Impact of Diagnosis Delay on Long Term Lupus Outcomes

Anne E. Winkler, MD, PhD, MACP: Adding to that, we know that there's many of our patients who end up getting the delayed diagnosis before they get to us and get the diagnosis. Can you comment on what the problems are when patients with lupus have a delayed diagnosis in terms of long-term outcomes?

Kristi V. Mizelle, MD, MPH, FACR: There are a couple of things that lend itself to the delayed diagnosis. One is that there's not one clear laboratory or radiographic finding that definitively makes the diagnosis. Providers who are sending patients to rheumatologists for the diagnosis of lupus or potential lupus are not as comfortable and may not have their antenna up as much in thinking about that as a possible diagnosis. Sometimes there's a delay because patients are seeing other sub-specialists but not rheumatologists because there's a whole picture that's there. And the focus may be on one organ system manifestation instead of looking at the systemic manifestations in total to help think about is there an overarching diagnosis that might explain multiple clinical manifestations. That part of it is that it's a hard diagnosis to make, that the referrers may not always have it at the forefront of their minds. And then also sometimes people don't check that sort of ANA, they just may say, they're just fatigued. Some of the vagueness of the symptoms that may go along with lupus may lend itself to making it harder to make the diagnosis as well. And then once patients get to the rheumatologist it's not always a clear-cut diagnosis either. Then we have to go through our process of doing both the science part of the diagnosis but the art part of the diagnosed with history exam to put it all together to come up with that diagnosis.

Anne E. Winkler, MD, PhD, MACP: I also think part of the problem with the delay in diagnosis is that lot of us have not really realized until we looked at cohort studies. Is that 10 to 30% of patients within one year of diagnosis already have irreversible damage. And we don't think about that. And if we think about then at 5 years somewhere between 30 to 50% of patients based on cohort data also have permanent irreversible organ damage. And that's with the average age of patients entering the cohort studies of 30 and entering with lower 70% on moderately high doses of prednisone, 70 and a half mg or higher. When you think about that, maybe they were seeing someone else, was maybe on a lot of steroids, and finally gets into treatment that we need to be doing besides steroids. They may already have some irreversible damage.

Kristi V. Mizelle, MD, MPH, FACR: It speaks to the urgency of the lupus diagnosis because that's where it all begins. That's how we get to the place where we can help patients and begin treatments to try to prevent the irreversible damage that you brought up. And sometimes there's a lack of that urgency or lack of an understanding of how quickly damage can occur. And like you said, in the first 5 years of disease if they haven't had a diagnosis, we could have potentially prevented some organ damage, even in that short period of time. And often times when patients do have particularly lupus nephritis, they're going to have the lupus nephritis start within the first 5 years of their lupus diagnosis. And we have to have our antenna up or our clinical suspicion very high for patients who come in for evaluation and to be assertive and aggressive in evaluating patients. And that is somewhat on the rheumatologist as well in that we must see the urgency in it. It should be just as urgent in a new lupus evaluation as it is to see someone with rheumatoid arthritis with multiple joints that are inflamed and impacting their life such that they can't do their activities of daily living. We must see it as just as urgent. And when patients come in say for an example, with rheumatoid arthritis it's very severe. You see them present and say, I'm feeling horrible, I'm feeling terrible. They don't look good, but a lupus patient may not in your clinical examination look as bad but could have nearly as much of a risk of permanent damage as that patient who has hot swollen joints that are, that has rheumatoid arthritis. We have to have a greater understanding of the urgency of diagnosing these patients and being assertive and slash aggressive with treatment so that we prevent the damage. Because once that scope occurred, we cannot reverse it.

Anne E. Winkler, MD, PhD, MACP: I was just thinking the same thing is we take our RA patients but we don't always take our lupus patients seriously. So great point.

Transcript Edited for Clarity