Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort

Abstract

ObjectivesTo determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc).MethodsWe evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression.ResultsOf 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09).ConclusionsProgressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.

Applicability of shear wave elastography for the evaluation of skin strain in systemic sclerosis

“…In line with the current American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) classification criteria, a skin thickening of the fingers extending proximal to the metacarpophalangeal joints (MCPs) is sufficient to be classified as SSc [2]. The degree of skin thickening in SSc is also an established prognostic factor, which can be used to assess the risk of internal organ involvement, determine the activity of the disease and evaluate treatment response [3][4][5][6][7]. Thus, assessment of skin thickness, which is a manifestation of fibrosis, is one of the essential tests performed in SSc [8].…”

Early trajectories of skin thickening are associated with severity and mortality in systemic sclerosis

“…In particular, it appears relevant to distinguish especially ATA-associated lcSSc from ATAassociated dcSSc [33,34]. Recently, Wu et al reported that in dcSSc, skin progression that occurred within 1 year was independently associated with forced vital capacity decline and all-cause death [35], and Zheng et al reported worst disease outcomes in early dcSSc patients with worsening skin score (≤ 3 years), whose (See figure on previous page.) Fig.…”

Damage index changes in patients with systemic sclerosis: a retrospective analysis over a five-year period

“…Этот прирост был связан в основном с поражением дыхательной системы и ЖКТ. Он демонстрирует, с одной стороны, наибольший темп накопления клинических изменений вследствие избыточного фиброза и микроангиопатии именно в этих системах и согласуется с данными изучения темпа снижения функции легких в регистре EUSTAR [9] и прогрессирования интерстициальной болезни легких по данным КТ органов грудной клетки высокого разрешения в исследовании О.Б. Овсянниковой и соавт.…”