Classifying Lupus: A Spectrum Disorder

Fotios Koumpouras, MD, FACR: Welcome to this Rheumatology Network presentation titled, “Evolving Approaches to the Treatment of Systemic Lupus Erythematosus and Lupus Nephritis.” My name is Dr Fotios Koumpouras, and I’m the assistant professor of medicine and director of the lupus program at Yale University.

I’m joined today by Dr Ronald van Vollenhoven, who is the chair of the Department of Rheumatology and Clinical Immunology at the Amsterdam University Medical Center. Dr van Vollenhoven is also the director of the Amsterdam Rheumatology & Immunology Center.

Our discussion today will focus on the practical considerations and approaches in the diagnosis and management, particularly focusing on new advances in treatment targets and new therapeutic advances in care, for lupus and lupus nephritis. We hope that you’ll enjoy this discussion as we explore the current and emerging therapies within this arena.

I think the best place to start, Dr von Vollenhoven, is in the diagnosis, and making the diagnosis for patients with lupus, and an overview of what lupus disease is. I’ll start with you. Can you tell the audience what populations are at risk for developing lupus disease, particularly systemic lupus?

Ronald van Vollenhoven, Prof. PhD: Thank you, Fotios, for having me here, and it’s great to be able to discuss a disease where so much is happening. I’m very excited about some of the developments that we’ll be touching on a little later, but that is not really the diagnosis, because that’s still the same. This is a disease that, of course, was described more than 100 years ago. It has evolved. It turns out that it is a disease where there are lots of autoantibodies, the clinical manifestations can be very variable, very different from patient to patient. The ACR [American College of Rheumatology] has with criteria, which is very useful, and they are updated. We’re already getting into this millennium, and there’s another update. The SLICC [Systemic Lupus Erythematosus International Collaborating Clinics] group has some new criteria. The most recent thing is, of course, that the ACR and the European organization, EULAR [European Alliance of Associations for Rheumatology], working jointly, developed new criteria that are meant to classify lupus. They always emphasize that because it’s not really diagnostic criteria. I like to think that classification criteria, that’s the science of medicine, but diagnosis, that’s the art of medicine.

Fotios Koumpouras, MD, FACR: You’re exactly right. You initially stated that the signs and symptoms of lupus are quite variable, and lupus is a spectrum disease. We look at these clinical criteria to help (a) instruct rheumatologists about the diversity of clinical presentations that patients with lupus can have, and (b) to kind of clue them in when multisystem complaints might actually be a connective tissue disease. You’re right, there have been several criteria, different ways to help classify lupus, that have been evolving over the last several years. And I think for us as rheumatologists, it’s really important to understand that things we use daily, for example, complement levels were recently introduced in the 2012 SLICC criteria. Fever, which is a very common feature of connective tissue diseases, is now a part of the diagnostic 2019 criteria. I think as the criteria have evolved, they have encompassed the great spectrum of lupus disease.

Ronald van Vollenhoven, Prof. PhD: Clearly it has evolved, and it is better now than it was. But it’s still not perfect, and we still need the clinician. We need them to look at the patient and the totality of the data. I often compare it to when you play a piece of music, you can just play the notes, or you can play it with feeling, and you put your soul into it. When we deal with a patient, we have to sort of do the latter thing. We can look at the list of the symptoms and the manifestations and the tests, but we also have to use that feeling that we have as physicians. Don’t you agree?

Fotios Koumpouras, MD, FACR: Yes, and I think you’re touching on the intangibles of lupus and the limitation of scientific testing in these multisystem diseases. You’re right, if the number is normal, but the patient has clearly abnormal symptoms and signs, you should treat the patient, not the number. I think that is particularly true for patients with lupus who may be partly controlled on therapy.

Ronald van Vollenhoven, Prof. PhD: Right…I’m just thinking it can be confusing. These manifestations of lupus can happen over the course of time. They add up, and that is sometimes also made explicit in the criteria, that people may not have all the manifestations all at once, and it’s still that same disease.

Transcript Edited for Clarity