Ep 149: A 20-Year-Old with an Abnormal Chest X-Ray
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This is Katarina Heidhausen, Executive Editor of Harrison's Principles of Internal Medicine. Harrison's Pod Class is brought to you by McGraw-Hills Access Medicine, the online medical resource that delivers the latest content from the best minds in medicine. And now, on to the episode. Hi, everyone. Welcome back to Harrison's Pod Class. We're your co-hosts. I'm Dr. Kathy Handy. And I'm Dr. Charlie Wiener, and we're joining you from the Johns Hopkins School of Medicine. Welcome to today's episode about a 20-year-old with an abnormal chest X-ray.

Kathy, today's patient is a 20-year-old college student who comes in to see you for an abnormal chest x-ray performed at Student Health. She went there for 10 days of persistent non-productive cough, low-grade fevers, and mild dyspnea and exertion. She tested herself negative for COVID, RSV, and flu. Last night, she developed new painful red skin lesions on her shin.

She went to student health and they performed a chest x-ray that demonstrates clear lungs, a normal heart, but bilateral, hyalur, and right paratracheal adenopathy. Gosh, that can be scary. I'd be terrified that I had something like lymphoma or another malignancy. Exactly. She's terrified because she thinks she has cancer. Ken, I have more history first and then a better description, especially of the skin lesions.

How about the skin lesions?

On the anterior aspect of both lower legs, she has two to three 1 to 1.5 inch red to reddish nodules that are painful when you touch them. They all seem about similar in size and are reddest at the center. There's no fluctuants. The rest of her skin exam is entirely normal. As far as the rest of her physical examination is concerned, her vital signs are normal except she has a temperature of 100.5 Fahrenheit. The rest of her physical examination is normal, including her lungs and her other joints.

She has no palpable adenopathy in her neck, axilla, arms, or groin, and she has no splenometaly.

It actually sounds like she has erythema nodosum on her skin. So just to summarize, we have a woman with a recent onset illness that's characterized by respiratory symptoms, a chest x-ray with clear lungs, but bilateral symmetric mediastinal and paratracheal adenopathy, and erythema nodosum. What's our question asking? The question is asking, in this patient, which of the following statements is true?

Option A is a lymph node biopsy is likely to show caseating granulomas. B, her illness is uncommon in adults over the age of 55. C, it is reasonable to observe her with no therapy at this time. D, she likely has hypercalcemia. And E, the presence of erythema nodosum is a poor prognosis.

This patient is presenting with a typical presentation of acute sarcoidosis. In fact, she almost certainly has Lofgren syndrome. What is Lofgren syndrome? It's an acute presentation of sarcoidosis that typically presents in young people with acute onset erythema nodosum, bilateral hyaluronidopathy, and fever. It also often has migratory polyarthritis and uveitis. Okay, let's step back a little bit. Tell me about sarcoidosis.

Sure. Sarcoidosis is an acute and chronic inflammatory disease characterized by the presence of non-caseating granulomas. Wait, you said non-caseating granulomas. Option A mentions caseating granulomas, so I assume that is false. Yes. Caseating granulomas are typical of tuberculosis. These are different. Sorry for interrupting. Keep going.

So, it's a multi-system disease and it can affect virtually every organ of the body. The lung is most commonly affected, but other organs that you have to look for include the skin, the liver, eyes, heart, and even CNS. Do we know what causes sarcoidosis? No, we still do not. Despite years of investigations, the singular cause of sarcoidosis remains unknown.

Currently, the most likely explanation is it's a granulomatous inflammatory response to an infectious or non-infectious environmental agent in a genetically susceptible host. Now, of the infectious agents, Propionibacterium and Mycobacterial species have been associated, but there are also environmental associations. I think the best explanation at this point is that sarcoidosis is a particular host response to multiple potential agents.

Okay, well, what about age? Our patient's young, and option B says sarcoidosis is uncommon in those over 55. Is that true? No, that option is false also. Sarcoidosis often occurs in young, otherwise healthy adults like in our patient. It's uncommon to diagnose a disease in someone younger than 18. However, it's become clear that a second peak in incidence develops around age 60.

In a large US study, the median age of diagnosis was 55. So you have to also think about sarcoidosis in our older patients. Let me also say that option D is false. - You mean the hypercalcemia? I thought that was typical of sarcoidosis.

You're partly correct. Hypercalcemia or hypercalciuria occur in about 10% of sarcoidosis patients. It's more common in whites than African Americans. The granulomas are at fault because they may produce 125-dihydroxyvitamin D. Serum calcium should be determined as part of the initial evaluation of all sarcoidosis patients, and a repeat determination may be useful during the summer months with increased sun exposure. Okay, that leaves us option C and E. Which one of those is true?

While they're related, it turns out that Lofgren syndrome, which is characterized by the presence of the erythema nodosum, which portends a good prognosis, so much so that over 90% of patients with Lofgren syndrome have resolution without any therapy within two years. I would likely obtain a CT to better delineate the amnopathy. If there are no other abnormalities and no worrisome signs of lymphoma or infection, it's reasonable to follow this patient. What about her symptoms? Can we treat them?

Yes, you can use non-steroidals for the painful skin lesions. Colchicine is useful in patients with joint symptoms. There really is so much more about sarcoidosis we could talk about, but today's teaching points are that Lofkin syndrome presenting with erythema nodosum, bilateral hyaluronidinopathy, fever, and sometimes uveitis or arthritis is a subset of sarcoidosis. Lofkin syndrome has an outstanding prognosis and in most cases resolves spontaneously.

Also, remember that sarcoidosis is a multi-system disease that affects not only the young, but also older individuals. And you can find this question and other questions like it in Harrison's self-review. And you can read more about this topic in the Harrison's chapter on sarcoidosis.

Visit the show notes for links to helpful resources, including related chapters and review questions from Harrison's, available exclusively on Access Medicine. If you enjoyed this episode, please leave us a review so we can reach more listeners just like you. Thanks so much for listening.