Vasculitis in systemic lupus erythematosus

Abstract

We studied the frequency, location, clinical and histopathological features, associated manifestations, and prognosis of vasculitides in a cohort of 667 SLE patients. Exclusion of patients with previous vasculitis or insufficient information left 540 patients, 194 of whom has vasculitis (incidence density: 0.053 new cases/person/year, cumulative incidence 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 five, 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, visceral in 24, both in 10. In the first episode of cutaneous vasculitides, 111 had punctuate lesions, 32 palpable purpura, 6 urticaria, 6 ulcers, 8 papules, 5 erythematous plaques or macules confirmed with biopsy, 2 erythema with necrosis, and 1 panniculitis (plus small vessel vasculitis). 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 vasculitis had longer disease duration and followup, younger age of onset of SLE, and were more frequently males than those without. Lupus manifestations associated with vasculitis in univariate logistic regression included myocarditis, psychosis, Raynaud's phenomenon, serositis, leukopenia, lymphopenia and pleuritis. Vasculitis also associated with the antiphospholipid syndrome. The strength of this association increased when patients with vasculitis confirmed by biopsy and/or arteriography were considered separately. Visceral vasculitis associated with increased mortality when controlled for age of onset and nephropathy.

A 26‐year‐old white man with a systemic lupus erythematosus flare and acute multiorgan ischemia: Vasculitis or thrombosis?

“…Pulmonary capillaritis with alveolar hemorrhage is a rare but severe manifestation of the disease. Excluding these disorders, vasculitis was noted in approximately 11-36% of SLE patients in two large retrospective studies, and mainly affected the small vessels of the skin (7,8). The most common skin lesions were erythematous punctate lesions on the fingertips and palms (such as in this case), followed by palpable purpura.…”

“…The most common skin lesions were erythematous punctate lesions on the fingertips and palms (such as in this case), followed by palpable purpura. Histologically, leukocytoclastic followed by lymphocytic vasculitis were the most common pathologies (8). Medium-vessel vasculitis comprised 14 -15% of all vasculitis cases and presented mainly with mononeuritis multiplex, and less commonly with digital necrosis and visceral involvement, with necrotizing vasculitis of the vessel wall on histology.…”

“…There was overlap between the 2 entities. Lupus vasculitis appears to be associated with livedo reticularis, high disease activity, antiphospholipid antibodies (aPL), high ESR, hypocomplementemia, and anti-La, among other manifestations (7,8). Small/medium-size vasculitis has rarely been reported in the kidneys of patients with lupus nephritis and morphologically resembles microscopic polyangiitis (9).…”

Gastrointestinal Manifestations of Systemic Lupus Erythematosus and Scleroderma

“…Intestinal vasculitis has been reported to be 0.2% in a study by Drenkard et al of 540 SLE patients. 8 Other studies have shown an incidence rate of up to 53%. A recent chart review study of 175 patients by C-K Lee et al showed that vasculitis was the most common cause of acute abdominal pain in SLE patients (found in 45% of patients).…”

Digital Ulcer as a Rare Complication of Systemic Lupus Erythematosus: A Case Report

“…3 Several risk factors have been identified with digital ulcers in SLE, including duration of lupus, male gender, atherosclerosis, vasculitis, and AAS. 4 Liu et al 5 demonstrated that duration of disease, the presence of Raynaud's phenomenon, and high serum CRP levels were determinants of digital ulcer development in a cohort of 2,600 lupus patients. When we looked at the risk factors in our patient, she had a 10-year history of lupus, but her disease activity was under control with small oral doses of corticosteroids.…”