Researchers pool experience to examine discoid lupus diagnosis, outcomes in children

Lisa M. Arkin, MD
Lisa M. Arkin

Significant differences in perceived risk factors for systemic disease along with other substantial differences exist in screening and treating children with discoid lupus erythematosus within and across dermatology and rheumatology, Lisa M. Arkin, MD, and colleagues found.

The online survey of dermatologists and rheumatologists managing patients with systemic lupus erythematosus revealed areas of consensus in laboratory studies and also some practice-based differences. Read more on the study here.

Arkin, who is assistant professor in the department of dermatology and pediatrics at the School of Medicine and Public Health at University of Wisconsin-Madison, spoke with Healio Dermatology about the study and a multicentered cohort that is in the works with the Pediatric Dermatology Research Alliance (PeDRA) and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to further uncover this rare condition. – by Abigail Sutton

 

What do you want dermatologists to learn from your study?

We did this study because the quality of evidence to guide clinicians taking care of children with discoid lupus is low. These are kids who present with skin findings without feeling sick or having evidence of end organ disease that would classify them as systemic lupus erythematosus (SLE). Even when discoid lupus is limited to the skin, it is a diagnosis that carries a high risk for permanent scarring, and an uncertain risk for the development of systemic lupus, which is associated with significant morbidity/mortality in kids. But it’s a rare disease, so we don’t have a lot of information to help quantify those risks or how best to treat it.

We surveyed two groups of academic pediatric subspecialists — dermatologists and rheumatologists — who both care for these kids — to see if we could highlight areas of agreement on management and areas of difference. We used a modified Delphi technique to achieve consensus, which set greater than 70% agreement between specialties as the standard.

The study showed agreement from rheum and derm about what labs to order at diagnosis for a pediatric patient with discoid lupus erythematosus (DLE). Both groups agreed that hydroxychloroquine should be used first line as a systemic medication. But there wasn’t any agreement about whether there were baseline features (like family history, ethnicity or age) that might increase your risk for developing SLE or change the clinician’s screening strategy for it.

There was consensus that patients who developed arthritis or kidney involvement (nephritis) were at higher risk for developing SLE, and this should intensify surveillance. This isn’t surprising since both arthritis and nephritis are part of the classification criteria for SLE. And there was absolutely no consensus on what to do next in terms of management for patients whose skin disease was refractory to hydroxychloroquine. Dermatologists and rheumatologists favored different agents, and even within specialties there wasn’t greater than 70% agreement in using one agent over another.

In many ways, the study revealed more questions about this diagnosis than it provided answers. The take-away is that we need more information about the natural history of this diagnosis in kids before we can make definitive conclusions. The holy grail would be to identify subsets of patients (like those at high risk for scarring or high risk for SLE) that would enable us to choose the best treatment to optimize outcomes from the start.

 

Did any of the findings surprise you?

I was surprised that most of these experienced clinicians who answered the survey had treated fewer than 10 patients with this diagnosis in their entire career. This speaks to the rarity of this condition, and underscores why it’s hard to agree on the best thing to do for these patients. We just don’t know enough based on personal experience. It’s why we undertook this multicenter retrospective cohort study, to pool our experience and see if we can help to draw conclusions about the natural history/outcomes in this condition.

 

Can you describe the multicentered PeDRA -CARRA cohort study and what you hope it will accomplish?

This is a study that just completed data collection. We retrospectively reviewed the records of patients with discoid lupus at 18 North American academic sites to evaluate outcomes in these patients and look at the natural history of their disease. We collected clinical and serologic data retrospectively at all the follow-up visits in the medical record to answer some of the questions that our survey study had generated. Our primary outcome was to evaluate the incidence of SLE in patients who had just skin findings of DLE at diagnosis. We also looked at baseline and evolving risk factors for SLE to see if we could identify those patients at highest risk for poor outcomes from the start. We have over 440 patients in this registry and are finishing the analysis. We hope it will provide a lot more information about this rare condition.

 

Disclosures: The authors report support from CARRA and the ongoing support of CARRA through the Arthritis Foundation.

 

Lisa M. Arkin, MD
Lisa M. Arkin

Significant differences in perceived risk factors for systemic disease along with other substantial differences exist in screening and treating children with discoid lupus erythematosus within and across dermatology and rheumatology, Lisa M. Arkin, MD, and colleagues found.

The online survey of dermatologists and rheumatologists managing patients with systemic lupus erythematosus revealed areas of consensus in laboratory studies and also some practice-based differences. Read more on the study here.

Arkin, who is assistant professor in the department of dermatology and pediatrics at the School of Medicine and Public Health at University of Wisconsin-Madison, spoke with Healio Dermatology about the study and a multicentered cohort that is in the works with the Pediatric Dermatology Research Alliance (PeDRA) and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to further uncover this rare condition. – by Abigail Sutton

 

What do you want dermatologists to learn from your study?

We did this study because the quality of evidence to guide clinicians taking care of children with discoid lupus is low. These are kids who present with skin findings without feeling sick or having evidence of end organ disease that would classify them as systemic lupus erythematosus (SLE). Even when discoid lupus is limited to the skin, it is a diagnosis that carries a high risk for permanent scarring, and an uncertain risk for the development of systemic lupus, which is associated with significant morbidity/mortality in kids. But it’s a rare disease, so we don’t have a lot of information to help quantify those risks or how best to treat it.

We surveyed two groups of academic pediatric subspecialists — dermatologists and rheumatologists — who both care for these kids — to see if we could highlight areas of agreement on management and areas of difference. We used a modified Delphi technique to achieve consensus, which set greater than 70% agreement between specialties as the standard.

The study showed agreement from rheum and derm about what labs to order at diagnosis for a pediatric patient with discoid lupus erythematosus (DLE). Both groups agreed that hydroxychloroquine should be used first line as a systemic medication. But there wasn’t any agreement about whether there were baseline features (like family history, ethnicity or age) that might increase your risk for developing SLE or change the clinician’s screening strategy for it.

There was consensus that patients who developed arthritis or kidney involvement (nephritis) were at higher risk for developing SLE, and this should intensify surveillance. This isn’t surprising since both arthritis and nephritis are part of the classification criteria for SLE. And there was absolutely no consensus on what to do next in terms of management for patients whose skin disease was refractory to hydroxychloroquine. Dermatologists and rheumatologists favored different agents, and even within specialties there wasn’t greater than 70% agreement in using one agent over another.

In many ways, the study revealed more questions about this diagnosis than it provided answers. The take-away is that we need more information about the natural history of this diagnosis in kids before we can make definitive conclusions. The holy grail would be to identify subsets of patients (like those at high risk for scarring or high risk for SLE) that would enable us to choose the best treatment to optimize outcomes from the start.

 

Did any of the findings surprise you?

I was surprised that most of these experienced clinicians who answered the survey had treated fewer than 10 patients with this diagnosis in their entire career. This speaks to the rarity of this condition, and underscores why it’s hard to agree on the best thing to do for these patients. We just don’t know enough based on personal experience. It’s why we undertook this multicenter retrospective cohort study, to pool our experience and see if we can help to draw conclusions about the natural history/outcomes in this condition.

 

Can you describe the multicentered PeDRA -CARRA cohort study and what you hope it will accomplish?

This is a study that just completed data collection. We retrospectively reviewed the records of patients with discoid lupus at 18 North American academic sites to evaluate outcomes in these patients and look at the natural history of their disease. We collected clinical and serologic data retrospectively at all the follow-up visits in the medical record to answer some of the questions that our survey study had generated. Our primary outcome was to evaluate the incidence of SLE in patients who had just skin findings of DLE at diagnosis. We also looked at baseline and evolving risk factors for SLE to see if we could identify those patients at highest risk for poor outcomes from the start. We have over 440 patients in this registry and are finishing the analysis. We hope it will provide a lot more information about this rare condition.

 

Disclosures: The authors report support from CARRA and the ongoing support of CARRA through the Arthritis Foundation.