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[Dr. Shapiro] This is Dr. Samantha Shapiro, executive editor of Harrison's Principles of Internal Medicine. Harrison's Podclass is brought to you by McGraw Hill's AccessMedicine, the online medical resource that delivers the latest trusted content from the best minds in medicine. And now, onto the episode.
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[Dr. Handy] Hi, everyone. Welcome back to Harrison's Podclass. We're your co-hosts. I'm Dr. Cathy Handy.
[Dr. Wiener] And I'm Dr. Charlie Weiner. And we're joining you from the Johns Hopkins School of Medicine.
[Dr. Handy] Welcome to episode 101, a 27-year-old woman with right-sided weakness.
[Dr. Wiener] Here's the patient again from season one and her problem. So the patient is a 27-year-old woman who's brought to the emergency department after recent delivery of a full-term infant three days ago. She was discharged home on day two, but over the last six hours, she's developed right arm and right leg weakness, as well as a left blue hand. Her physical examination in addition to those findings is notable for diffuse livedo reticularis, a right hemiparesis with diminished reflexes and a cold blue left hand with no palpable radial or ulnar pulse. Her brachial pulse is palpable on that side. She's awake and alert and appropriately scared. Her laboratories are notable for a white cell count of 10,200, a hematocrit of 35%, and a platelet count of 13,000. Her BUN is 36 and her creatinine is 2.3. Although this pregnancy was uneventful, the three prior pregnancies that she'd had resulted in early losses. A peripheral blood smear shows no evidence of schistocytes.
[Dr. Handy] I remember when we talked about this before.
[Dr. Wiener] Okay, but I'm going to change the question to kick off our discussion with Dr. Langford. So the question's going to ask in this case, which of the following is the most likely diagnosis? A. antiphospholipid antibody syndrome; B. autoimmune hemolytic anemia; C. HELLP or H-E-L-L-P syndrome; D. systemic lupus erythematosus; or E. thrombotic thrombocytopenic purpura.
[Dr. Handy] The answer, in this case, is A., antiphospholipid antibody syndrome. The patient has multiple clinical manifestations of arterial thrombosis in her hand and brain and likely had placental insufficiency in the three prior pregnancies. So all of that makes the diagnosis of antiphospholipid antibody syndrome the most likely.
[Dr. Wiener] Okay, well let's welcome back Dr. Carol Langford to the Podclass to help us with this case today, Cathy.
[Dr. Handy] Welcome back, Dr. Langford. As a reminder, Dr. Langford is a professor of medicine and director of the Center for Vasculitis Care and Research at Cleveland Clinic and has been named one of Cleveland's top doctors. So welcome.
[Dr. Langford] Thank you, it's a pleasure to be here.
[Dr. Handy] So getting back to the case, what are your thoughts on this patient?
[Dr. Langford] Yeah, this is certainly a very complex patient and when you're faced with someone like that, as you illustrated, it's very helpful to outline the features that are present as this may help lead to what the differential diagnosis would be. And in this setting again, and where we see clinical manifestations of arterial thrombus in the hand and brain, we're seeing features of thrombocytopenia and renal insufficiency in a pregnant woman, antiphospholipid syndrome does come very strongly in the differential. When we think about antiphospholipid syndrome, this can occur as a primary diagnosis or secondary to other causes. And one of the other diagnoses on the list was systemic lupus erythematosus. About 30% of patients with antiphospholipid syndrome can have associated lupus. And this is something that needs to be kept in mind. We don't have enough information yet from the case as far as information from serologies, urinalysis as far as providing guidance about whether lupus could be a consideration, and this should be kept in mind as we look further into her investigations.
[Dr. Wiener] Dr. Langford, talk a little bit more about pregnancy, does pregnancy change the clinical presentation of rheumatologic diseases? Does it make you more vulnerable, less vulnerable, or how does that fit into this case?
[Dr. Langford] So when we talk about pregnancy and rheumatic diseases, we need to look at what has been found in the literature from each of the individual diagnoses. So in the case of rheumatoid arthritis, there is the possibility that the rheumatoid arthritis could actually improve during pregnancy. In the case of lupus, we worry that this could worsen, particularly if pregnancy occurs at the time of active disease being present. So we need to look at facets as far as not only what the diagnosis is, but what the status of their disease is at the time of pregnancy.
[Dr. Handy] And how do you counsel patients with rheumatologic disease who want to pursue a pregnancy?
[Dr. Langford] I think it's very important when we see young women with a rheumatic disease to bring in the discussion about planning for pregnancy at a very early point. And so that when they identify that they would like to pursue a pregnancy, the different factors can be examined and discussed with them. The main area that I do focus on when I am working with a young woman with vasculitis is that we discuss about what has been the organ dysfunction that has occurred. So the pattern of organ involvement or the vessels that have been affected. So for example, a woman who has underlying renal insufficiency, that's something that should be taken into account, or maybe a woman who has had vessel stenoses involving the abdominal vessels. We do want to look at what is known about the risk of relapse of that disease and discuss what would need to happen if a relapse occurred. What would be the risks to the mother and the baby? And related to that would be about medications. What medications is the woman on at the time as far as are those contraindicated in pursuing a pregnancy, and would they need to be switched before conception? And similarly, if a relapse were to occur, what medications could we safely use in a setting of pregnancy during that time?
[Dr. Wiener] That's great. So in summary, the teaching points in this case are that the antiphospholipid syndrome may present as an acute stroke or an acute arteriovascular event. Importantly, the antiphospholipid syndrome can be primary or secondary, so think about other potential autoimmune illnesses as part of your evaluation. Pregnancy does influence the course of autoimmune diseases, but the effect may be variable and heightens the importance of counseling before pregnancy in vulnerable patients.
[Dr. Handy] And to learn more about this, you can check out Harrison's chapter on antiphospholipid syndrome.
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[Dr. Shapiro] Thanks for listening to Harrison's Podclass. You can listen to this episode and more on AccessMedicine.com, which includes the complete Harrison's Principles of Internal Medicine text, Harrison's review questions, which complement and expand upon the questions in this episode, and much more. AccessMedicine.com may already be available to you via your academic institution. Check it out.
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