von Itzstein Mitchell S, Abeykoon Jithma P, Summerfield Daniel D, Whitaker Jennifer A
School of Medicine, Griffith University, Gold Coast, Australia.
Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
BMJ Case Rep. 2017 Jul 19;2017:bcr-2017-220135. doi: 10.1136/bcr-2017-220135.
Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel. Biopsy of the skull base/clivus revealed necrotising granulomatous inflammation with focal vasculitis consistent with GPA, and multiple bone cultures were positive for This patient was diagnosed concurrently with GPA and skull base osteomyelitis. He was started on a 6-week course of cefepime intravenously and oral prednisone, with the plan to initiate rituximab infusion 2 weeks after initiation of antibiotic therapy.
肉芽肿性多血管炎（GPA）背景下的颅底骨髓炎罕见，且诊断面临重大挑战。我们报告一例65岁的白种男性，有类风湿关节炎病史，已停用免疫抑制剂18个月，出现慢性头痛2年、严重疲劳、鞍鼻畸形和20公斤非故意体重减轻。颌面CT显示广泛的鼻窦破坏性病变和颅底侵蚀性病变。实验室检查显示抗蛋白酶3抗体轻度升高，抗中性粒细胞胞浆抗体检测阴性。颅底/斜坡活检显示坏死性肉芽肿性炎症伴局灶性血管炎，符合GPA，多次骨培养阳性。该患者同时被诊断为GPA和颅底骨髓炎。开始静脉注射头孢吡肟和口服泼尼松6周疗程，并计划在抗生素治疗开始2周后开始输注利妥昔单抗。
