Neves Inês, Marinho Anabela, Melo Natália, Jesus José Miguel, Moura Conceição Souto, Bernardes Miguel, Vaz Carlos
Acta Reumatol Port. 2013 Oct-Dec;38(4):295-8.
The authors present a clinical case of 55 years old female patient with limited form of Wegener's granulomatosis (WG), which first manifestation was non-erosive polyarthritis with rheumatoid factor positive that antedates one decade the pulmonary manifestations. She had acute episodes with purulent expectoration, fever and hemoptysis, with resolution in a week. The chest x-rays demonstrated migratory bilateral pulmonary infiltrates. Transthoracic lung biopsy was performed and revealed capilaritis and signs of old and recent hemorrhage. At that time, autoimmunity study was repeated and showed positive for rheumatoid factor, negative anti-cyclic citrullinated peptide antibodies (anti-CCP) and high sustained PR3 anti-neutrophil cytoplasmic antibodies. The diagnosis of WG was established and cyclophosphamide started. This patient had a less common presentation and a less common histological pattern compared to the typical necrotizing granulomatous inflammation. She was treated with immunosuppression therapy which could have contributed to a mild clinical expression and a lower diagnostic yield. In suspicious cases, repeat the autoimmunity study, when facing new findings, could confirm the correct diagnosis.
作者报告了一例55岁女性韦格纳肉芽肿（WG）局限型患者的临床病例，其首发表现为类风湿因子阳性的非侵蚀性多关节炎，早于肺部表现十年出现。她有脓性咳痰、发热和咯血的急性发作，一周后缓解。胸部X线显示双侧肺部游走性浸润。进行了经胸肺活检，显示有毛细血管炎以及陈旧性和近期出血迹象。当时重复进行了自身免疫研究，结果显示类风湿因子阳性、抗环瓜氨酸肽抗体（抗CCP）阴性以及PR3抗中性粒细胞胞浆抗体持续高水平。确诊为WG并开始使用环磷酰胺治疗。与典型的坏死性肉芽肿性炎症相比，该患者的表现和组织学模式不太常见。她接受了免疫抑制治疗，这可能导致了临床症状较轻和诊断率较低。在可疑病例中，面对新发现时重复进行自身免疫研究可确诊。
