Lupus Vasculitis: An Overview

Abstract

Lupus vasculitis (LV) is one of the secondary vasculitides occurring in the setting of systemic lupus erythematosus (SLE) in approximately 50% of patients. It is most commonly associated with small vessels, but medium-sized vessels can also be affected, whereas large vessel involvement is very rare. LV may involve different organ systems and present in a wide variety of clinical manifestations according to the size and site of the vessels involved. LV usually portends a poor prognosis, and a prompt diagnosis is fundamental for a good outcome. The spectrum of involvement ranges from a relatively mild disease affecting small vessels or a single organ to a multiorgan system disease with life-threatening manifestations, such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex. Treatment depends upon the organs involved and the severity of the vasculitis process. In this review, we provide an overview of the different forms of LV, describing their clinical impact and focusing on the available treatment strategies.

Keywords: lupus vasculitis; small vessel vasculitis; systemic lupus erythematosus; vasculitis.

Figure 1

Pathogenesis of lupus vasculitis. Abbreviations: SLE = systemic lupus erythematosus, Ag = antigen, Ab = antibody, IC = immune complex, ICAM-1 = intercellular adhesion molecule 1, VCAM-1 = vascular cell adhesion molecule 1, IL = interleukin, and PMN = polymorphonuclear cell.

Figure 2

Necrotic purpuric plaques with ulcerations.

Figure 3

Deep leg ulcer.

Figure 4

Skin biopsies showing leukocytoclastic vasculitis in SLE patients. The inflammatory lesions are evident in the perivascular derma, associated with nuclear dust and subepithelial myxoid degeneration. (A,B) original magnification: 40×, scale bar, 50 µm and (C,D) original magnification: 100×, scale bar, 25 µm.

Figure 5

Diffuse alveolar hemorrhage. A chest radiography shows bilateral widespread infiltrates in both medium lower fields. A chest computed tomography shows left pulmonary embolism and massive diffuse infiltration of both lung fields. R means right.