ANCA Associated Vasculitis

by Carlo Raj, MD

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    00:01 And this discussion takes us into what ANCA means? So what is ANCA? It stands for Anti-neutrophil cytoplasmic antibody.

    00:09 Stop. There are two types of ANCAs.

    00:14 Aren’t there? c-ANCA, p- ANCA.

    00:17 At some point during your medical education you've definitely been exposed to c-ANCA, now I have to take you a little bit further.

    00:24 I’ll tell you what does c and p stands for.

    00:26 Well, we said that c was the third letter of the alphabet: A, B, C.

    00:30 Therefore, anti-proteinase 3 ANCA.

    00:34 The p in p-ANCA is anti-myeloperoxidase ANCA process. Clear? Are we done? No. A wish.

    00:43 Here’s the p. The p in p-ANCA, apart from you should knowing that it’s myeloperoxidase means perinuclear.

    00:51 What the heck! Why do I have to know this, Doc Raj! Are you just actually vomiting information at us? No! I'm giving you information so that you can clinically differentiate; you get every single question right. And you're not thrown off.

    01:06 That p in p-ANCA is perinuclear.

    01:09 You will be responsible for identifying an ANCA, okay? You will be responsible for literally identifying on immunofluorescence, an anti-neutrophil cytoplasmic antibody.

    01:20 And when you do so, you have to be able to distinguish between a p, perinuclear versus c, cytoplasmic.

    01:28 What’s p mean? The perinuclear, around the nuclear.

    01:32 So here we have polyarteritis nodosa as being one possible differential.

    01:36 p-ANCA myeloperoxidase.

    01:38 It is a small medium vessel disease commonly affecting your abdomen, the mesenteric arteries, and also affecting your renal arteries resulting in your aneurysms.

    01:51 Anti-myeloperoxidase.

    01:53 What other differentials would also exist with p-ANCA? Churg Strauss, also called eosinophilic, have to.

    01:59 As soon as you heard Churg Strauss, you're thinking allergic, allergic, allergic- eosinophylic polyangiitis.

    02:05 And the last differential for p-ANCA here is going to be microscopic polyangiitis.

    02:10 Now, I'm just gonna group this together quickly for you.

    02:13 Here are a couple of other differentials that one determines right, I’ll introduce.

    02:16 At this point, you definitely know these three.

    02:18 In fact, with polyarteritis nodosa, only 50% at the time is it found to be positive.

    02:25 Let’s move in to cytoplasmic. What’s this? This is c-ANCA.

    02:30 c-ANCA stands for cytoplasmic. What does that mean? That means that if you’ll take a look at the ANCA, immunofluorescence, in it, in the cytoplasm, you'd find increase deposition.

    02:41 Formerly known as Wegener, we now call this GPA.

    02:45 Granulomatosis with polyangitis.

    02:47 Who is your patient? Where do you find granulomas? Where? In both lungs. Granulomas, granulomatosis.

    02:54 In addition, you might find skin lesions necrotizing.

    02:58 What else are you going to find? Hematuria, hemoptysis, and recurrent sinusitis.

    03:06 You see my ugly nose? You see the septum? Well in GPA, that septum can be completely annihilated.

    03:13 And you might wanna ride it.

    03:15 What does that mean? Yee ha! Saddle nose, saddle nose, saddle nose of your septum.

    03:21 Crazy, you won't find that in Goodpasture.

    03:24 Focal necrotizing vasculitis and granulomas in your upper airways, the lungs.

    03:31 There’s going to be hematuria because of glomerulonephritis.

    03:35 c-ANCA codes for anti-proteinase 3.

    03:39 Clinically perforation of that nasal septum, could that be any more dramatic.

    03:44 You will now find this with Goodpasture.

    03:47 Recurrent sinusitis you'll find this here with GPA but not Goodpasture.

    03:54 Cough, hemoptysis, you’ll find it in both Goodpasture and GPA.

    03:59 Red cell casts, both, you’ll find in Goodpasture and you’ll find in GPA.

    04:04 Use this information so that you're able to differentiate between Goodpasture and granulomatosis with polyangiitis.

    04:11 Welcome to c-ANCA and anti-proteinase 3.

    About the Lecture

    The lecture ANCA Associated Vasculitis by Carlo Raj, MD is from the course Glomerulonephritis.

    Included Quiz Questions

    1. Immune complex deposition is subendothelial.
    2. It is classified as the membranous form of glomerulonephritis.
    3. It has a linear pattern on immunofluorescence.
    4. It presents with a spike and dome appearance on light microscopy.
    5. It presents with nephrotic syndrome features.
    1. 10%
    2. 85%
    3. 50%
    4. 1%
    5. 20%
    1. Migratory arthalgias
    2. Abdominal pain
    3. Palpable purpura
    4. Intestinal bleeding
    5. Vomiting
    1. Goodpasture’s syndrome
    2. Polyarteritis nodosa
    3. All answers are correct.
    4. Microscopic angiitis
    5. Churg-strauss
    1. Saddle nose
    2. Hypertension
    3. Cough
    4. Hemoptysis
    5. Hematuria
    1. Elevated serum IgE
    2. Positive c-ANCA
    3. Decreased serum complement levels
    4. Hyponatremia
    5. Hypoalbuminemia

    Author of lecture ANCA Associated Vasculitis

     Carlo Raj, MD

    Carlo Raj, MD

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