Mubashir Eisha, Ahmed M Mubashir, Hayat Samina, Latif Shahnila, Heldmann Maureen, Berney Seth Mark
Center of Excellence for Arthritis and Rheumatology, Section of Rheumatology, Department of Medicine, Louisiana State University Health Sciences Center, Shreveport, LA, USA.
South Med J. 2006 Sep;99(9):977-88. doi: 10.1097/01.smj.0000232210.04881.d3.
Wegener granulomatosis (WG) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a triad involving the upper airways, lungs and kidneys. Ninety percent of patients present with symptoms involving the upper and/or lower airways, and 80% will eventually develop renal disease. WG should be suspected in any patient with progressive or unresponsive sinus disease, glomerulonephritis, pulmonary hemorrhage, mononeuritis multiplex or unexplained multisystem disease. Before the routine use of glucocorticoids and cyclophosphamide, the one year mortality was 82%. However in 1973, Fauci and Wolf discovered that daily prednisone and cyclophosphamide induced complete remission in 75% of patients. The continued use of prednisone and cyclophosphamide for 1 year past remission leads to marked improvement in more than 90% of patients; however, is also associated with serious toxicities. Depending on the disease severity, current treatments employ induction with short-term cyclophosphamide followed by less toxic agents such as methotrexate to maintain disease remission. Although it is a rare disorder, it is pertinent to internists because it is a multisystem disease that presents in a variety of ways. We describe a 63-year-old white male with WG who presented with progressively worsening headaches, bilateral eye redness, epistaxis, hemoptysis and an unintentional 20 pound weight loss, and review the current treatment recommendations.
韦格纳肉芽肿病（WG）是一种病因不明的全身性疾病，其特征为坏死性肉芽肿性炎症、组织坏死以及中小血管不同程度的血管炎。典型的临床症状为三联征，累及上呼吸道、肺部和肾脏。90%的患者出现上呼吸道和/或下呼吸道症状，80%最终会发展为肾脏疾病。对于任何患有进行性或难治性鼻窦疾病、肾小球肾炎、肺出血、多发性单神经炎或不明原因的多系统疾病的患者，均应怀疑患有韦格纳肉芽肿病。在常规使用糖皮质激素和环磷酰胺之前，一年死亡率为82%。然而在1973年，福西和沃尔夫发现每日使用泼尼松和环磷酰胺可使75%的患者完全缓解。在缓解后继续使用泼尼松和环磷酰胺1年可使90%以上的患者有显著改善；然而，这也会带来严重的毒性。根据疾病严重程度，目前的治疗方法是先用短期环磷酰胺诱导，然后使用毒性较小的药物如甲氨蝶呤来维持疾病缓解。尽管这是一种罕见疾病，但对内科医生来说很重要，因为它是一种以多种方式呈现的多系统疾病。我们描述了一名63岁患有韦格纳肉芽肿病的白人男性，他出现了逐渐加重的头痛、双眼发红、鼻出血、咯血以及意外减重20磅的症状，并回顾了当前的治疗建议。
