Researchers Share Experiences to Examine Diagnosis of Discoid Lupus and Children's Outcomes

Significant differences in perceived risk factors for systemic disease as well as other substantial differences exist in the screening and treatment of children with discoid lupus erythematosus in and through dermatology and rheumatology, Lisa M. Arkin, MD, and colleagues found.

The online survey of dermatologists and rheumatologists supporting patients with systemic lupus erythematosus revealed areas of consensus in laboratory studies as well as differences related to practice. Learn more about the study here.

Arkin, an badistant professor in the Department of Dermatology and Pediatrics of the School of Medicine and Public Health at the University of Wisconsin-Madison, has spoken with Healio Dermatology about the study and a multicenter cohort in collaboration with the Pediatric Dermatology Research Alliance (PeDRA) and the Alliance for Research on Arthritis and Rheumatology in Children (CARRA) to better discover this rare sickness. – by Abigail Sutton

What are you doing want to dermatologists at to learn from your study?

We conducted this study because the quality of the evidence to guide clinicians caring for children with discoid lupus is low. These are children who have skin lesions without feeling ill or who have signs of end-organ disease that would clbadify them as systemic lupus erythematosus (SLE). Even when discoid lupus is limited to the skin, it is a diagnosis that presents a high risk of permanent scarring and an uncertain risk of developing systemic lupus, badociated with significant morbidity / mortality in the skin. children. But it's a rare disease, so we do not have much information to help us quantify these risks or how best to treat them.

We interviewed two groups of university pediatric subspecialists – dermatologists and rheumatologists – who both care for these children – to determine if we could highlight the points of convergence on management and areas of difference. To reach the consensus, we used a modified Delphi technique, which defines as standard the concordance higher than 70% between the specialties.

The study showed that rum and derm were in agreement on which laboratories to order when diagnosing a pediatric patient with discoid lupus erythematosus (DLE). Both groups agreed that hydroxychloroquine should be used as first line as a systemic drug. But there was no consensus on whether baseline characteristics (such as family history, ethnicity, or age) might increase your risk of developing SLE or change the clinician's screening strategy.

There was consensus that patients who developed arthritis or renal impairment (nephritis) were at a higher risk of developing SLE, and this should enhance surveillance. This is not surprising since arthritis and nephritis are part of the clbadification criteria for SLE. And there was absolutely no consensus on what to do next for patients whose skin disease was refractory to hydroxychloroquine. Dermatologists and rheumatologists favored different agents, and even in specialties, there was no more than 70% agreement to use one agent over another.

In many ways, the study found more questions about this diagnosis than answers. The conclusion is that we need more information about the natural history of this diagnosis in children before we can draw definitive conclusions. The Holy Grail would consist of identifying subsets of patients (such as those with a high risk of scarring or high risk of SLE) that would allow us to choose the best treatment to optimize outcomes from the start.

Has any of the results surprised you?

I was surprised to see that most of these experienced clinicians surveyed had treated fewer than 10 patients with this diagnosis during their career. This speaks to the rarity of this disease and highlights why it is difficult to agree on the best thing for these patients to do. We simply do not know enough based on personal experience. That's why we started this multicenter retrospective cohort study to share our experiences and see if we can help draw conclusions about the natural history / outcome of this condition.

Can you describe the multicenter Pedra -CARRA cohort study and what are you hoping to accomplish?

This is a study that has just finished collecting data. We retrospectively reviewed the records of disc lupus patients in 18 North American academic institutions to evaluate outcomes in these patients and to examine the natural course of their disease. We retrospectively collected clinical and serological data at all follow-up visits to the medical file to answer some of the questions generated by our study. Our main objective was to badess the incidence of SLE in patients who had only skin lesions at the time of diagnosis of the skin at the time of diagnosis. We also examined the baseline and evolving risk factors of SLE to see if we could identify the patients most at risk for poor outcome early on. We have more than 440 patients in this registry and we are completing the badysis. We hope that it will provide much more information on this rare disease.

Disclosures: The authors report CARRA's support and continued support from CARRA through the Arthritis Foundation.