Ectopic and high CXCL13 chemokine expression in myasthenia gravis with thymic lymphoid hyperplasia

Different molecular expression in thymoma with ocular or generalized myasthenia gravis

“…of germinal center-like follicles in autoimmune diseases. Overexpression of CXCL13 has been observed in the MG thymi [17], and ectopic thymic CXCL13 expression is associated with aberrant B cell trafficking to the thymus of MG [18]. A higher CXCL13 level in MG patients correlates with the more severe symptoms [17].…”

“…Furthermore, the chemokine CXCL13 is known to mediate the homing and motility of B cells in secondary lymphoid tissue [13][14][15], and play an essential role in maintaining T cells in lymphocytic infiltrates and ectopic follicles of patients with various autoimmune diseases [16]. Previous evidence has shown that CXCL13 is increased in MG patients and high CXCL13 expression is associated with severe clinical stages, especially in females with thymic hyperplasia [17,18]. Unfortunately, It remains unclear which molecule plays a fundamental role in the development of OMG or GMG in the presence of thymoma.…”

The Course of Myasthenia Gravis with Systemic Lupus Erythematosus

“…There are similar correlations with MG and CXCL13. The high CXCL13 concentrations were correlated with severe clinical stage of myasthenia especially in female patients with thymic lymphoid hyperplasia [13]. In addition, Meraouna et al showed that the CXCL13 concentration was increased in thymus and sera of glucocorticoid-untreated patients and decreased in glucocorticoid-treatment in correlation with clinical improvement [14].…”

Effects of CXCL13 inhibition on lymphoid follicles in models of autoimmune disease

“…CXCL13 expression is associated with secondary lymphoid tissue in RA [6] (Table 1), predominantly localized to FDC in the B-cell areas of ectopic lymphoid aggregates with germinal centre-like structures [4] but also in loose aggregations suggestive of a role in generation and organization of lymphoid [52][53][54][55][56][57] Positive correlation between CXCL13 levels and relapse rates [54] CXCL13 levels correlate with intrathecal Ig production and the presence of B cells, T cell and plasma cells in the CNS [55,56] Rheumatoid Arthritis Joint CXCL13 is expressed in lymphoid follicles in synovium [6,14,15] Decreased percentage of CXCR5+ B cells in peripheral blood, while CXCR5 expression is elevated in synovia [58,59] CXCR5 highly expressed on B cells and T cells in inflamed joints [59] Sjogren's syndrome Salivary gland CXCL13 expression is elevated in salivary glands [60][61][62][63][64] A higher degree of lymphoid organization in salivary gland biopsies correlates with increased expression of CXCL13 [65] CXCR5+ B cells and T cells accumulate in the salivary gland [60,63] Autoimmune thyroid disease Thyroid gland CXCL13 and CXCR5 expression is increased in thyroid tissue of patients with Hashimoto thyroiditis and Graves' Disease [66,67] CXCL13 and CXCR5 expression positively correlate with lymphocytic infiltrates and germinal centres in the thyroid [67] Myasthenia gravis Thymus CXCL13 and CXCR5 expression is upregulated in thymus [68,69] Serum CXCL13 levels associate with disease severity in subsets of patients [69] neogenesis in the synovium [14]. Additionally, stromal cells and macrophages/monocytes at inflammatory lesions can also contribute to CXCL13 expressio...…”