Flores-Suárez Luis Felipe, Alba Marco Antonio, Tona Gabriel
Instituto Nacional de Enfermedades Respiratorias, Ciudad Mexico , Mexico.
Colomb Med (Cali). 2017 Mar 30;48(1):32-34.
A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement.
Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis. Renal biopsy confirmed an ANCA-associated vasculitis. She was diagnosed with microscopic polyangiitis.
High-dose glucocorticoids, intravenous cyclophosphamide, plasma exchange and finally, hemodialysis and renal transplantation.
In contrast to granulomatosis with polyangiitis (Wegener), ear, nose and throat manifestations in microscopic polyangiitis are uncommon, while involvement of the lungs and kidneys are usual. We present a case with an isolated rare involvement, which progressed to severe disease. This atypical case warns about laryngeal symptoms as initial manifestation of an anti-myeloperoxidase positive systemic vasculitides, and emphasizes the relevance of close observation when unexplained isolated conditions with accompanying evidence of autoimmunity, in this case high levels of specific autoantibodies, are present.
一名16岁女性，最初表现为耳鼻喉症状，随后发展为严重肾病，在独特的喉部受累11个月后需要进行血液透析。
单侧声带麻痹，无其他症状或体征，但核周抗中性粒细胞胞浆抗体（ANCA）和抗髓过氧化物酶自身抗体呈阳性，数月后病情发展不利，出现快速进展性肾小球肾炎。肾活检证实为ANCA相关性血管炎。她被诊断为显微镜下多血管炎。
大剂量糖皮质激素、静脉注射环磷酰胺、血浆置换，最终进行血液透析和肾移植。
与肉芽肿性多血管炎（韦格纳肉芽肿）不同，显微镜下多血管炎的耳鼻喉表现并不常见，而肺部和肾脏受累则较为常见。我们报告了一例罕见的孤立受累病例，该病例发展为严重疾病。这个非典型病例警示了喉部症状作为抗髓过氧化物酶阳性系统性血管炎的初始表现，并强调了在出现无法解释的孤立病症且伴有自身免疫证据（在本病例中为高水平的特异性自身抗体）时密切观察的重要性。
