The British Journal of Rheumatology, Vol 36, 1059-1066, Copyright © 1997 by British Society for Rheumatology
KA Boki, U Dafni, GA Karpouzas, C Papasteriades, AA Drosos and HM Moutsopoulos
The clinical spectrum and outcome of necrotizing vasculitis were evaluated
in a retrospective study in order to assess: (1) the clinical expression
and evolution of the disease; (2) the response to several therapeutic
approaches based on major events (organ involvement causing disability or
death); (3) the immunogenetic background of patients. Sixty-six Greek
patients fulfilling the ACR criteria for the diagnosis of vasculitis
entered the study. Thirty-seven were diagnosed with Wegener's
granulomatosis (WG), 22 with polyarteritis nodosa (PAN) and seven with
Churg-Strauss syndrome (CSS). The demographic characteristics of patients
with WG and PAN were similar. Cutaneous manifestations, gastrointestinal
and peripheral nervous system involvement occurred more often in patients
with PAN, whereas pulmonary and upper respiratory tract involvement, renal,
ear abnormalities and fever were more frequent in patients with WG. Muscle
weakness and asthma were found exclusively in patients with PAN and CSS,
respectively, while the presence of classic-antineutrophil cytoplasmic
antibodies (c-ANCA) characterized WG patients. Hepatitis B surface antigen
(HBsAg) was found in 22% of PAN patients. No significant differences were
detected when comparing the PAN and WG groups with respect to the first
major event (log-rank P = 0.50) with and without potential confounders
(age, gender, therapy or c-ANCA). For WG patients, a statistically
significant difference was found on different routes of administration of
cyclophosphamide (oral vs pulse) (P = 0.006). Regarding the HLA antigens,
an increased frequency of DR1 (26.9% vs 10.3%, P = 0.057) in WG and the
absence of DR3 in patients with PAN and CSS were noted. It appears that
although the immunogenetic background and the clinical expression of the
diseases differ, the response to treatment as well as the evolution and the
survival rate of these patients are similar in the two groups.
ORIGINAL PAPERS
Necrotizing vasculitis in Greece: clinical, immunological and immunogenetic aspects. A study of 66 patients
Department of Pathophysiology, School of Medicine, National University of Athens, Greece.
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