Jerrentrup A, Müller T, Mariss J, Dylla S
Zentrum für Unerkannte und Seltene Erkrankungen, Universitätsklinikum Gießen und Marburg, Standort Marburg, Baldingerstr., 35043, Marburg, Deutschland.
MVZ für diagnostische und interventionelle Radiologie und Neuroradiologie, RadMedics GmbH, Radiologie Nordhessen, Standort Frankenberg, Frankenberg (Eder), Deutschland.
Internist (Berl). 2018 May;59(5):497-504. doi: 10.1007/s00108-017-0303-3.
This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years. Although EGPA belongs to the spectrum of ANCA-associated vasculitis, less than 50% of EGPA patients are ANCA positive. Cardiac involvement (such as endomyocardial infiltration, arrhythmia and pericarditis) is the major cause of early death and a poor prognosis. In therapeutic regimens glucocorticoids and/or another immunosuppressant (e. g. cyclophosphamide, methotrexate or azathioprine) are used. The so-called five-factor score is a useful tool for assessment of prognosis.
本文介绍了一名患有呼吸困难的患者的病例，该患者有哮喘控制不佳、鼻息肉病、血液嗜酸性粒细胞增多和短暂性肺部浸润病史。抗核抗体、胞浆抗中性粒细胞胞浆抗体（c-ANCA）和核周抗中性粒细胞胞浆抗体（p-ANCA）自身抗体均为阴性。这些症状是嗜酸性肉芽肿性多血管炎（EGPA，又称Churg-Strauss综合征）的典型表现。该病例从发病到诊断的延迟为数年。虽然EGPA属于抗中性粒细胞胞浆抗体相关血管炎范畴，但不到50%的EGPA患者抗中性粒细胞胞浆抗体呈阳性。心脏受累（如心肌内膜浸润、心律失常和心包炎）是早期死亡和预后不良的主要原因。在治疗方案中，使用糖皮质激素和/或另一种免疫抑制剂（如环磷酰胺、甲氨蝶呤或硫唑嘌呤）。所谓的五因素评分是评估预后的有用工具。
