Lupus Vasculitis
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VASCULITIS (L ESPINOZA, SECTION EDITOR)
Lupus Vasculitis Estefania Calle-Botero 1 & Andy Abril 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review The purpose of this manuscript is to review the most recent literature pertinent to the presence of vasculitis in patients with systemic lupus erythematosus (SLE), including previously published landmark articles and studies and to update the different clinical and diagnostic aspects of vasculitic manifestations in patients with this important autoimmune disorder. As a multisystem autoimmune disease, systemic lupus may attack practically any organ system in the human body. Even though vasculitis is not the most common presentation or pathogenic mechanism of disease, it frequently causes significant morbidity and mortality in patients with SLE. The most common manifestation of lupus vasculitis is cutaneous involvement; visceral involvement is less common but causes severe disease; it may occur in different areas including central nervous system, peripheral nervous system, gastrointestinal system, kidneys, lungs and even retina. Recent Findings Recent findings regarding the pathogenesis of lupus CNS and peripheral nerve disease, and vascular injury in lupus nephritis are reviewed as well. Summary Vasculitis is an uncommon but serious manifestation of SLE; it may involve different organ systems and present in a wide variety of clinical syndromes, and thus the importance of its recognition and early diagnosis by physicians who deal with this disease, in order to start prompt and aggressive therapy when indicated. Keywords Systemic lupus erythematosus . Vasculitis . Lupus enteritis . Lupus vasculitis
Introduction Systemic lupus erythematosus (SLE) is the quintessential systemic autoimmune disorder, where autoimmune mechanisms such as auto antibody production lead to the potential attack of any organ system. It affects both genders and all races, being more common in females and minority group such as African Americans, Native Americans, and Hispanics [1]. Involvement of the blood vessels by systemic lupus has been recognized, and even though it is not the most common manifestation, it could lead to serious morbidity and mortality. Prevalence fluctuates between 11 and 35.9% [2], even though This article is part of the Topical Collection on Vasculitis * Andy Abril [email protected] Estefania Calle-Botero [email protected] 1
Clinica SOMER, Calle 38 #No. 54A-35, Rionegro, Antioquia, Colombia
2
Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
large studies and cohorts addressing specifically vasculitis in lupus are very few. It predominantly involves small vessels; medium-sized vessels can also be affected, and large vessel involvement is very rare [3]. Lupus patients with vasculitis tend to have longer disease duration and SLE starting at younger ages than those without vasculitis [4]. Ninety percent of the cases present as cutaneous vasculitis but visceral involvement can also occur, mainly in the form of
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