Pathogenic significance of BSF-2/IL-6 in castleman's disease

Anti‐interleukin‐6 receptor antibody, tocilizumab, for the treatment of autoimmune diseases

“…IL-6 was highly expressed in hyperplastic lymph nodes and in a patient with a solitary hyperplastic lymph node surgical removal of the involved lymph node led to clinical improvement and reduced serum IL-6 concentration [26], suggesting that the generation of IL-6 by hyperplastic lymph nodes of Castleman's disease is the key element responsible for the various clinical symptoms. Two open-label clinical trials of tocilizumab for Castleman's disease showed its marked effect in clinical symptoms and laboratory findings as well as in histologically determined amelioration [52,53], leading to approval of tocilizumab as an orphan drug for Castleman's disease in Japan in 2005.…”

“…The cultured fluid from the myxoma tissues, obtained by autopsy, yielded a large quantity of IL-6 [25], which suggested that IL-6 might contribute to chronic inflammation and autoimmunity. Subsequent studies have shown that dysregulation of IL-6 production is implicated in the pathogenesis of Castleman's disease [26], RA [27] and various other autoimmune, inflammatory and malignant diseases [11,28].…”

Hyaline-Vascular Type Castleman’s Disease, Sarcoidosis, and Crohns Disease

“…IL-6 is a cytokine released by macrophages, fibroblasts and lymphocytes and leads to Band T-lymphocyte proliferation and neutrophil chemotaxis. Overproduction of IL-6 has been associated with CD, Crohn's disease and sarcoidosis [10][11][12]. Tocilizumab and siltuximab, anti-IL-6 antagonists have demonstrated their efficacy in treating multicentric CD [13,14].…”

Synchronous Squamous Cell Carcinoma of Tongue and Unicentric Cervical Castleman’s Disease Clinically Mimicking a Stage IV Disease: A Rare Association or Coincidence?

“…On the other hand, in the case of thymic carcinoma associated with the mediastinal CD, the authors hypothesize that the carcinoma cells release cytokines (IL-6 like) which stimulate development of HVCD [6]. Key role of IL-6, a potent, pleiotropic pro-inflammatory cytokine in the etio-pathogenesis of CD is already well known [4]. Interestingly, significantly elevated serum IL-6 levels have been documented in head and neck squamous cell carcinomas (HNSCC) [11] and IL-6 over expression has been associated with tumor aggressiveness, immune unresponsiveness and distant metastsis [8].…”

“…Small hyalinized and hypervascular germinal centers with hypervascular interfollicular stroma and sinus effacement are common features of the HVCD, whereas hyperplastic germinal centers with plasma cell aggregates in lymph node paracortex and partially spared sinuses are characteristic features of the PCCD [2]. A key event in the pathogenesis of CD has been recently suggested to be an abnormal production of a B cell growth factor, such as IL-6, leading to lymphoid cell proliferation and plasma cell differentiation [3,4].…”