Does regression of skin thickening predict improvement of internal organ involvement and survival in patients with diffuse cutaneous systemic sclerosis? A EUSTAR analysis
Küçük Resim Yok
Tarih
2024
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
BMC
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Objective Patients with diffuse cutaneous systemic sclerosis (dcSSc) frequently show spontaneous improvement of skin fibrosis. Our aim was to examine whether an improvement in skin fibrosis predicts lower likelihood of visceral organ progression and better survival. Methods Patients from the European Scleroderma Trials and Research (EUSTAR) cohort with dcSSc, baseline modified Rodnan skin score (mRSS) >= 7, and valid mRSS at 12 +/- 3 months follow up were included. Regression/progression of skin fibrosis was defined as a decrease/increase in mRSS >5 points and >= 25% from baseline to follow up. The outcomes included progression of lung, renal, cardiac and gastrointestinal manifestations using consensus derived definitions and all-cause death. Regressive, stable and progressive patients were compared by univariate, Kaplan-Meier survival curve and Cox regression analysis. Results Of 1257 included patients, 883 (70.2%) were stable, 282 (22.4%) regressive, and 92 (7.3%) progressive. Regressive patients, adjusted for baseline mRSS, baseline immunosuppression, baseline FVC, and disease duration, showed a significantly lower probability of FVC decline >= 10% than progressive patients (p=0.00003), lower probability of all-cause mortality during follow up (p=0.035) compared to progressive patients. .Improvement of skin fibrosis was not associated with progression of other organ manifestations. Conclusion We found that regression of skin fibrosis is associated with a lower probability of lung progression and better survival at follow up. The link between the disease course of skin and lung fibrosis in SSc can help to better stratify patients in clinical practice and enrich for ILD progressive patients in clinical trials.
Açıklama
Anahtar Kelimeler
Observation studies, Rare diseases, Respiratory, Scleroderma and related disorders, Skin
Kaynak
Arthritis Research & Therapy
WoS Q Değeri
Q1
Scopus Q Değeri
Q1
Cilt
26
Sayı
1
Künye
Wyss, A., Jordan, S., Graf, N., Carreira, P. E., Distler, J., Cerinic, M. M., Siegert, E., Henes, J., Zanatta, E., Riccieri, V., Truchetet, M., Oksel, F., Li, M., Kucharz, E. J., Eyerich, K., Del Galdo, F., Vonk, M. C., Vold, A. H., Gabrielli, A., . . . EUSTAR collaborators. (2024). Does regression of skin thickening predict improvement of internal organ involvement and survival in patients with diffuse cutaneous systemic sclerosis? A EUSTAR analysis. Arthritis Research & Therapy, 26(1), 187-10.
