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Ann Rheum Dis. 2019 Dec;78(12):1681-1685. doi: 10.1136/annrheumdis-2019-215787. Epub 2019 Aug 17.

Revised European Scleroderma Trials and Research Group Activity Index is the best predictor of short-term severity accrual.

Author information

1
Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy serena.fasano@unicampania.it.
2
Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy.
3
Department of Rheumatology, University of Verona, Verona, Italy.
4
Dipartimento di Medicina Traslazionale e di Precisione, Sapienza University of Rome, Roma, Italy.
5
Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
6
Department of Rheumatology, University Hospital Ghent, Gent, Belgium.
7
Department of Rheumatology, Charit University Hospital, Berlin, Germany.
8
Department of Development and Regeneration, Laboratory of Tissue Homeostasis and Disease, Skeletal Biology and Engineering Research Center, KU Leuven, Leuven, Belgium.
9
Clinical Medicine and Therapy, Sapienza University of Rome, Rome, Italy.
10
Rheumatology and Clinical Immunology Department, Spedali Civili di Brescia, Brescia, Italy.
11
Department of Rheumatology, Carol Davila University of Medicine and Pharmacy, Bucarest, Romania.
12
Department of Rheumatology, Paris Descartes University, Rheumatology A and INSER U1016, Cochin Hospital, Paris, France.
13
Dipartimento di Medicina, DIMED, Universita degli Studi di Padova, Padova, Italy.
14
Department of Rheumatology, Basel University, Basel, Switzerland.
15
Department of Rheumatology, University of Bari, Bari, Italy.
16
Department of Rheumatology, Ramon y Cajal University Hospital, Madrid, Spain.
17
Department of Internal Medicine III, University of Erlangen, Erlangen, Germany.
18
Chair and Rheumatology Unit, University Clinic AOU Cagliari, Monserrato, Italy.
19
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland.

Abstract

BACKGROUND:

The European Scleroderma Trials and Research Group (EUSTAR) recently developed a preliminarily revised activity index (AI) that performed better than the European Scleroderma Study Group Activity Index (EScSG-AI) in systemic sclerosis (SSc).

OBJECTIVE:

To assess the predictive value for short-term disease severity accrual of the EUSTAR-AI, as compared with those of the EScSG-AI and of known adverse prognostic factors.

METHODS:

Patients with SSc from the EUSTAR database with a disease duration from the onset of the first non-Raynaud sign/symptom ≤5 years and a baseline visit between 2003 and 2014 were first extracted. To capture the disease activity variations over time, EUSTAR-AI and EScSG-AI adjusted means were calculated. The primary outcome was disease progression defined as a Δ≥1 in the Medsger's severity score and in distinct items at the 2-year follow-up visit. Logistic regression analysis was carried out to identify predictive factors.

RESULTS:

549 patients were enrolled. At multivariate analysis, the EUSTAR-AI adjusted mean was the only predictor of any severity accrual and of that of lung and heart, skin and peripheral vascular disease over 2 years.

CONCLUSION:

The adjusted mean EUSTAR-AI has the best predictive value for disease progression and development of severe organ involvement over time in SSc.

KEYWORDS:

autoimmune diseases; outcomes research; systemic sclerosis

Conflict of interest statement

Competing interests: None declared.

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