VASCULITIS IN SYSTEMIC LUPUS-ERYTHEMATOSUS

We studied the frequency, location, clinical and histopathological fea tures, associated manifestations, and prognosis of vasculitides in a c ohort of 667 SLE patients. Exclusion of patients with previous vasculi tis or insufficient information left 540 patients, 194 of whom had vas culitis (incidence density: 0.053 new cases/person/year, cumulative in cidence of 0.051 at one year, 0.232 at 5 years and 0.411 at 10 years). Vasculitis was confirmed by biopsy in 46 cases, by arteriography in f ive, and by both in three. A single episode of vasculitis occurred in 119 and two or more in 75 patients. Vasculitis was cutaneous in 160, v isceral in 24, both in 10. In the first episode of cutaneous vasculiti des, 111 had punctuate lesions, 32 palpable purpura, 6 urticaria, 6 ul cers, 8 papules, 5 erythematous plaques or macules confirmed with biop sy, 2 erythema with necrosis, and 1 panniculitis (plus small vessel va sculitis). Of 29 with visceral vasculitis in the first episode, 19 had mononeuritis multiplex, 5 digital necrosis, 3 large artery vasculitis of limbs, one mesenteric, and one coronary. More than one type could appear simultaneously or in subsequent episodes. Patients with vasculi tis had longer disease duration and followup, younger age of onset of SLE, and were more frequently males than those without. Lupus manifest ations associated with vasculitis in univariate logistic regression in cluded myocarditis, psychosis, Raynaud's phenomenon, serositis, leukop enia, lymphopenia and pleuritis. Vasculitis also associated with the a ntiphospholipid syndrome. The strength of this association increased w hen patients with vasculitis confirmed by biopsy and/or arteriography were considered separately. Visceral vasculitis associated with increa sed mortality when controlled for age of onset and nephropathy.