结缔组织病和血管炎中的糖皮质激素抵抗性血小板减少症

[Corticosteroid resistance thrombocytopenia in connective tissue disorders and vasculitis].

作者信息

Rezuş Elena, Rezuş C, Răşcanu Anca, Bârzoi Raluca, Rodica Chiriac

机构信息

Universitatea de Medicină şi Farmacie Gr. T. Popa, Iaşi, Facultatea de Medicină, Clinica I Reumatologie si Recuperare Medicală.

出版信息

Rev Med Chir Soc Med Nat Iasi. 2006 Apr-Jun;110(2):267-74.

PMID:17802930

Abstract

In rheumatic diseases there can appear deteriorations of the thrombocytes number in the sense of increase or decrease of this number.Thrombocytosis has 3 major causes: (1) reactive or secondary thrombocytosis; (2) family thrombocytosis and (3) clonal thrombocytosis. Thrombocytopenia, that is, decrease of the thrombocytes number below 150000/mmc is unusually in rheumatic diseases. Their mechanism of production can be central and peripheral. In the connective tissue disorders and vasculitis thrombocytopenia can has different causes: (1) decrease thrombocytes production; (2) splenic platelets sequestration; (3) peripheral platelets consumption; (4) peripheral immune mediated destruction of platelets. Thrombocytopenia is present in the following rheumatic diseases: systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, Felty syndrome, vasculitis. Steroids are the conventional first line therapy for immune thrombocytopenia. Corticosteroid resistance can develop as a result of deteriorations that appear to the any level of pathway action of corticosteroids.

摘要

在风湿性疾病中，血小板数量可能会出现增减变化。血小板增多症有3个主要原因：（1）反应性或继发性血小板增多症；（2）家族性血小板增多症；（3）克隆性血小板增多症。血小板减少症，即血小板数量降至150000/mmc以下，在风湿性疾病中并不常见。其产生机制可分为中枢性和外周性。在结缔组织疾病和血管炎中，血小板减少症可能有不同原因：（1）血小板生成减少；（2）脾脏血小板滞留；（3）外周血小板消耗；（4）外周免疫介导的血小板破坏。血小板减少症存在于以下风湿性疾病中：系统性红斑狼疮、抗磷脂综合征、类风湿关节炎、费尔蒂综合征、血管炎。类固醇是免疫性血小板减少症的传统一线治疗药物。由于皮质类固醇作用途径任何水平出现的恶化，可能会产生皮质类固醇耐药性。