New insights into the progression from cutaneous lupus to systemic lupus erythematosus

ABSTRACT

Introduction

Between 5 and 25% of patients with cutaneous lupus erythematosus (CLE) can progress to systemic lupus erythematosus (SLE) during the course of the disease. There is no clear predictive guideline for the progression of CLE to SLE.

Areas covered

Lupus erythematosus (LE), a chronic autoimmune disease, has a wide spectrum of manifestations. On one side of the spectrum is CLE, in which patients only display skin lesions. On the other side of the spectrum is SLE, which develops severe systemic involvement. CLE has even been considered as a separate entity from LE, while CLE is also proposed to be associated with SLE. In this review, the authors will describe the relationship between CLE and SLE; summarize the incidence, risk factors, systemic involvement, and management of patients who transition to SLE. The literature search was conducted mainly through PubMed from March to July 2020.

Expert opinion

The identification of clinical characteristics and biomarkers in patients facing risk of developing SLE and monitoring the disease on a regular basis are essential to promptly manage and hopefully prevent transition to the systemic form.

KEYWORDS:

• Cutaneous lupus erythematosus
• discoid lupus erythematosus
• disease progression
• subacute lupus erythematosus
• systemic lupus erythematosus

Article highlights

• CLE can present not only as a separate disease but also in relation to SLE.

• There are three main forms of CLE: one presenting as a localized entity, one as CLE with SLE or a manifestation within the scope of SLE, and another presents as CLE progressing to SLE later in life.

• Between 5 and 25% of patients with CLE can progress to SLE during the course of the disease and up to 50–60% of patients with SCLE will transition to SLE.

• The majority of CLE patients who develop SLE may have mild systemic involvement with primarily mucocutaneous and musculoskeletal manifestations and elevated ANA titers.

• Whether early use of antimalarial drugs can prevent high-risk CLE patients from progressing to SLE is still controversial.

• Periodic follow-up is vital for patients with CLE to monitor for systemic involvement and disease progression.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was funded by National Natural Science Foundation of China [No. 81972943, No.81830097], Hunan Talent Young Investigator [No. 2019RS2012], and CAMS Innovation Fund for Medical Sciences (CIFMS) [2019-I2M-5-033].