Toker Ori, Aggmon-Levin Nancy, Somech Raz
Harefuah. 2016 Mar;155(3):170-6, 195.
Our immune system protects us from various pathogens, autoimmune processes and malignancy. Primary immunodeficiency disorders are rare, however in contrast to the conventional perception, primary immunodeficiency diseases are more common than expected and may occur at any age. An insult to the immune system, primary or secondary, may lead to an increased incidence of infectious diseases, autoimmune diseases and malignancies. Primary care physicians, frequently encounter children and adults who suffer from recurrent infections, emphasizing the need for a structured approach for the evaluation of patients with suspected immunodeficiency. The growing knowledge of the fundamental mechanisms and function of the immune system together with recent developments in the field of clinical immunodeficiency enables us to use advanced diagnostic tools for the early diagnosis and treatment of these patients. In this review, we summarize the main aspects and updates of primary and secondary immune deficiency diseases, outline the "red flags" of immunodeficiency states and offer a stepwise workup approach for primary physicians and clinical immunology specialists. Some of the immunodeficiency "red flags" include recurrent infections, invasive infections, atypical pathogens, partial response to antibiotic treatment and frequent use of antibiotics, failure to thrive, chronic diarrhea and fungal infections, unexplained skin rash and a family history.
我们的免疫系统保护我们免受各种病原体、自身免疫过程和恶性肿瘤的侵害。原发性免疫缺陷疾病较为罕见,然而,与传统认知相反,原发性免疫缺陷疾病比预期更为常见,且可能发生于任何年龄。对免疫系统的损害,无论是原发性还是继发性的,都可能导致传染病、自身免疫性疾病和恶性肿瘤的发病率增加。初级保健医生经常会遇到反复感染的儿童和成人,这凸显了对疑似免疫缺陷患者进行评估时采用结构化方法的必要性。对免疫系统基本机制和功能的认识不断增加,以及临床免疫缺陷领域的最新进展,使我们能够使用先进的诊断工具对这些患者进行早期诊断和治疗。在本综述中,我们总结了原发性和继发性免疫缺陷疾病的主要方面和最新进展,概述了免疫缺陷状态的“警示信号”,并为初级医生和临床免疫学专家提供了逐步的检查方法。一些免疫缺陷的“警示信号”包括反复感染、侵袭性感染、非典型病原体、对抗生素治疗的部分反应和频繁使用抗生素、生长发育迟缓、慢性腹泻和真菌感染、不明原因的皮疹以及家族病史。