TY - JOUR T1 - Wegener's granulomatosis in patients with rheumatoid arthritis. JF - The Journal of Rheumatology JO - J Rheumatol SP - 2064 LP - 2069 VL - 30 IS - 9 AU - Glenn Douglas AU - Kristin Bird AU - Patrick Flume AU - Richard Silver AU - Marcy Bolster Y1 - 2003/09/01 UR - http://www.jrheum.org/content/30/9/2064.abstract N2 - OBJECTIVE: 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. METHODS: 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). RESULTS: 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. CONCLUSION: Although rare, WG may develop in patients with preexisting RA and may present with end-organ involvement. ER -