Kokan Norio, Hosomi Yoshikazu, Inamoto Shinya, Ohnishi Kazuo, Tanimoto Hitoshi, Nibu Ken-ichi
Department of Otolaryngology, Kobe-Rosai, Hospital, Kobe, Japan.
Rheumatol Int. 2006 Aug;26(10):936-8. doi: 10.1007/s00296-006-0127-2. Epub 2006 Apr 20.
Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis affecting small vessels without necrotizing granulomatous inflammation and is commonly associated with necrotizing glomerulonephritis. Diagnosis is based on typical clinical features, the presence of antimyeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), and histopathologic findings. Cases of pathologically proven small-vessel vasculitis in nasal biopsy specimens are sparse. Here we report a patient with MPA that was histopathologically confirmed by nasal and paranasal biopsy. A 67-year-old man presented with fever and general fatigue. Laboratory examinations showed severe inflammation and acute progressive renal failure. The serum MPO-ANCA level was elevated. The patient also had nasal polyps that seemed to be nonspecific chronic sinusitis. To obtain a pathologic diagnosis, bilateral ethmoidectomy and nasal polypectomy were performed. Pathological findings revealed vasculitis of small vessels in the mucosal surface. MPA was diagnosed on the basis of clinical symptoms, elevated MPO-ANCA and the pathological findings of the nasal and paranasal surgical specimen.
显微镜下多血管炎(MPA)是一种累及小血管的系统性坏死性血管炎,无坏死性肉芽肿性炎症,通常与坏死性肾小球肾炎相关。诊断基于典型的临床特征、抗髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)的存在以及组织病理学发现。经病理证实的鼻活检标本中小血管血管炎的病例较为少见。在此,我们报告一例经鼻和鼻旁组织活检病理确诊的MPA患者。一名67岁男性出现发热和全身乏力。实验室检查显示严重炎症和急性进行性肾衰竭。血清MPO-ANCA水平升高。患者还患有鼻息肉,看似为非特异性慢性鼻窦炎。为获得病理诊断,进行了双侧筛窦切除术和鼻息肉切除术。病理结果显示黏膜表面小血管血管炎。根据临床症状、MPO-ANCA升高以及鼻和鼻旁手术标本的病理结果,诊断为MPA。