Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres

Fransen, Jaap; Popa-Diaconu, D.A.; Hesselstrand, Roger; Carreira, Patrícia; Valentini, Gabriele; Beretta, Lorenzo; Airò, Paolo; İnanç, Murat; Ullman, S; Balbir‐Gurman, Alexandra; Sierakowski, S; Allanore, Yannick; Czirják, László; Riccieri, Valeria; Giacomelli, Roberto; Gabrielli, Armando; Riemekasten, Gabriela; Matucci‐Cerinic, Marco; Farge, Dominique; Hunzelmann, Nicolas; Hoogen, F.H.J. van den; Vonk, Madelon C.

doi:10.1136/ard.2010.144360

Cited by 114 publications

(81 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…1 Severe progressive cutaneous SSc with visceral involvement has a poor prognosis, with mortality rates that may reach 30% 5 years after diagnosis, despite standard therapies. 2,3 In the past 20 years, autologous hematopoietic stem cell transplantation (AHSCT) has been shown to be effective to treat severe or rapidly progressive SSc. Improved skin score and quality of life and at least stabilization of lung function were consistently reported in early phase II trials.…”

Section: Introductionmentioning

confidence: 99%

Does ex vi vo CD34+ positive selection influence outcome after autologous hematopoietic stem cell transplantation in systemic sclerosis patients?

Soga

Labopin

Henes

et al. 2015

Bone Marrow Transplant

Self Cite

View full text Add to dashboard Cite

This EBMT Autoimmune Disease Working Party study aimed to evaluate the influence of CD34+ positive graft selection (CD34+) on the outcome of systemic sclerosis (SSc) patients after autologous hematopoietic stem cell transplantation (AHSCT). Clinical and laboratory data from 138 SSc patients at diagnosis, before and after AHSCT were retrospectively analyzed. CD34+ selection was performed in 47.1% (n = 65) patients. By multivariate analysis adjusting for all factors differing between the two groups (without or with CD34+), there was no statistically significant difference in terms of overall survival (hazard ratio (HR): 0.98, 95% confidence interval (CI) 0.40-2.39, P = 0.96), PFS (HR: 1.55, 95% CI 0.83-2.88, P = 0.17) and incidence of relapse or progression (HR: 1.70, 95% CI 0.85-3.38, P = 0.13). We demonstrate that CD34+ does not add benefit to the outcome of SSc patient treated with AHSCT. These findings should be further confirmed by prospective randomized trials.

show abstract

Section: Introductionmentioning

confidence: 99%

Does ex vi vo CD34+ positive selection influence outcome after autologous hematopoietic stem cell transplantation in systemic sclerosis patients?

Soga

Labopin

Henes

et al. 2015

Bone Marrow Transplant

Self Cite

View full text Add to dashboard Cite

show abstract

“…Although prior studies have shown that male sex 1) is a predictor of poor survival in SSc (without lung involvement) (25) and SSc-related PAH (13,26), 2) is a predictor of the development of severe ILD (without associated PH) (27), and 3) may be overrepresented in SSc-related PH-ILD (versus SSc-related PAH) (18), our study is the first to suggest that male sex is an independent predictor of poor survival in the setting of wellcharacterized SSc-related PH-ILD. In addition, the PVR index has been previously reported by Mathai and coworkers (5) as a predictor of mortality in SSc-related PH-ILD; however, the hazard ratio (1.05) was similar to that in our study (1.00).…”

Section: Discussionmentioning

confidence: 99%

Improved Transplant‐Free Survival in Patients With Systemic Sclerosis–Associated Pulmonary Hypertension and Interstitial Lung Disease

Volkmann

Saggar

Khanna

et al. 2014

Arthritis & Rheumatology

View full text Add to dashboard Cite

Objective. Survival in patients with systemic sclerosis (SSc)-associated pulmonary hypertension (PH) and interstitial lung disease (ILD) is poor. Evidence supporting the efficacy of aggressive pulmonary arterial hypertension (PAH)-targeted therapy in this population is limited. The aim of this study was to investigate transplant-free survival in patients with isolated SScrelated PAH or SSc-related PH-ILD who were treated with aggressive PAH-targeted therapy.Methods. SSc patients with right-sided heart catheterization (RHC)-diagnosed precapillary PH (mean pulmonary artery pressure >25 mm Hg, pulmonary capillary wedge pressure <15 mm Hg, and pulmonary vascular resistance >240 dynes ؋ second/cm 5 )were included. Patients were classified as having ILD based on review of high-resolution computed tomography (CT) chest imaging and spirometry. The Kaplan-Meier method was applied and Cox proportional hazards models were constructed to analyze survival and identify predictive variables. Results. Of 99 patients with SSc-related precapillary PH, 28% had SSc-related PAH and 72% hadSSc-related PH-ILD. The 1-and 2-year survival estimates were, respectively, 72% and 59% in the SScrelated PH-ILD group versus 82% and 66% in the SSc-related PAH group (P ‫؍‬ 0.5). Within 6 months of the diagnostic RHC, 24% of all patients were started on prostanoid therapy; an additional 24% were started on prostanoid therapy after 6 months. In the multivariate model, male sex (hazard ratio [HR] 0.7, P ‫؍‬ 0.01) and prostanoid therapy initiation within 6 months of the RHC (HR 1.4, P ‫؍‬ 0.01) were the only factors significantly associated with transplant-free survival, after accounting for the presence of ILD and severity of PH.Conclusion. In this study, survival of patients with SSc-related PH-ILD was modestly improved relative to historical series. While these findings may not be generalizable, improved survival may be due partly to aggressive PAH-targeted therapy.

show abstract

“…Weiterhin ergaben diese Untersuchungen, dass der Nachweis von CCP-Antikör-pern signifikant mit einer Synovitis assoziiert ist (OR 4,8;Mierau et al,pers. Mitteilung).…”

Section: Die Kooperationspartner Des Dnssunclassified

“…Dies spiegelt sich nicht zuletzt in einer verglichen mit dem Ende des letzten Jahrhunderts deutlich günstigeren Prognose der Erkrankung wider [1,4] …”

Section: Fazit Für Die Praxisunclassified

Das Deutsche Netzwerk für Systemische Sklerodermie (DNSS)

Hunzelmann

Krieg²,

Dnss³

2012

Hautarzt

View full text Add to dashboard Cite

An essential prerequisite for progress in understanding the pathophysiology and the clinical treatment of rare diseases is the national cooperation of specialized centers. The German network for systemic sclerosis (DNSS) is such an interdisciplinary union of hospitals and research centers with a special interest in systemic sclerosis (SSc). A core activity is the patient register of the DNSS which includes over 3,100 patients. It is one of largest national registers of SSc patients worldwide and comprises prospective data on diagnostics as well as primarily therapy of the patients. The register has now proven to be an extremely successful basis for clinical research and basic studies within the framework of international cooperation. The most important results of the cooperation and the register will be presented in this article.

show abstract

Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres

Abstract: A simple prognostic model using three disease factors to predict 5-year survival at diagnosis in SSc showed reasonable performance upon validation in a European multicentre study.

Cited by 114 publications

References 17 publications

Does ex vi vo CD34+ positive selection influence outcome after autologous hematopoietic stem cell transplantation in systemic sclerosis patients?

Does ex vi vo CD34+ positive selection influence outcome after autologous hematopoietic stem cell transplantation in systemic sclerosis patients?

Improved Transplant‐Free Survival in Patients With Systemic Sclerosis–Associated Pulmonary Hypertension and Interstitial Lung Disease

Das Deutsche Netzwerk für Systemische Sklerodermie (DNSS)

Contact Info

Product

Resources

About