Lupus Case Scenario: Patient History and Presentation

Anne E. Winkler, MD, PhD, MACP: Let’s go ahead and switch gears a little bit and start talking about a hypothetical case. And even though it’s hypothetical, I will tell you I’ve had patients just like this. So, this is a 24-year-old Afro American computer programmer. She was referred to rheumatologist for a sensitive rash and joint pain. On exam, she had a rash in the Malar regions of the face and forearms, she was tendering in some of the small joints of the hands and the wrist. Normal blood pressure, labs did show however a low C3 of 63, elevated double stranded DNA antibody of over 300, her UPCR was 0.2 and she was initially treated with hydroxychloroquine and leflunomide. Again, pretty typical kind of scenario we would start. She comes back and every three months she’s getting UPCR which of course, we’re big on these days because we know already because young woman of color, low C3, high double stranded DNA antibody, she’s at high risk for lupus nephritis and on a routine visit, because again, I’ve seen this happen, no other symptoms that maybe some fatigue. Her UPCR now is four, so obviously she’s developed lupus nephritis. Kind of if you can comment a little bit on what you think and how you would approach this patient.

Kristi V. Mizelle, MD, MPH, FACR: Yeah, I’d like to actually take it back to the initial presentation. So when I see a patient, and the concern is potentially for lupus, most- a lot of people ask, well, what’s the ANA? That’s the first thing we want to know. And yes, the ANA is important. But I’d also want to get a little bit more of a history, I’d also want to see what the examination findings are. These are very important things because there’s some things that can mimic manifestations of lupus that we have to make sure that we are clearly seeing things that look like lupus. For example, patient presents with malar rash, OK, a lot of people have rosacea, a lot of rosacea is called malar rash. So it’s very important to understand the difference between rosacea and malar rash, looking for if there’s fine capillaries present? Does it spare the nasal labial folds? Is it raised, is it painful, or uncomfortable on the face? Those things are very important because we may be chasing something that’s not necessarily a Lupus manifestation. And so it’s important to distinguish those things when we do see patients. For example, you mentioned she had some joint pain. Well, joint pain in and of itself is not enough to make a Lupus diagnosis and patients can have arthralgias, but diagnostic and classification criteria actually speak to inflammatory arthritis. Is there synovitis at the joints? When we examine the joints and do X-rays, is there erosion? If there’s erosion, then that makes us take a step back and say maybe this isn’t truly lupus because lupus very rarely, usually does not cause erosions at the joints. So, we have to make sure that we’re very clear in our clinical approach and that we’re very detail oriented as we take the history, because on its face when you give this presentation for an initial visit with the patient, oh, it’s like lupus, slam dunk. But I can’t tell you the number of patients who’ve come in and I’ve had similar findings, and I say, you know what? These things are not technically meeting what I would expect the criteria to be in order for us to say it’s truly a manifestation of lupus. And so we have to have our minds open. I’ve had patients come in with an ANA one 180, one to 160 and have joint inflammation and we check their inflammatory markers as well as their lupus and rheumatoid arthritis studies and they have a high CCP or they have discoid lupus that’s biopsy proven, but no ANA, and then they have inflammatory joint changes with erosions. I have some patients who have discoid lupus and an seropositive erosive rheumatoid arthritis. So, we have to make sure we understand the diagnosis because we have to- that then is so-

Anne E. Winkler, MD, PhD, MACP: You just froze again, you have a patient with seropositive, I think is where you stopped.

Kristi V. Mizelle, MD, MPH, FACR: Seropositive rheumatoid arthritis. We have to make sure that we’re very clear on our initial diagnosis before we start running and putting on treatments, because our treatment could directly flow from what the diagnosis is and we may treat or choose inappropriately as far as choice of drug if we don’t have the right diagnosis. When that patient presents to me, like you said, and I’d probe on some of those things that she kind of came in with. She’s got a very good history with UV light exposure, which can sometimes activate or be a trigger potentially for lupus. The history sounds like it could work, a young woman of reproductive age sounds like it could work, African American, increased likelihood, because African Americans have an increased risk of lupus as compared to other races and ethnicities. A lot of those things are suggestive, but we still have to make sure we do the work to understand clearly, do the various manifestations meet what we would expect as sort of the clinical and or laboratory findings that would speak more to a Lupus diagnosis? Getting the right diagnosis, super, super important. I can’t tell you how many patients I’ve seen who were evaluated by other rheumatologist and come in and I say, I want to start from the beginning. Let’s make sure we have the right diagnosis. We’re just going to go back and rethink it all. Let’s go get the old labs. Let’s go back and take the history. And then let’s come back and see are we arriving at the same point, same diagnosis? If we are, great. If we’re not, OK, well, what is it that we think is going on? And what would we do differently based upon that? It’s important to be a good diagnostician when we’re thinking about lupus patients and to take the time to be detailed. When you present that case, to me, the first thing is, let’s make sure we got the right diagnosis.

Transcript edited for clarity