Successful long-term systemic sclerosis treatment by high-frequent low-dose B cell-depleting therapy

Abstract: Objectives
Systemic sclerosis (SSc) is a multiorgan disease with a 10-year mortality rate of up to 50 %. B cell-depleting therapy with rituximab (RTX) appears effective in SSc treatment, but data from randomized controlled trials (RCTs) are lacking, and the frequency and dosage of RTX in SSc have no consensus. We aimed to evaluate the long-term efficacy and safety of quarterly RTX administration in SSc.

Methods
This study retrospectively analyzed 40 patients with SSC treated with RTX twice within 14 days every 3 months from 2010 to 2020. The patients fulfilled the LeRoy and the American College of Rheumatology/European League Against Rheumatism Criteria for SSc. Modified Rodnan skin score (mRSS), lung function test results, and serum immunoglobulin (IgG, IgA, and IgM) concentrations were analyzed.

Results
A total of 40 patients with SSc received RTX over a median time of 3.9 years (range: 1–10 years). The median mRSS (baseline: 19, 24 months: 16, p < 0.001) demonstrated a significant improvement, and the predicted forced vital capacity was stable. No new or unexpected safety signals, especially regarding treatment-related infectious adverse events, were observed. Immunoglobulin concentrations were within normal range, and specific antibodies to pneumococcal polysaccharides were preserved despite long-term B cell-depleting therapy. None of the patients died during the observation period of up to 10 years.

Conclusion
SSc was effectively and safely treated with low-dose RTX quarterly. RCTs are warranted to validate the advantage of continuous B cell depletion by quarterly low-dose RTX administration compared to other treatment intervals

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch
• Notes: Journal of autoimmunity. - 147 (2024) , 103246, ISSN: 1095-9157

Contributors, Places and Time

• Event: Veröffentlichung
• (where): Freiburg
• (who): Universität
• (when): 2024
• Creator: Moazedi-Fuerst, Florentine C. ; Lackner, Angelika ; Kreuzer, S.M ; Eller, Kathrin ; Odler, Balázs ; Kovacs, Gabor ; Flick, Holger ; Talakic, Emina ; Hermann, Johann A. ; Venhoff, Nils ; Venhoff, Ana ; Hafner, Franz ; Brodmann, Marianne ; Jud, Philipp ; Yazdani-Biuki, Babak ; Husic, Rusmir ; Salmhofer, Wolfgang ; Stradner, Martin Helmut ; Graninger, Winfried B. ; Thiel, Jens ; Brezinschek, Hans Peter

Further information

• DOI: 10.1016/j.jaut.2024.103246
• URN: urn:nbn:de:bsz:25-freidok-2492785
• Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 14.08.2025, 10:52 AM CEST