A Systematic Review of the Progression of Cutaneous Lupus to Systemic Lupus Erythematosus

Abstract

Lupus erythematosus is an autoimmune disease that may manifest in a variety of organs and tissues including the skin, kidney, brain, heart and lung. Many patients present with cutaneous lupus, where disease is often limited to the skin, but are at risk for developing systemic lupus. The objective of our present study is to perform a systematic review of studies that investigated patient cohorts and populations for the occurrence of cutaneous lupus progressing to systemic lupus. Inclusion criteria required that studies present longitudinal data of patients with limited cutaneous lupus erythematosus who were followed for development of systemic lupus erythematosus. Studies were excluded if patients had concurrent diagnosis of SLE, or if they failed to present longitudinal data. Medline and Embase were searched for English language studies using the Ovid platform. A total of 25 adult studies were identified, as well as 8 pediatric studies. The rate of cutaneous to systemic lupus progression ranged between 0% to 42% in the adult studies and 0% to 31% in the pediatric groups. The variability in these rates were due to differences in patient populations, study design, criteria used to diagnose systemic lupus, and follow-up time. Common risk factors associated with systemic lupus erythematosus development including having positive anti-nuclear antibodies, hematologic abnormalities, and higher number of lupus classification criteria at baseline. This study emphasizes the importance for providers to routinely monitor for systemic lupus in patients with cutaneous lupus.

Keywords: autoimmunity; cutaneous lupus erythematosus (CLE); progression; systematic review; systemic lupus erythematosus.