Douglas Glenn, Bird Kristin, Flume Patrick, Silver Richard, Bolster Marcy
Department of Internal Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 912, PO Box 250637, Charleston, SC 29425, USA.
J Rheumatol. 2003 Sep;30(9):2064-9.
To describe 2 cases of coexisting rheumatoid arthritis (RA) and Wegener's granulomatosis (WG), and to summarize the clinical and serological data for all 6 patients reported in the English literature since 1966.
Medline review over a 35-year period (1966-2002) revealed 4 reported cases of RA associated with WG. Patients were diagnosed based on symptoms, radiographic changes, bronchoalveolar lavage fluid analysis, hematuria, serology, and biopsy. We describe 2 additional cases of WG developing in Caucasian women with RA. These are the first reported patients to possess positive antineutrophil cytoplasmic antibodies (ANCA) and autoantibodies to proteinase 3 (PR3).
All 6 cases of coexisting RA and WG were female. The diagnosis of RA preceded WG diagnosis in all cases; mean age at RA onset was 43.7 +/- 15.0 years, duration of RA prior to WG diagnosis 7.9 +/- 9.1 years. Clinical findings included erosive articular disease on radiographs (n = 4; 67%), positive rheumatoid factor (n = 6; 100%), upper respiratory involvement (n = 5; 83%), lower respiratory signs (n = 4; 67%), renal involvement (n = 2; 33%), and positive ANCA (n = 2/3; 67%). Five patients were treated with corticosteroids and cyclophosphamide, with clinical improvement.
Although rare, WG may develop in patients with preexisting RA and may present with end-organ involvement.
描述2例类风湿关节炎（RA）与韦格纳肉芽肿（WG）并存的病例，并总结自1966年以来英文文献中报道的所有6例患者的临床和血清学资料。
对35年期间（1966 - 2002年）的Medline进行检索，发现4例报道的RA合并WG病例。根据症状、影像学改变、支气管肺泡灌洗分析、血尿、血清学及活检对患者进行诊断。我们描述另外2例在患有RA的白人女性中发生的WG病例。这些是首次报道的抗中性粒细胞胞浆抗体（ANCA）和抗蛋白酶3（PR3）自身抗体呈阳性的患者。
所有6例RA与WG并存的病例均为女性。所有病例中RA的诊断均先于WG诊断；RA发病的平均年龄为43.7±15.0岁，WG诊断前RA的病程为7.9±9.1年。临床发现包括X线片显示侵蚀性关节疾病（n = 4；67%）、类风湿因子阳性（n = 6；100%）、上呼吸道受累（n = 5；83%）、下呼吸道体征（n = 4；67%）、肾脏受累（n = 2；33%）以及ANCA阳性（n = 2/3；67%）。5例患者接受了皮质类固醇和环磷酰胺治疗，临床症状改善。
尽管罕见，但WG可在已有RA的患者中发生，并可能出现终末器官受累。
