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Clinical Decision Making: Evaluating Biologics in Lupus Management - Episode 2

Lupus Clinical Presentation and Manifestations

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Kristi V. Mizelle, MD, MPH, FACR, provides an overview of the clinical presentations and manifestations of both systemic lupus erythematosus and lupus nephritis.

Anne E. Winkler, MD, PhD, MACP: When we think about lupus we know it can present in a wide range of ways. I had one patient who presented with upper airway stridor years ago. But when we think about the more characteristic presentations for lupus patients, what would you say are the most common things that we see?

Kristi V. Mizelle, MD, MPH, FACR: The most common manifestations of lupus are going to be cutaneous and joint or musculoskeletal. And those are the things that are probably going to be the bread and butter for most lupus patients to some extent as far as manifestations. But lupus can affect pretty much every organ system in the body and we have to then be looking out for multiple organ system involvement. And that's the nature of lupus is that it is a multi-system disease. And we have to be detectives when we're thinking about whether a patient has lupus or not because of the various protean manifestations. And as I mentioned, those commonly cutaneous and musculoskeletal manifestations but of course, constitutional symptoms also which are very hard sometimes to pick up on. If someone just says they're fatigued, they kind of feel achy and their muscles or their joints, even fever. And those are the constitutional symptoms are very nonspecific. And they often will coexist with other more specific lupus manifestations and that it can include renal manifestations which is one of the most common internal organ involvement sort of areas with SLE. And then there's also some more less common things like pulmonary involvement which still is not uncommon, but it still does happen. As well as some of the things that we really worry about like CNS or neurologic manifestations of lupus. There are a number of different manifestations including even [gastrointestinal] I haven't seen it often, but I have a couple of patients who have severe GI manifestations related to lupus as well as cardiac vascular manifestations with our good old friend, raynaud's phenomenon and livedo reticularis. But then also obstetric manifestations we must be careful and be aware of in those patients because they're very vulnerable and may have more lupus activity during pregnancy. But then also rare endocrine manifestations and of course hematologic manifestations we can't forget, it's very common to have cytokines in patients who do have lupus.

Anne E. Winkler, MD, PhD, MACP: You mentioned again lupus nephritis which we think about as one of the most serious consequences of lupus and perhaps one of the more common organs that can be seriously involved in terms of lupus. What are the typical presentations for patients who have lupus nephritis? And what are the characteristics of those patients that make you more concerned that they may develop lupus nephritis?

Kristi V. Mizelle, MD, MPH, FACR: As you said, lupus nephritis is not uncommon and it's more prevalent than even many rheumatologists think about. They estimate that it is at about 50% of patients who do have lupus or systemic lupus erythematosus will actually have lupus nephritis of some type. And when you think about as a rheumatologist your patient population with lupus, you don't think of them as 50% as having lupus nephritis. Looking at post-mortem studies etcetera, it's very common even if it's mild lupus nephritis it still can be present. That's something that I would want to potentially raise awareness of even among our colleagues as far as lupus nephritis being something that's common and that we should have a high suspicion for with our patients. As far as clinical disease and lupus, a new lupus nephritis presentation, most commonly we think of things like elevated blood pressure, swelling in the particularly the lower extremities. And those are probably 2 of the most common. Maybe seeing patients may report foamy or bubbly urine, occasionally they may also complain of hematuria but a gross hematuria is not that common it's more often a microscopic hematuria. Those are the most common manifestations. If you think about it a little bit of elevated blood pressure a little bit of swollen legs, we may not have our antenna up to sense that that may be something we need to investigate further. We want to make sure that we're screening our patients quite frequently for a new onset lupus nephritis. The earlier it's found the more likely we're able to potentially prevent it from progressing to end-stage renal disease which can be an unfortunate outcome with either severe lupus nephritis or lupus nephritis with delayed diagnosis. And then patients unfortunately don't have appropriate renal function and have to go on dialysis.

Anne E. Winkler, MD, PhD, MACP: That brings up the EULAR 2019 guidelines in terms of lupus nephritis. As they are now recommending that for those high-risk patients, that we should be screening them every 3 months with UPCR as well as blood pressure. And I'm not sure all of us have thought about doing that in the past until we've been more aware that lupus nephritis is common. And as you said, they may have symptoms, but they may be mild and some people are totally asymptomatic. And there's some theoretical data suggesting that one episode of lupus nephritis can wipe out 30% of nephrons. And again, it's somewhat theoretical, they mostly talk about class 4. But again, if we can catch them early, we can probably make a significant difference in terms of their life down the road because we can hopefully prevent the onset of renal failure.

Transcript Edited for Clarity