The clinical profile of systemic sclerosis without a 'scleroderma pattern' at nailfold capillaroscopy: results from the multicenter SPRING registry of the Italian Society of Rheumatology.
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Tonutti Antonio, De Santis Maria, Motta Francesca, Bajocchi Gianluigi, Giuggioli Dilia, Bellando-Randone Silvia, Bruni Cosimo, Zanframundo Giovanni, Foti Roberta, Cuomo Giovanna, Ariani Alarico, Rosato Edoardo, Lepri Gemma, Girelli Francesco, Zanatta Elisabetta, Bosello Silvia Laura, Cavazzana Ilaria, Ingegnoli Francesca, Cacciapaglia Fabio, Murdaca Giuseppe, Abignano Giuseppina, Pettiti Giorgio, Della Rossa Alessandra, Caminiti Maurizio, Iuliano Anna Maria, Ciano Giovanni, Beretta Lorenzo, Bagnato Gianluca, Lubrano Ennio, De Andres Ilenia, Idolazzi Luca, Saracco Marta, Agnes Cecilia, Campochiaro Corrado, De Luca Giacomo, Orlandi Martina, Di Battista Marco, Nivuori Mariangela, Milazzo Livio, Cipolletta Edoardo, Lumetti Federica, Spinella Amelia, Cocchiara Emanuele, Codullo Veronica, Moccaldi Beatrice, Visalli Elisa, Iandoli Carlo, Gigante Antonietta, Pellegrino Greta, Pigatto Erika, Lazzaroni Maria Grazia, De Lorenzis Enrico, Mennillo Gianna, Pagano-Mariano Giuseppa, Furini Federica, Vultaggio Licia, Parisi Simone, Peroni Clara Lisa, Fusaro Enrico, Bianchi Gerolamo, Sebastiani Gian Domenico, Govoni Marcello, D'Angelo Salvatore, Cozzi Franco, Franceschini Franco, Guiducci Serena, Riccieri Valeria, Dagna Lorenzo, Salvarani Carlo, Iannone Florenzo, Matucci-Cerinic Marco, Ferri Clodoveo, De Angelis Rossella
Аннотация
METHODS: This retrospective analysis on the multicenter Italian SPRING cohort of SSc patients assessed the prevalence and characteristics of patients without NVC-SP. Associations with demographic and clinical features were evaluated using logistic regression (cross-sectional) and mixed-effects models (longitudinal).
RESULTS: Out of 1689 SSc patients with available NVC information, 90 (5.3%) did not have the NVC-SP. These patients were older at SSc onset (53 vs. 49 years) and more frequently sine scleroderma (31% vs 12%, p < 0.001). The overall burden of vascular complications was milder in this subset, with a lower prevalence of pitting scars (33% vs 48%), telangiectasias (52% vs 74%), and calcinosis (3.4% vs 12%). During the follow-up, mRSS remained lower (60-month mean 4 vs 7, p = 0.002), as well as the estimated probability of vascular complications. Moreover, despite comparable values at baseline, DLCO remained significantly higher in patients without NVC-SP at the following time points (12, 24, and 60 months, p < 0.05). The presence of "late" NVC-SP was independently associated with ILD (OR 1.83, 95% CI 1.05-3.18).
CONCLUSIONS: The absence of NVC-SP in 5.3% of SPRING SSc patients may identify a subset characterized by milder disease, with a lower burden of vascular and organ complications. Conversely, lung involvement was more frequently observed in patients with more severe NVC microangiopathy.