Department of Hematology, Oncology, and Rheumatology, Heidelberg University Hospital, Heidelberg, Germany.
Department of Hematology, Oncology, and Rheumatology, Heidelberg University Hospital, Heidelberg, Germany
Ann Rheum Dis. 2024 Aug 27;83(9):1200-1207. doi: 10.1136/ard-2023-225114.
AA amyloidosis (AA) can be the consequence of any chronic inflammatory disease. AA is associated with chronic inflammatory diseases (cid+AA), autoinflammatory syndromes (auto+AA) or AA of unknown origin or idiopathic AA (idio+AA). The major organ manifestation is renal AA that can progress to end-stage renal disease (ESRD) and multiple organ failure.
This study is a monocentric retrospective analysis of the renal outcome and survival of patients with cid+AA (n=34), auto+AA (n=24) and idio+AA (n=25) who were treated with cytokine-inhibiting biological disease-modifying antirheumatic drugs (bDMARDs).
83 patients with renal AA were identified and followed for a mean observational period of 4.82 years. C reactive protein (CRP), serum amyloid alpha and proteinuria were significantly reduced with bDMARD therapy. Progression to ESRD was prevented in 60% (cid+AA), 88% (auto+AA) and 81% (idio+AA) of patients. Tocilizumab was given to 34 patients with cid+AA and idio+AA and was more effective in reducing CRP and progression to ESRD and death compared with other bDMARDs.
bDMARDs reduce systemic inflammation in various diseases, leading to a reduction of proteinuria and prevention of ESRD. Importantly, tocilizumab was more effective than other bDMARDs in controlling systemic inflammation in patients with chronic inflammatory diseases and idiopathic AA, leading to better renal and overall survival.
AA 淀粉样变性(AA)可能是任何慢性炎症性疾病的结果。AA 与慢性炎症性疾病(cid+AA)、自身炎症性综合征(auto+AA)或原因不明的 AA(idio+AA)或特发性 AA(idio+AA)相关。主要的器官表现是肾 AA,可进展为终末期肾病(ESRD)和多器官衰竭。
本研究是对接受细胞因子抑制生物改善病情抗风湿药物(bDMARDs)治疗的 cid+AA(n=34)、auto+AA(n=24)和 idio+AA(n=25)患者的肾脏结局和生存的单中心回顾性分析。
确定了 83 例肾 AA 患者,并进行了平均 4.82 年的观察期。bDMARD 治疗可显著降低 C 反应蛋白(CRP)、血清淀粉样蛋白 alpha 和蛋白尿。60%(cid+AA)、88%(auto+AA)和 81%(idio+AA)的患者进展为 ESRD 得到预防。34 例 cid+AA 和 idio+AA 患者给予托珠单抗,与其他 bDMARDs 相比,托珠单抗在降低 CRP 和进展为 ESRD 和死亡方面更有效。
bDMARDs 可降低各种疾病中的全身炎症,导致蛋白尿减少和 ESRD 预防。重要的是,与其他 bDMARDs 相比,托珠单抗在控制慢性炎症性疾病和特发性 AA 患者的全身炎症方面更有效,从而导致更好的肾脏和总体生存。