Goodpasture's Syndrome

by Carlo Raj, MD

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    00:01 Goodpasture, quickly walk through, and let’s take a look at the presentations.

    00:06 First, antibody affecting your basement membrane.

    00:10 Two systems, kidney, we’ve done plenty resulting in hematuria.

    00:16 Lungs, basement membrane. Pulmonary alveolar basement membrane.

    00:22 What are you coughing up? Blood, hemoptysis, hematuria.

    00:29 Clear? Give me differential? Hematuria, hemoptysis, the other big one, formerly called Wegener.

    00:36 One more time. Broken record.

    00:38 You're gonna tell me, granulomatosis with polyangiitis, GPA.

    00:44 How can you tell the difference? You will clearly be given information further.

    00:49 With granulomatosis with polyangiitis, you will have nasal issues, upper respiratory tract maybe recurrent sinusitis.

    00:59 Our topic at hand is Goodpasture. Linear formation.

    01:04 Clinically young, male, 20s.

    01:08 Nephritic, look for hematuria, look for hypertension, look for hemoptysis, may progress to RPGN.

    01:18 Goodpasture, male dominant, HLA-BR2, I would memorize the genes.

    01:24 Type 2, linear immunofluorescence begins with hemoptysis, ends with renal failure, that’s unfortunate.

    01:31 Try to prevent this from occurring.

    01:34 What do you wanna do here? Well, this patient has a type 2 hypersensitivity.

    01:40 Plasma exchange immunosuppressive therapy with corticosteroids, cyclophosphamide of course, these themselves have a host to side effects, don’t they? And if you're worried about your patient going into renal failure within 12 weeks, then you're thinking about renal transplant.

    01:56 Easier said than done, isn't it? This is immunofluorescence.

    02:02 What you're seeing here is a ribbon.

    02:04 Have you ever seen a parade? And as you see a parade go by the one that I like quite a bit is Thanksgiving.

    02:11 The Macy’s Day Parade.

    02:13 And you see all these floats going by and every ones in a while you get these band members, right? And these cheerleaders and such men and women and they have these things with little handle and they are waving the ribbons around.

    02:25 You’ll love medicine so much at some point that all you’ll ever see in life is always medicine.

    02:32 So whenever I see these cheerleaders throwing around the ribbons I think to myself linear immunofluorescence.

    02:38 Is that going to be as effective for you, I don’t know.

    02:41 But anyhow, here’s a linear pattern of immunofluorescence.

    02:45 It’s completely clear, it’s green, doesn’t they just look like entangled, entangled ribbons? The whole thing of glomerular basement membrane is filled with immunoglobulins.

    02:57 Not a single immune-complex.

    03:02 This is not granular, ladies and gentlemen. This is for your Goodpasture.

    03:07 Linear immunofluorescence is caused by deposition of IgG antibodies, hence you do not see the words complex and directed against your basement membrane.

    03:17 Either in the kidney or it would be up in your lungs. SLE.

    About the Lecture

    The lecture Goodpasture's Syndrome by Carlo Raj, MD is from the course Glomerulonephritis.

    Included Quiz Questions

    1. HLA-DR2
    2. HLA- B27
    3. HLA-DQ8
    4. HLA-B47
    5. HLA-DR3
    1. Sinusitis
    2. Hemoptysis
    3. Pneumonitis
    4. Acute renal failure
    5. Hematuria
    1. NSAIDs
    2. Plasma exchange
    3. Renal transplantation
    4. Cyclophosphamides
    5. Corticosteroids
    1. Plasma exchange in conjunction with corticosteroids and cyclophosphamide
    2. NSAIDs
    3. Antibiotics
    4. Lung transplantation
    5. Anti-histamines
    1. Pneumonitis with hemoptysis
    2. A male patient in mid-60s
    3. Nephrotic syndrome
    4. Sinusitis
    5. A female patient in mid-20s

    Author of lecture Goodpasture's Syndrome

     Carlo Raj, MD

    Carlo Raj, MD

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