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Type I Membranoproliferative Glomerulonephritis (MPGN)

by Carlo Raj, MD
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    00:00 Type I MPGN. Most common type it is.

    00:03 Nephrotic syndrome presentation, what does that mean to you? You begin with protein loss.

    00:09 And greater than 3.5 grams of protein per day.

    00:11 Hypoalbuminemia and generalized edema.

    00:15 You would have loss of antithrombin III, Anticoagulation, maybe a little bit of hypertension.

    00:20 That's your nephrotic presentation, isn't it? Some cases it will have nephritic.

    00:25 I told you earlier that MPGN and DPGN will have a little bit of nephritic, a little bit of nephrotic.

    00:33 Now, most of this with MPGN in fact, is nephrotic.

    00:36 Associated with hepatitis C, more common or cryoglobulinemia.

    00:42 What does that even mean? That means that you might have conditions and at some point in time, when you get into cryoglobulinemia, there are three different types.

    00:51 One, two, and three, cryoglobulinemia.

    00:52 The big ones.

    00:53 Type I associated with multi-myeloma.

    00:57 Type II and III, more or less associated with infections and autoimmune diseases.

    01:02 What do all of these have in common? Autoimmune diseases.

    01:05 Monoclonal gammopathies and hepatitis C could be put in there as well.

    01:10 They are associated with antigen-antibody complexes. Okay.

    01:19 Two components, circulating through the body.

    01:23 All of a sudden cryo.

    01:24 What does that mean? Cold exposure.

    01:29 The globulins will precipitate resulting in all kinds of issues in your patient.

    01:34 Mostly, what do you know this about Waldenstrom? In Waldenstrom, what then happens when you have your globulins that will precipitate in the blood? It’s almost like a thrombus, isn't it? And so therefore it’s hyperviscosity. Hyperviscosity.

    01:52 Cryoglobulinemia is a big deal, a big clinical condition or syndrome, and I've given you a few examples of type I, type II, type III.

    02:01 My point at this point though, what kind of MPGN are we dealing with? Type I.

    02:07 In the previous discussion what branch of our MPGN would that follow? Immunecomplex mediated or would it be complement-mediated? Good, immune complex mediated. Much more common, type I.

    02:23 Which type I? On your light microscopy, can't really tell the difference between type I and type II, that I can tell you for certain.

    02:31 However, where you can start seeing differences? When you hear at this point, it should be reflexive.

    02:39 When you say subendothelial immune complexes, what kind of biopsy is this? Good. Electron microscopy.

    02:46 If you see a granular pattern, what kind of biopsy is that? Good. Immunofluorescence.

    02:51 Aren’t you feeling good about this now? You're reading, as if you’re reading a clinical vignette.

    02:55 You're reading as if it was a medical journal.

    02:56 Lancet, New England’s Journal Of Medicine. Whatever it may be.

    02:59 And these are the kind of information that it has and you should be able to- These are the kind of questions, information that everyone expects you to know, at your level of medical education.

    03:11 The immune complexes activate classical and alternative complement pathway.

    03:16 You know that already.

    03:17 Electron microscopy shows tram-track.

    03:22 What’s that even mean? Tram-track means, the membranes being involved, membranoproliferative.

    03:28 The membranes been involve, what happens? Split of the glomerular basement membrane by ingrowth of mesangium.

    03:37 Tram-track, is that specific? No. It’s not.

    03:41 Anything that weakens that membrane, do you think that you would then have deposition of mesangium and splitting of it? Sure. Another good differential, Alport Syndrome.

    03:51 Hypertension, 35% of the majority of your patients will have hematuria.

    03:56 Remember, MPGN? Nephritic/Nephrotic presentation.

    03:59 Majority will progress to chronic renal failure.

    04:02 Which was most common, type I or type II? Type I.

    04:06 If it’s type I, what kind of association? Immune complexes.

    04:10 Examples, autoimmune disease, infections such as Hepatitis C and cryoglobulinemia.

    04:17 And we have monoclonal gammopathies, including Waldenstrom.

    04:21 With type I, response to corticosteroids not established, let it go.


    About the Lecture

    The lecture Type I Membranoproliferative Glomerulonephritis (MPGN) by Carlo Raj, MD is from the course Glomerulonephritis.


    Included Quiz Questions

    1. Subendothelial and mesangial immune complex and C3 deposits.
    2. IgA, IgG and C3 deposits in mesangium.
    3. No pattern on immunofluorescence.
    4. Linear pattern of immune complex deposition.
    5. Subendothelial immune complex deposits.
    1. Mesangial cells
    2. Parietal epithelial cells
    3. Fenestrated cells
    4. Endothelial cells
    5. Visceral epithelial cells
    1. All statements are correct.
    2. It most commonly has a nephrotic presentation but may also have a nephritic presentation.
    3. Immune complexes may activate the classical complement pathway, the alternative complement pathway, or both.
    4. It may be associated cryglobulinemia.
    5. It is the most common type of MPGN.
    1. Large amount of protein usually immunoglobulins that precipitate at reduced temperatures.
    2. Immunoglobulins that cause agglutination of red blood cells.
    3. It is a type of hypoviscosity syndrome.
    4. It is often associated with bacterial infections.
    5. It often leads to type II membranoproliferative glomerulonephritis.

    Author of lecture Type I Membranoproliferative Glomerulonephritis (MPGN)

     Carlo Raj, MD

    Carlo Raj, MD


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