Flossmann Oliver, Jayne David R W
Vasculitis and Lupus Unit, Addenbrooke's Hospital, Cambridge, UK.
Nat Clin Pract Rheumatol. 2008 Sep;4(9):499-504. doi: 10.1038/ncprheum0863. Epub 2008 Jul 22.
A 40-year-old man was referred to a specialist vasculitis center 4 years after being diagnosed with Wegener's granulomatosis. At the time of diagnosis he had presented with skin, ear, nose and throat involvement, pulmonary hemorrhage, and microscopic hematuria. Remission was achieved with plasma exchange and with daily oral prednisolone and cyclophosphamide. The patient was switched to maintenance treatment with azathioprine and prednisolone but suffered a relapse shortly afterwards. Further treatment with cyclophosphamide achieved a second remission, but the patient relapsed again despite remission-maintaining treatment with mycophenolate mofetil.
Physical examination, laboratory testing, serological testing, culture of eye swabs and sputum, chest X-ray, chest CT scan, head MRI scan, bronchoscopy and bronchoalveolar lavage, and consultation with ophthalmological and otorhinolaryngological specialists.
Refractory Wegener's granulomatosis with involvement of the eyes, upper and lower respiratory tracts, and kidneys.
Disease activity was controlled following treatment with deoxyspergualin and oral steroids in addition to aggressive management of intercurrent infections with repeated courses of oral and intravenous antibiotics. Relapses that occurred when deoxyspergualin was discontinued were treated with repeated courses of deoxyspergualin or with pulsed intravenous cyclophosphamide. Remission was achieved with rituximab. Pulmonary disease was closely monitored with repeated bronchoscopy.
一名40岁男性在被诊断为韦格纳肉芽肿4年后转诊至一家专业血管炎中心。诊断时，他出现了皮肤、耳、鼻、喉受累，肺出血和镜下血尿。通过血浆置换、每日口服泼尼松龙和环磷酰胺实现了缓解。患者改用硫唑嘌呤和泼尼松龙维持治疗，但不久后复发。再次使用环磷酰胺治疗实现了第二次缓解，但尽管使用霉酚酸酯维持缓解治疗，患者仍再次复发。
体格检查、实验室检查、血清学检查、眼拭子和痰液培养、胸部X线、胸部CT扫描、头部MRI扫描、支气管镜检查和支气管肺泡灌洗，以及咨询眼科和耳鼻喉科专家。
难治性韦格纳肉芽肿，累及眼、上呼吸道和下呼吸道以及肾脏。
除了积极处理并发感染（反复口服和静脉使用抗生素）外，使用去氧精胍菌素和口服类固醇治疗后疾病活动得到控制。停用去氧精胍菌素时发生的复发，采用反复使用去氧精胍菌素疗程或脉冲静脉输注环磷酰胺治疗。使用利妥昔单抗实现了缓解。通过反复支气管镜检查密切监测肺部疾病。
