Kukushev Georgi, Kalinova Desislava, Sheytanov Ivan, Rashkov Rasho
Department of Otolaryngology, Military Medical Academy, Sofia, Bulgaria.
MHAT "St. Ivan Rilski", Clinic of Rheumatology, Medical University, Sofia, Bulgaria.
Reumatologia. 2017;55(5):256-260. doi: 10.5114/reum.2017.71643. Epub 2017 Oct 28.
Granulomatosis with polyangiitis (GPA) is characterised by granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract, often associated with pauci-immune glomerulonephritis. The diagnosis of granulomatosis with polyangiitis is made according to the classification criteria of the ACR criteria for granulomatosis with polyangiitis. We present two cases of granulomatosis with polyangiitis limited/localised form. The common feature between two clinical cases were not sufficient criteria for a definite diagnosis at the beginning. In both cases the clinical presence was otitis media with acute mastoiditis, peripheral facial nerve palsy, and severe headache. Early diagnosis and treatment of patients with granulomatosis with polyangiitis define favourable prognosis. On the other hand, the treatment of granulomatosis with polyangiitis (corticosteroids and immunosuppressive therapy) has various side effects, and the "ex juvantibus" therapy is hazardous.
肉芽肿性多血管炎（GPA）的特征是上、下呼吸道的肉芽肿性坏死性炎症病变，常伴有寡免疫性肾小球肾炎。肉芽肿性多血管炎的诊断依据美国风湿病学会（ACR）肉芽肿性多血管炎分类标准进行。我们报告两例肉芽肿性多血管炎局限型病例。这两例临床病例最初的共同特征并不足以明确诊断。两例患者的临床表现均为中耳炎伴急性乳突炎、周围性面神经麻痹和严重头痛。早期诊断和治疗肉芽肿性多血管炎患者可获得良好预后。另一方面，肉芽肿性多血管炎的治疗（皮质类固醇和免疫抑制疗法）有多种副作用，且“根据疗效推断病因”的疗法存在风险。
