Clinical and serological features of triple autoantibody-negative patients with systemic sclerosis: insights from the multicentric SPRING registry of the Italian Society for Rheumatology.
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Batani Veronica, Cavazzana Ilaria, Orlandi Martina, De Angelis Rossella, Campochiaro Corrado, De Lorenzis Enrico, Natalello Gerlando, Verardi Lucrezia, Bajocchi Gianluigi, Bellando-Randone Silvia, Zanframundo Giovanni, Foti Rosario, Cacciapaglia Fabio, Cuomo Giovanna, Ariani Alarico, Rosato Edoardo, Lepri Gemma, Girelli Francesco, Riccieri Valeria, Zanatta Elisabetta, Ingegnoli Francesca, De Santis Maria, Murdaca Giuseppe, Abignano Giuseppina, Giorgio Pettiti, Della Rossa Alessandra, Caminiti Maurizio, Iuliano Anna Maria, Beretta Lorenzo, Bagnato Gianluca, Lubrano Ennio, De Andres Ilenia, Idolazzi Luca, Bruni Cosimo, Fornaro Marco, Saracco Marta, Agnes Cecilia, Cipolletta Edoardo, Lumetti Federica, Spinella Amelia, De Pinto Marco, Magnani Luca, Codullo Veronica, Visalli Elisa, Iandoli Carlo, Gigante Antonietta, Pellegrino Greta, Pigatto Erika, Lazzaroni Maria-Grazia, Franceschini Franco, Motta Francesca, Tonutti Antonio, Mennillo Gianna, Di Battista Marco, Mariano Giuseppa Pagano, Furini Federica, Vultaggio Licia, Parisi Simone, Peroni Clara Lisa, Bianchi Gerolamo, Fusaro Enrico, Sebastiani Gian Domenico, Govoni Marcello, D'Angelo Salvatore, Cozzi Franco, Guiducci Serena, Doria Andrea, Salvarani Carlo, Iannone Florenzo, D'Agostino Maria Antonietta, Bosello Silvia Laura, Dagna Lorenzo, Giuggioli Dilia, Ferri Clodoveo, Matucci Cerinic Marco, De Luca Giacomo, On the behalf of SPRING collaborators
Аннотация
METHODS: We conducted a retrospective evaluation of triple-negative patients with SSc prevalence and clinical features among the multicentric Systemic sclerosis Progression INvestiGation registry.
RESULTS: Out of 1480 patients with SSc, 295 (19.9%) were triple-negative, while 1185 (81.1%) had SSc-specific antibodies: ACA (54.3%), ATA (43.6%) and RNAP3 (2.1%). The triple-negative group showed a higher prevalence of myopathy (16.7% vs 10.1%, p=0.003), suggested by higher creatine phosphokinase (CPK) levels (126.2 vs 92.5 U/mL, p=0.002), more frequent CPK increase over 2-3 times (2.4% vs 0.2%, p=0.028). Triple-negative patients also exhibited fewer vascular complications, including digital ulcers (17.3% vs 22.8%, p=0.04) and calcinosis (8.2% vs 12.8%, p=0.027), and a higher prevalence of interstitial lung disease (p<0.001). Consistently, lower diffusing capacity for carbon monoxide (66.4% vs 70.98%, p=0.004) and forced vital capacity (97.01% vs 102.92%, p<0.001) were found in the triple-negative group. Triple-negative patients more frequently received corticosteroids (79.3% vs 67.9%, p=0.003), cyclophosphamide (43.4% vs 26%, p<0.001) and azathioprine (38.5% vs 22.3%, p=0.002), while less frequently received prostanoids (71.6% vs 85.9%, p<0.001), calcium channel blockers (80.1% vs 87.7%, p=0.005) and phosphodiesterase-5 inhibitors (4% vs 20%, p<0.001).
CONCLUSIONS: A higher prevalence of myopathy and interstitial lung disease and a reduced vascular burden were found in the triple-negative patients, suggesting that the non-specific and non-routinely tested autoantibodies may identify an SSc endotype resembling sclero-myositis.