期刊
CLINICAL RHEUMATOLOGY
卷 40, 期 7, 页码 2621-2631出版社
SPRINGER LONDON LTD
DOI: 10.1007/s10067-021-05665-z
关键词
B cell; CD19; CD22; Rituximab; Scleroderma; Systemic sclerosis
类别
Systemic sclerosis is a debilitating autoimmune disease with unknown pathogenesis, characterized by vascular and immunologic aberrations. B cells play a dysregulated role in the disease, with abnormal subsets observed in peripheral blood and target organs like the skin and lungs. Therapeutic targeting of B cells has shown promise as a treatment alongside current options like mycophenolate.
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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