to 36.0% (>10 yrs) (p< 0.001) and was associated with internal organ involvement, anti-topoisomerase antibodies, and more therapies (p< 0.01). Conversely, the “early” and “active” patterns were associated with the limited-cutaneous subset (p< 0.01), and anti-centromere antibodies (p< 0.001). Multivariable analysis confirmed a strong association between the “late” pattern and skin/peripheral vascular involvement. Notably, the presence of the “late” pattern in patients with ≤2 yrs (10.9%) was significantly associated with scleroderma renal crisis (p= 0.012).

Conclusions: SSc-NVC patterns are not strictly time-dependent and can be observed at any stage of the disease, suggesting that microvascular damage progression is heterogeneous across different disease periods. Therefore, a revised classification of NVC changes considering both disease duration and NVC severity could improve its prognostic accuracy.