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Sarcoidosis: Diagnosis & Treatment

by Carlo Raj, MD
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    00:00 Sarcoidosis, well you need, well you need to do biopsy in which tissues are almost always required Now, I told you about that hypertension, what the heck is going on To understand, lung normally houses an enzyme called ACE, doesn't it? In sarcoidosis, you might find an increased serum level of your ACE Next, there may be hypercalcemia Every once in a while with sarcoid, you might have a patient that has an increased increased level of what's known as 1-alpha hydroxylase Dr. Raj, i think I've heard that enzyme, of course you have.

    00:37 That 1-alpha hydroxylase activates Vitamin D, welcome to calcitriol And that calcitriol is going to do what? reabsorption, reabsorption, reabsorption of calcium welcome to hypercalcemia.

    00:47 I'll tell you what you're gonna find in tissues soon enough.

    00:50 The pulmonary function test will be restrictive, That's where we are. So we have a PFT definitely not less than 80 We have adenopathy, interstitial infiltrate, what does that mean to you? This then represents your reticular pattern and the caviterial disease in severe type of cases And looking for what is known as a non-caseating type of granuloma and within the non-caseating granuloma, what are you then going to find? You're going to find, you HAVE to find what's known as multinucleated giant cells In multinucleated giant cell, you then refer to it as being Langhan's cell Also referred to as epithelioid, I will then go into this in further detail And in granuloma formation, we'll take a look at this as well in which you will then find a particular pattern where for the most part, think of it as being imprisonment of whatever antigen that might be giving rise to this What we'll find here, on this CT, on the right side an arrow that's then pointing to a lymphadenopathy What we're looking at here upon your biopsy of the tissue, that arrow is showing you a horseshoe pattern of a multinucleated giant cell In other words, these are fused macrophages.

    02:08 Now, this is a non-caseating granuloma if you take a look at the middle of this granuloma. This whole thing that you're seeing is a granuloma.

    02:17 Inside the granuloma, you'd find an epithelioid cell.

    02:20 An epithelioid cell is a multinucleated giant cell, welcome to Langhan.

    02:25 a horseshoe type of pattern that you see here, peripheral nuclei.

    02:28 And well, what is it that the T-helper1 cell is releasing that then activates your macrophage? Good, Interferon-Gamma, you learned that in immunology.

    02:39 That IFN-gamma is also called the macrphage activating factor So if you have so much of this MAF or IFN-gamma, you're going to take these macrophages, fuse them and form a multinucleated giant cell.

    02:53 You must, I repeat, you must find at least 3 of these multinucleated giant cell or epithelioid cell in order for you to diagnose or describe a granuloma Well, what about your patient? Asymptomatic- observe.

    03:12 In terms of therapy, well kinda difficult.

    03:14 Patients with advanced disease are treated with systemic corticosteroids And then for those individuals that are refractory such cases, then you start getting into immunosuppressants You have such drugs such as methotrexate, leflunamide, azathioprine and anti-TNF therapy With anti-TNF therapy, you're always worried about what? Good, you're worried about those patients that may then have what is known as reactivation TB Think of kenercept, think of infliximab reserved for patients with persistent disease.

    03:49 So you really wanna be careful about when to use such immunosuppresive agents Really, the complications from the medications could be worse than the actual disease as you know..


    About the Lecture

    The lecture Sarcoidosis: Diagnosis & Treatment by Carlo Raj, MD is from the course Restrictive Lung Disease.


    Included Quiz Questions

    1. All of the answers would be expected to be elevated.
    2. Serum phosphate levels.
    3. Vitamin D levels.
    4. Serum calcium levels.
    5. Serum ACE levels.
    1. At least 3 multinucleated giant cells must be found with negative cultures.
    2. At least 1 multinucleated giant cell with regions of friable cellular debris and necrosis.
    3. At least 3 multinucleated giant cells with regions of friable cellular debris and regions with acellular ghost cells.
    4. At least 1 multinucleated giant cell infiltrating a preformed cavitary space.
    5. At least 3 multinucleated giant cells surrounding angle branching, septate hyphae.
    1. Asymptomatic patients should be treated with corticosteroids to avoid progression of disease.
    2. Patients diagnosed incidentally by CXR should be observed.
    3. Many patients’ conditions improve spontaneously.
    4. Persistent disease that is steroid refractory is an indication for anti-TNF therapy.
    5. Symptomatic disease is often treated with systemic corticosteroids.

    Author of lecture Sarcoidosis: Diagnosis & Treatment

     Carlo Raj, MD

    Carlo Raj, MD


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