Inextricable association of connective tissue disease with B‑cell lymphoma (Review).

Connective tissue disease (CTD) is a kind of autoimmune disease with multisystem damage that mainly involves the bone, muscle and the vascular system. Patients with CTD have an increased incidence of malignant tumors, particularly hematological malignancies, compared to the general population. This association of autoimmune diseases with lymphoproliferative diseases is bidirectional. There is a heightened risk of B-cell lymphoma development among patients with CTD, and patients with autoimmune disease display a higher prevalence of non-Hodgkin lymphoma compared to the general population. More than 80% of malignant tumours occur after or at the same time as CTD develops. Among secondary lymphomas, the most common aggressive type of lymphoma is diffuse large B-cell lymphoma, while the most common indolent type is marginal zone lymphoma. Novel targets in patients with B-cell lymphoma are BCL2, the NF-κB pathway, components of the BCR activator of RhoGEF and GTPase signalling pathway and the PI3K-mTOR pathway. In this review, information is provided on the common types of B-cell lymphoma in CTD, the pathogenic factors implicated in lymphoma development and recent advancements in therapies effective for both autoimmune conditions and malignant lymphoproliferative diseases.