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01-07-2021 | Systemic Sclerosis | Review Article

B cells in systemic sclerosis: from pathophysiology to treatment

Authors: Konstantinos Melissaropoulos, Dimitrios Daoussis

Published in: Clinical Rheumatology | Issue 7/2021

Abstract

Systemic sclerosis is a debilitating autoimmune disease with unknown pathogenesis. The clinical phenotype of fibrosis is preceded by vascular and immunologic aberrations. Adaptive immunity has been extensively studied in patients with the disease and B cells appear to be dysregulated. This is evident in peripheral blood B cell subsets, with activated effector B cells and impaired B regulatory function. In addition, B cells infiltrate target organs and tissues of patients with the disease, such as the skin and the lung, indicating a probable role in the pathogenesis. Impaired B cell homeostasis explains the rationale behind B cell therapeutic targeting. Indeed, several studies in recent years have shown that depletion of B cells appears to be a promising treatment alongside current established therapeutic choices, such as mycophenolate. In this review, B cell aberrations in animal models and human patients with systemic sclerosis will be presented. Moreover, we will also summarize current existing data regarding therapeutic targeting of the B cells in systemic sclerosis.

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Title: B cells in systemic sclerosis: from pathophysiology to treatment
Authors: Konstantinos Melissaropoulos
Dimitrios Daoussis
Publication date: 01-07-2021
Publisher: Springer International Publishing
Keywords: Systemic Sclerosis
Rituximab
Scleroderma
Rituximab
Published in: Clinical Rheumatology / Issue 7/2021
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-021-05665-z

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