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.

The Rise and Rise of Lupus

“…In a systematic review, a broad range of progression rates from cutaneous lupus to SLE was reported, ranging from 0% to 42% in adult studies, and 0%–31% in pediatric studies. 90 Discoid lupus followed by subacute cutaneous lupus was the most common cutaneous lupus subtypes studied. Time to progression was informed inconsistently among studies and ranged from 5.6 months to 8.2 years in adult studies.…”

“…Time to progression was informed inconsistently among studies and ranged from 5.6 months to 8.2 years in adult studies. 90 Rates of progression may vary according to the classification criteria used for SLE. For example, in a single center, historical, medical records review study of 93 patients with cutaneous lupus, 10.8% progressed to SLE under the SLICC criteria, whereas 16.1% did so under the ACR criteria.…”

“…91 The most common risk factors associated with progression to SLE were positive ANA, hematologic abnormalities, lupus-specific antibodies (e.g., anti-dsDNA), and higher number of lupus classification criteria at baseline. 90…”

Clinical aspects of cutaneous lupus erythematosus

“…The risk for progression to SLE in DLE patients is estimated between 5 and 30%. The generalized form of DLE has a higher potential of progressing to SLE compared to those with localized lesions (12,(19)(20)(21)(22). Potential risk factors for progression to systemic disease are suggested to be anemia, arthritis and positivity for ANA (19).…”

Influence of Socio-Demographic Factors in Patients With Cutaneous Lupus Erythematosus

“…Cutaneous lupus erythematosus (CLE) is an autoimmune disorder associated with a broad spectrum of cutaneous manifestations. It may progress into systemic lupus erythematosus (SLE) and involve multiple organ systems ( 1 ). CLE can be further classified into acute, subacute, or chronic subtypes according to the clinical characteristics of the skin lesions ( 2 ).…”