Universität zu Köln

Zanatta, Elisabetta, Huscher, Doerte, Ortolan, Augusta, Avouac, Jerome, Airo, Paolo, Balbir-Gurman, Alexandra, Siegert, Elise ORCID: 0000-0002-9594-0446, Cerinic, Marco Matucci, Cozzi, Franco, Riemekasten, Gabriela, Hoffmann-Vold, Anna-Maria, Distler, Oliver, Gabrielli, Armando, Heitmann, Stefan, Hunzelmann, Nicolas, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, Ribi, Camillo, Doria, Andrea ORCID: 0000-0003-0548-4983 and Allanore, Yannick (2022). Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort. RHEUMATOLOGY, 61 (12). S. 4786 - 4797. OXFORD: OXFORD UNIV PRESS. ISSN 1462-0332

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Abstract

Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods 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. Results 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. Conclusion 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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Zanatta, Elisabetta UNSPECIFIED UNSPECIFIED UNSPECIFIED Huscher, Doerte UNSPECIFIED UNSPECIFIED UNSPECIFIED Ortolan, Augusta UNSPECIFIED UNSPECIFIED UNSPECIFIED Avouac, Jerome UNSPECIFIED UNSPECIFIED UNSPECIFIED Airo, Paolo UNSPECIFIED UNSPECIFIED UNSPECIFIED Balbir-Gurman, Alexandra UNSPECIFIED UNSPECIFIED UNSPECIFIED Siegert, Elise UNSPECIFIED orcid.org/0000-0002-9594-0446 UNSPECIFIED Cerinic, Marco Matucci UNSPECIFIED UNSPECIFIED UNSPECIFIED Cozzi, Franco UNSPECIFIED UNSPECIFIED UNSPECIFIED Riemekasten, Gabriela UNSPECIFIED UNSPECIFIED UNSPECIFIED Hoffmann-Vold, Anna-Maria UNSPECIFIED UNSPECIFIED UNSPECIFIED Distler, Oliver UNSPECIFIED UNSPECIFIED UNSPECIFIED Gabrielli, Armando UNSPECIFIED UNSPECIFIED UNSPECIFIED Heitmann, Stefan UNSPECIFIED UNSPECIFIED UNSPECIFIED Hunzelmann, Nicolas UNSPECIFIED UNSPECIFIED UNSPECIFIED Montecucco, Carlomaurizio UNSPECIFIED UNSPECIFIED UNSPECIFIED Morovic-Vergles, Jadranka UNSPECIFIED UNSPECIFIED UNSPECIFIED Ribi, Camillo UNSPECIFIED UNSPECIFIED UNSPECIFIED Doria, Andrea UNSPECIFIED orcid.org/0000-0003-0548-4983 UNSPECIFIED Allanore, Yannick UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-697277
DOI:	10.1093/rheumatology/keac188
Journal or Publication Title:	RHEUMATOLOGY
Volume:	61
Number:	12
Page Range:	S. 4786 - 4797
Date:	2022
Publisher:	OXFORD UNIV PRESS
Place of Publication:	OXFORD
ISSN:	1462-0332
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language EULAR SCLERODERMA TRIALS; LUNG-FUNCTION; DISEASE; RECOMMENDATIONS; CLASSIFICATION; TOCILIZUMAB; SUBSETS; UPDATE Multiple languages Rheumatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/69727