Wegener's granulomatosis in patients with rheumatoid arthritis.
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.


CONCLUSIONS
Although rare, WG may develop in patients with preexisting RA and may present with end-organ involvement.
Douglas
Glenn
G
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. douglagc@musc.edu
Bird
Kristin
K
Flume
Patrick
P
Silver
Richard
R
Bolster
Marcy
M
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D001707:Biopsy, Needle; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D013902:Radiography, Thoracic; D012217:Rheumatoid Factor / Q000032:analysis*; D018570:Risk Assessment; D012720:Severity of Illness Index
D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D012217:Rheumatoid Factor