Gutierrez Paulo Sampaio, Aiello Vera Demarchi
Laboratory of Pathology - Instituto do Coração - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.
Autops Case Rep. 2014 Mar 31;4(1):7-14. doi: 10.4322/acr.2014.002. eCollection 2014 Jan-Mar.
Microscopic polyangiitis (MPA) is part of the anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis, which usually presents as renal pulmonary syndrome. It is defined as a pauci-immune necrotizing small vessel vasculitis, which usually affects the kidneys, followed by the lungs. It also presents systemic symptoms. The etiology of MPA is still unclear, but evidence reinforces the autoimmune mechanisms as the main etiopathogenic factor. Aortic valve stenosis (AS) is not an uncommon disease whose etiology varies according to geographical differences and the patient's age. The natural history of AS begins with a prolonged asymptomatic period, but when symptomatic, respiratory failure is one of its main clinical presentations. The authors present the case of a 55-year-old woman who was admitted with the diagnosis of renal failure, anemia, and a cardiac murmur. The patient had been recently diagnosed with pneumonia. During hospitalization, diagnostic workup disclosed a normal kidney size as well as parenchymal thickness. A renal biopsy was undertaken but the specimen was exiguous, showing 4 sclerotic glomeruli and 1 glomerulus with crescentic glomerulonephritis. The search for ANCA was positive. The investigation of the cardiac murmur disclosed AS. The patient, on hemodialysis, presented episodes of respiratory failure, which was interpreted as acute pulmonary edema, but a suspicion of ANCA-related pulmonary renal syndrome was raised. However, the aortic valve replacement was prioritized. While awaiting cardiac surgery, the patient died because of respiratory insufficiency. Autopsy findings concluded that MPA with pulmonary hemorrhage due to vasculitis was the immediate cause of death. Although AS was present at autopsy and classified as moderate/severe, this lesion was a bystander in the process of this patient's end of life, demonstrating the value of autopsy for medical learning and reasoning purposes.
显微镜下多血管炎（MPA）是抗中性粒细胞胞浆抗体（ANCA）相关血管炎的一部分，通常表现为肾肺综合征。它被定义为寡免疫坏死性小血管炎，通常累及肾脏，其次是肺部。它也会出现全身症状。MPA的病因仍不清楚，但有证据支持自身免疫机制是主要的致病因素。主动脉瓣狭窄（AS）是一种并不罕见的疾病，其病因因地域差异和患者年龄而异。AS的自然病程始于较长的无症状期，但出现症状时，呼吸衰竭是其主要临床表现之一。作者报告了一例55岁女性患者，因肾衰竭、贫血和心脏杂音入院。该患者最近被诊断为肺炎。住院期间，诊断检查显示肾脏大小及实质厚度正常。进行了肾活检，但标本很少，显示4个硬化性肾小球和1个新月体性肾小球肾炎的肾小球。ANCA检测呈阳性。心脏杂音检查发现有AS。该患者接受血液透析，出现呼吸衰竭发作，最初被解释为急性肺水肿，但也怀疑与ANCA相关的肺肾综合征有关。然而，优先考虑进行主动脉瓣置换。在等待心脏手术期间，患者因呼吸功能不全死亡。尸检结果得出结论，血管炎导致的伴有肺出血的MPA是直接死因。尽管尸检发现存在AS并分类为中度/重度，但该病变在该患者生命终结过程中是一个旁观者，这表明尸检对于医学学习和推理的价值。
