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Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort

2022; Oxford University Press; Volume: 61; Issue: 12 Linguagem: Inglês

10.1093/rheumatology/keac188

1462-0332

Elisabetta Zanatta, Dörte Huscher, Augusta Ortolan, Jérôme Avouac, Paolo Airò, Alexandra Balbir‐Gurman, Elise Siegert, Marco Matucci‐Cerinic, Franco Cozzi, Gabriela Riemekasten, Anna‐Maria Hoffmann‐Vold, Oliver Distler, Tianlu Li, Stefan Heitmann, Nicolas Hunzelmann, Carlomaurizio Montecucco, Jadranka Morović‐Vergles, Camillo Ribi, Andrea Doria, Yannick Allanore, Giovanna Cuomo, Gianluca Moroncini, Jiří Štork, Florenzo Iannone, Ulrich A. Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, É. Hachulla, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anić, Maria Üprus, B. Granel, Alessandra Vacca, Cristina-Mihaela Tănăseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros P. Sfikakis, Maurizio Cutolo, Patrícia Carreira, Rosario Foti, Srđan Novak, Michele Iudici, Mislav Radić, Raffaele Pellerito, Carlo Francesco Selmi Rozzano, Lidia P Ananieva, G Szücs, Carlos de la Puente, Ruxandra Ionescu, Jörg H. W. Distler, Maria Rosa Pozzi, Juan José Alegre Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz Altunizade, Carolina de Souza Müller, Svetlana Agachi, Douglas J. Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, I. Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Raffaele Pellerito, Christopher P. Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria Granollers, Michaela Köhm, Bojana Stamenković,

Inflammatory Myopathies and Dermatomyositis

To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc.SSc patients enrolled in the EUSTAR cohort with a disease duration of ≤3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline ≥10% and ≥5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality.We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline ≥10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline ≥5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc.ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.