TY - JOUR
T1 - Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort
AU - Zanatta, Elisabetta
AU - Huscher, Dörte
AU - Ortolan, Augusta
AU - Avouac, Jérôme
AU - Airò, Paolo
AU - Balbir-Gurman, Alexandra
AU - Siegert, Elise
AU - Matucci Cerinic, Marco
AU - Cozzi, Franco
AU - Riemekasten, Gabriela
AU - Hoffmann-Vold, Anna-Maria
AU - Distler, Oliver
AU - Gabrielli, Armando
AU - Heitmann, Stefan
AU - Hunzelmann, Nicolas
AU - Montecucco, Carlomaurizio
AU - Morovic-Vergles, Jadranka
AU - Ribi, Camillo
AU - Doria, Andrea
AU - Allanore, Yannick
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
KW - SSc
KW - anti-topoisomerase I
KW - cutaneous form
KW - scleroderma
KW - interstitial lung disease
KW - outcome
KW - disease subset
KW - SSc
KW - anti-topoisomerase I
KW - cutaneous form
KW - scleroderma
KW - interstitial lung disease
KW - outcome
KW - disease subset
UR - http://hdl.handle.net/10807/305230
U2 - 10.1093/rheumatology/keac188
DO - 10.1093/rheumatology/keac188
M3 - Article
SN - 1462-0332
VL - 61
SP - 4786
EP - 4796
JO - Rheumatology
JF - Rheumatology
ER -