Warnatz K, Peter H-H
Abteilung für Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik Freiburg.
Internist (Berl). 2004 Aug;45(8):868-81. doi: 10.1007/s00108-004-1239-y.
Primary immunodeficiency diseases of the adult are rare disorders, but often lead to serious consequences. Therefore an early diagnosis is critical. The variety in the clinical presentation, the complexity of the immune system and the ongoing discovery of new defects render it a difficult area for the involved physician. Due to the often imprecise complaint of a weak immune system the primary task is the identification of patients with true immunodeficiency. Subsequently, the immune defect needs to be identified in collaboration with a center for immunodeficiency disorders. The diagnostic procedure is dependent on the pattern of infections and follows a defined series of steps. This procedure should prevent costly diagnostic evaluation when not indicated, and also prevent the delayed diagnosis of patients with manifest immunodeficiency disease.
成人原发性免疫缺陷病是罕见疾病,但常导致严重后果。因此,早期诊断至关重要。临床表现的多样性、免疫系统的复杂性以及新缺陷的不断发现,使其成为相关医生面临的一个困难领域。由于免疫系统虚弱的主诉往往不确切,首要任务是识别真正存在免疫缺陷的患者。随后,需要与免疫缺陷疾病中心合作识别免疫缺陷。诊断程序取决于感染模式,并遵循一系列明确的步骤。该程序应避免在不必要时进行昂贵的诊断评估,同时也应防止对患有明显免疫缺陷病的患者延迟诊断。