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. 2026 Jan 8;65(1):keaf672.
doi: 10.1093/rheumatology/keaf672.

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

Rossella De Angelis  1 , Clodoveo Ferri  2 , Edoardo Cipolletta  1 , Valeria Riccieri  3 , Marco Di Battista  4 , Gianluigi Bajocchi  5 , Silvia Bellando-Randone  6 , Cosimo Bruni  6 , Martina Orlandi  2 , Giovanni Zanframundo  7 , Roberta Foti  8 , Giovanna Cuomo  9 , Alarico Ariani  10 , Edoardo Rosato  11 , Gemma Lepri  6 , Francesco Girelli  12 , Elisabetta Zanatta  13 , Silvia Laura Bosello  14 , Ilaria Cavazzana  15 , Francesca Ingegnoli  16 , Maria De Santis  17 , Fabio Cacciapaglia  18 , Giuseppe Murdaca  19 , Giuseppina Abignano  20 , Giorgio Pettiti  21 , Alessandra Della Rossa  4 , Maurizio Caminiti  22 , Anna Maria Iuliano  23 , Giovanni Ciano  24 , Lorenzo Beretta  25 , Gianluca Bagnato  26 , Ennio Lubrano  27 , Ilenia De Andres  28 , Luca Idolazzi  29 , Marta Saracco  30 , Cecilia Agnes  31 , Corrado Campochiaro  32   33 , Marco Fornaro  34 , Federica Lumetti  2 , Amelia Spinella  2 , Emanuele Cocchiara  5 , Giacomo De Luca  32   33 , Veronica Codullo  7 , Elisa Visalli  8 , Carlo Iandoli  9 , Antonietta Gigante  11 , Greta Pellegrino  35   36 , Erika Pigatto  37 , Maria Grazia Lazzaroni  15 , Enrico De Lorenzis  14 , Francesca Motta  17 , Antonio Tonutti  17 , Gianna Mennillo  20 , Giuseppa Pagano-Mariano  22 , Federica Furini  38 , Licia Vultaggio  38 , Simone Parisi  39 , Clara Lisa Peroni  39 , Gerolamo Bianchi  40 , Enrico Fusaro  39 , Gian Domenico Sebastiani  23 , Marcello Govoni  38 , Salvatore D'Angelo  20 , Franco Cozzi  41 , Franco Franceschini  15 , Serena Guiducci  6 , Dilia Giuggioli  2 , Lorenzo Dagna  32   33 , Andrea Doria  13 , Carlo Salvarani  5 , Florenzo Iannone  34 , Marco Matucci-Cerinic  32   33   42 ; SPRING-SIR (Systemic Sclerosis Progression Investigation group of the Italian Society for Rheumatology)
Collaborators, Affiliations

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

Rossella De Angelis et al. Rheumatology (Oxford). .

Abstract

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 1689 patients undergoing standardized 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).

Conclusion: 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.

Keywords: nailfold capillaroscopy patterns; scleroderma; systemic sclerosis.

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