Pusey, "Proteinase-3 antineutrophil cytoplasm antibody positivity in patients without primary systemic vasculitis
," Journal of Clinical Rheumatology, vol.
This cohort is part of the Diagnostic and Classification Criteria in Vasculitis Study (DCVAS), which aims to develop classification and diagnostic criteria for primary systemic vasculitis
. Fully 35% of patients had cutaneous manifestations of ANCAassociated vasculitis, including 47% of those with EGPA, 34% of those with GPA, and 28% of those with microscopic polyangiitis (MPA).
Segelmark, "Primary systemic vasculitis
with severe [alpha]1-antitrypsin deficiency revisited," Scandinavian Journal of Rheumatology, vol.
Primary systemic vasculitis encompasses a group of disorders characterized by inflammation and necrosis of blood vessels which leads to end-organ damage.
We identified a cohort of individuals diagnosed with primary systemic vasculitis, as well as individuals specifically having GPA and PAN, from the Quebec provincial billing and hospitalization databases over the period Jan 1, 1994-Dec 31, 2003.
626 patients diagnosed with primary systemic vasculitis were identified within the observation period, of which 19.7% had PAN and 23.8% had GPA, 52.4% had GCA, 2.9% had Kawasaki disease, and 1.3% had Takayasu arteritis.
Biologic therapy in primary systemic vasculitis
of the young.
MPO is the most frequently recognized antigen in P-ANCA and primary systemic vasculitis
Savage CO, Harper L, Adu D: Primary systemic vasculitis
. Lancet: 1997; 349:553-558.