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Case Report

Wegener's Granulomatosis in Patients with Rheumatoid Arthritis

GLENN DOUGLAS, KRISTIN BIRD, PATRICK FLUME, RICHARD SILVER, and MARCY BOLSTER

ABSTRACT.

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. (J Rheumatol 2003;30:2064-9)

Key Indexing Terms:

RHEUMATOID ARTHRITIS
WEGENER'S GRANULOMATOSIS
RHEUMATOID FACTOR
ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES
PROTEINASE 3


From the Department of Internal Medicine, Divisions of Rheumatology and Pulmonary Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.

G.C. Douglas, MD, Clinical Fellow; K.S. Bird, MD, Clinical Fellow, Division of Rheumatology and Immunology; P.A. Flume, MD, Associate Professor, Pulmonary and Critical Care Medicine; R.M. Silver, MD, Director, Professor of Rheumatology and Immunology; M.B. Bolster, MD, Associate Professor, Division of Rheumatology and Immunology.

Address reprint requests to Dr. G.C. Douglas, Department of Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 912, PO Box 250637, Charleston, SC 29425. E-mail: douglagc@musc.edu

Submitted October 15, 2002; revision accepted February 21, 2003.




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