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.
Microvascular alterations can be detected with nailfold videocapillaroscopy, useful for systemic sclerosis (SSc) diagnosis upon identification of the "scleroderma pattern" (NVC-SP). However, this pattern is missing in a subset of SSc patients.
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).
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).
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.
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).
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).
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.
Authors
Tonutti Tonutti, De Santis De Santis, Motta Motta, Bajocchi Bajocchi, Giuggioli Giuggioli, Bellando-Randone Bellando-Randone, Bruni Bruni, Zanframundo Zanframundo, Foti Foti, Cuomo Cuomo, Ariani Ariani, Rosato Rosato, Lepri Lepri, Girelli Girelli, Zanatta Zanatta, Bosello Bosello, Cavazzana Cavazzana, Ingegnoli Ingegnoli, Cacciapaglia Cacciapaglia, Murdaca Murdaca, Abignano Abignano, Pettiti Pettiti, Della Rossa Della Rossa, Caminiti Caminiti, Iuliano Iuliano, Ciano Ciano, Beretta Beretta, Bagnato Bagnato, Lubrano Lubrano, De Andres De Andres, Idolazzi Idolazzi, Saracco Saracco, Agnes Agnes, Campochiaro Campochiaro, De Luca De Luca, Orlandi Orlandi, Di Battista Di Battista, Nivuori Nivuori, Milazzo Milazzo, Cipolletta Cipolletta, Lumetti Lumetti, Spinella Spinella, Cocchiara Cocchiara, Codullo Codullo, Moccaldi Moccaldi, Visalli Visalli, Iandoli Iandoli, Gigante Gigante, Pellegrino Pellegrino, Pigatto Pigatto, Lazzaroni Lazzaroni, De Lorenzis De Lorenzis, Mennillo Mennillo, Pagano-Mariano Pagano-Mariano, Furini Furini, Vultaggio Vultaggio, Parisi Parisi, Peroni Peroni, Fusaro Fusaro, Bianchi Bianchi, Sebastiani Sebastiani, Govoni Govoni, D'Angelo D'Angelo, Cozzi Cozzi, Franceschini Franceschini, Guiducci Guiducci, Riccieri Riccieri, Dagna Dagna, Salvarani Salvarani, Iannone Iannone, Matucci-Cerinic Matucci-Cerinic, Ferri Ferri, De Angelis De Angelis
View on Pubmed