抗中性粒细胞胞浆抗体阴性的肉芽肿性多血管炎：一项疑难诊断。
ANCA-negative Granulomatosis with Polyangiitis: A Difficult Diagnosis.
作者信息
McCarthy Eilís, Mustafa Muneeb, Watts Mike
机构信息
Department of General Internal Medicine, University Hospital Limerick, Limerick, Ireland.
出版信息
Eur J Case Rep Intern Med. 2017 Jun 18;4(8):000625. doi: 10.12890/2017_000625. eCollection 2017.
UNLABELLED
Granulomatosis with polyangitiis (GPA) is a systemic small and medium vessel vasculitis, commonly associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Presenting signs and symptoms in GPA are varied and patients may present with constitutional, non-specific symptoms, which can delay the diagnosis. Tissue biopsy of the site of active disease can confirm the diagnosis of GPA, in which necrotising granulomatous inflammation is seen. However, surrogate markers may be used for diagnosis without a tissue biopsy. They include upper and lower airway symptoms, signs of glomerulonephritis and a positive ANCA. However, approximately 10-20% of patients with GPA are ANCA negative, allowing for the diagnosis to be overlooked, particularly in those patients with non-specific findings. The reason for the absence of ANCAs is unclear.
LEARNING POINTS
A case of granulomatosis with polyangitiis presenting with non-specific findings is described.Some 10% of cases are ANCA negative.When a vasculitis is suspected, a negative ANCA does not exclude the diagnosis of GPA, so further investigations (i.e. tissue biopsy) should be considered.
未标记
肉芽肿性多血管炎（GPA）是一种系统性中小血管炎，通常与抗中性粒细胞胞浆抗体（ANCA）相关。GPA的临床表现多样，患者可能出现全身性、非特异性症状，这可能会延迟诊断。对活动性病变部位进行组织活检可确诊GPA，活检可见坏死性肉芽肿性炎症。然而，在未进行组织活检的情况下，替代标志物可用于诊断。这些标志物包括上、下呼吸道症状、肾小球肾炎体征和ANCA阳性。然而，约10%-20%的GPA患者ANCA阴性，这可能导致诊断被忽视，尤其是在那些有非特异性表现的患者中。ANCA缺失的原因尚不清楚。
学习要点
描述了一例以非特异性表现就诊的肉芽肿性多血管炎病例。约10%的病例ANCA阴性。当怀疑血管炎时，ANCA阴性不能排除GPA的诊断，因此应考虑进一步检查（即组织活检）。
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