II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies

ABSTRACT In the last few years, new primary immunodeficiencies and genetic defects have been described. Recently, immunoglobulin products with improved compositions and for subcutaneous use have become available in Brazil. In order to guide physicians on the use of human immunoglobulin to treat primary immunodeficiencies, based on a narrative literature review and their professional experience, the members of the Primary Immunodeficiency Group of the Brazilian Society of Allergy and Immunology prepared an updated document of the 1st Brazilian Consensus, published in 2010. The document presents new knowledge about the indications and efficacy of immunoglobulin therapy in primary immunodeficiencies, relevant production-related aspects, mode of use (routes of administration, pharmacokinetics, doses and intervals), adverse events (major, prevention, treatment and reporting), patient monitoring, presentations available and how to have access to this therapeutic resource in Brazil.摘要 近年来，学界陆续报道了多种新型原发性免疫缺陷病（Primary Immunodeficiencies）与遗传缺陷。近期，成分优化的皮下注射用人免疫球蛋白制剂已在巴西上市。为指导临床医师合理使用人免疫球蛋白（human immunoglobulin）治疗原发性免疫缺陷病，巴西变态反应与免疫学会（Brazilian Society of Allergy and Immunology）原发性免疫缺陷病工作组基于叙事性文献回顾与自身临床经验，对2010年发布的首部巴西原发性免疫缺陷病人免疫球蛋白治疗共识进行了更新修订。本共识文件涵盖了原发性免疫缺陷病免疫球蛋白治疗的适应证与疗效、相关生产工艺要点、给药方案（给药途径、药代动力学特征、给药剂量与间隔周期）、不良反应（严重不良反应、预防措施、治疗方案与不良事件上报流程）、患者监测规范、当前可用的制剂类型，以及巴西境内该治疗资源的获取渠道等内容。