El Aoud S, Tounsi H, Chaabène I, Ben Ammou B, Ben Ahmed I, Garbouj W, Bouzeidi K, Amri R
Service de médecine interne, hôpital universitaire Mohamed Taher Maamouri, Mrazka, 8000 Nabeul, Tunisie.
Service de médecine interne, hôpital universitaire Mohamed Taher Maamouri, Mrazka, 8000 Nabeul, Tunisie.
J Med Vasc. 2018 Sep;43(5):316-319. doi: 10.1016/j.jdmv.2018.06.001. Epub 2018 Jun 28.
Granulomatosis with polyangiitis is a systemic small-vessels vasculitis. It is characterized by a granulomatous inflammatory reaction around the arterial wall and is, in most cases, accompanied by the presence of cytoplasmic - type anti-neutrophil cytoplasmic antibodies (ANCA) with anti-proteinase 3 specificity. The predominant clinical presentation is frequent involvement of the upper and lower respiratory tract and the kidneys. This vasculitis is rarely revealed by pulmonary embolism. We report the observation of a 47-year-old male who presented with acute dyspnea. Chest X-ray disclosed an excavated opacity. Pulmonary angioscan revealed bilateral pulmonary embolism. The absence of clinical improvement after anticoagulant therapy with antibiotics and the presence of extra-respiratory signs led to the suspicion of a systemic inflammatory disease. The etiological investigations concluded with the diagnosis of granulomatosis with polyangiitis.
肉芽肿性多血管炎是一种系统性小血管血管炎。其特征是动脉壁周围出现肉芽肿性炎症反应,在大多数情况下,伴有具有抗蛋白酶3特异性的胞质型抗中性粒细胞胞质抗体(ANCA)。主要临床表现为上、下呼吸道和肾脏频繁受累。这种血管炎很少由肺栓塞引起。我们报告了一名47岁男性患者,他表现为急性呼吸困难。胸部X线显示有空洞性混浊。肺部血管造影显示双侧肺栓塞。抗生素抗凝治疗后临床症状无改善且存在呼吸道外体征,导致怀疑为系统性炎症性疾病。病因学检查最终诊断为肉芽肿性多血管炎。