Management of disease progression after autologous hematopoietic stem cell transplantation in systemic sclerosis: Results from an international questionnaire-based study

IF 4.6 2区医学 Q1 RHEUMATOLOGY

Seminars in arthritis and rheumatism Pub Date : 2025-01-31 DOI:10.1016/j.semarthrit.2025.152638

Julia Spierings , Giulia Bandini , Yannick Allanore , Nicoletta Del Papa , Christopher P Denton , Oliver Distler , Daniel E. Furst , Raffaella Greco , Dinesh Khanna , Masataka Kuwana , Marco Matucci-Cerinic , Mandana Nikpour , Anna van Rhenen , Jacob M van Laar , Michael Hughes

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引用次数: 0

Abstract

Objective

Autologous stem cell transplantation (AHSCT) is an established treatment in diffuse cutaneous systemic sclerosis (dcSSc). Optimal management of disease progression after AHSCT in dcSSc has not been defined. The aim of this study was to explore the experience and preferences of SSc experts on post-AHSCT management.

Methods

An online questionnaire study was conducted containing 17 questions concerning respondent demographics, definition of SSc progression after AHSCT, diagnostic work-up and treatment preferences.

Results

In total, 69 respondents from 21 countries completed the questionnaire. The majority (89.7 %) works at a university hospital, and were involved in decisions regarding AHSCT in patients with SSc (71 %). Most have 1 to 5 patients who underwent AHSCT under their care. They defined failure to improve after AHSCT as: an increase in mRSS, new onset or worsening of interstitial lung disease (ILD), new onset scleroderma renal crisis (SRC) or inflammatory arthritis. Progression after initial response was defined as: increase in mRSS, new or worsening of ILD, new SRC, inflammatory arthritis, new pulmonary arterial hypertension, digital vasculopathy or impaired physical functioning. The most frequent therapy in case of AHSCT failure was mycophenolate mofetil (N = 55, 88.7 %), rituximab (N = 54, 87.1 %), nintedanib (N = 39, 62.9 %) or/and tocilizumab (N = 36, 58.1 %). Combination therapy with more than one of these agents was considered by most respondents (N = 61, 88.4 %).

Conclusion

Our study benchmarks the unique combined experiences of post-AHSCT management among SSc experts. We summarize preferences regarding definition of AHSCT failure and progression after response, as well as approach to diagnostic work-up and treatment.

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来源期刊

Seminars in arthritis and rheumatism 医学-风湿病学

CiteScore

9.20

自引率

4.00%

发文量

176

审稿时长

46 days

期刊介绍： Seminars in Arthritis and Rheumatism provides access to the highest-quality clinical, therapeutic and translational research about arthritis, rheumatology and musculoskeletal disorders that affect the joints and connective tissue. Each bimonthly issue includes articles giving you the latest diagnostic criteria, consensus statements, systematic reviews and meta-analyses as well as clinical and translational research studies. Read this journal for the latest groundbreaking research and to gain insights from scientists and clinicians on the management and treatment of musculoskeletal and autoimmune rheumatologic diseases. The journal is of interest to rheumatologists, orthopedic surgeons, internal medicine physicians, immunologists and specialists in bone and mineral metabolism.