Kleymann J, Schütz C, Körholz J, Taube F, Vogler M, Halank M, Kolditz M, Langner S, Geberzahn L, Holotiuk O, Roesler J, Koschel D
Medizinische Klinik 1, Bereich Pneumologie, Universitätsklinikum Carl Gustav Carus, Dresden.
Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Carl Gustav Carus, Dresden.
Pneumologie. 2020 Oct;74(10):678-683. doi: 10.1055/a-1175-4122. Epub 2020 Jul 8.
Chronic granulomatous disease (CGD) should be considered as a differential diagnosis in children and adolescents with frequent infections, especially when caused by certain specific pathogens.This case report describes a 64-year-old female with multiple recurrent and complicated bronchopulmonary infections, caused by common, but also rare pathogens, autoimmune phenomena, malignancies and recurrent organizing pneumonia (OP) with granulomas. Finally, the patient was diagnosed with p47-deficient chronic granulomatous disease (CGD).Individuals with a primary immunodeficiency may survive multiple complications and may be diagnosed at an advanced age especially if the affected structure shows residual activity. When confronted with patients with recurrent bronchopulmonary infections, especially with certain specific rare pathogens, in combination with organizing pulmonary granulomas as well as autoimmune phenomena, CGD should be considered even in elderly patients. Delayed diagnosis significantly increases mortality and morbidity in such cases.
慢性肉芽肿病（CGD）应被视为儿童和青少年频繁感染的鉴别诊断，尤其是由某些特定病原体引起的感染时。本病例报告描述了一名64岁女性，患有多种复发性和复杂性支气管肺部感染，由常见病原体以及罕见病原体引起，伴有自身免疫现象、恶性肿瘤和伴有肉芽肿的复发性机化性肺炎（OP）。最终，该患者被诊断为p47缺陷型慢性肉芽肿病（CGD）。原发性免疫缺陷患者可能在经历多种并发症后存活，并且可能在高龄时才被诊断出来，特别是如果受影响的结构仍有残余活性。当面对复发性支气管肺部感染的患者，尤其是伴有某些特定罕见病原体，同时伴有肺机化性肉芽肿以及自身免疫现象时，即使是老年患者也应考虑慢性肉芽肿病。在这种情况下，延迟诊断会显著增加死亡率和发病率。
