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Vasculitis: A Quick Review

Vasculitis can be approached according to the size of the vessel that is affected and/or the mechanism of inflammation.



LARGE VESSEL VASCULITIS

  • Takayasu Arteritis

  • Giant cell arteritis


 

MEDIUM-SIZED VESSEL VASCULITIS

  • PolyArteritis Nodosa (PAN)

  • Kawasaki Disease


 

SMALL VESSEL VASCULITIS


To approach the etiology of vasculitis within small vessels, we further subdivide it into whether the inflammation is secondary to an antibody directed against some component of the vessel (ANCA-associated or the Anti-GBM antibody), or whether there is indirect destruction due to the deposition of immune complexes within the vessel wall (an antibody bound to some antigen)


ANCA-ASSOCIATED SMALL VESSEL VASCULITIS

ANCA-associated destruction affects multiple vessels all over (hence the term POLYangiitis). It can be further subdivided whether it demonstrates cytoplasmic or nuclear staining pattern on fluorescence


C-ANCA= CYTOPLASMIC + against Protease 3
  • (Wegner's) Granulomatosis with polyangitis


P-ANCA= PERINUCLEAR + against MyeloPerOxidase (MPO)
  • (Churg-Strauss) Eosinophilic granulomatosis with polyangitis

  • (PAN) Microscopic PolyANgitis

from ExpertPath

ANTI-GLOMERULAR BASEMENT MEMBRANE (ANTI-GBM)

SMALL VESSEL VASCULITIS

  • Goodpasture Syndrome


IMMUNE COMPLEX SMALL VESSEL VASCULITIS

  • Cryoglobulinemic Vasculitis

  • IgA Vasculitis (Henoch-Schonlein purpura)

  • Hypocomplementemic Urticarial vasculitis (Anti-C1q vasculitis)


There will be separate posts demonstrating histologic examples and further details of each of the above diseases.

 

POP QUIZ

Let's see if you can still remember a few of the high yield associations you learned about these diseases back in the good ol' med school days...


1. How can you DDx Wegner's from Churg Strauss?


Both have granulomatous inflammation with geographic necrosis

Wegner's does NOT have inc eosinophils

Churg Strauss has inc eosinophils + often a history of asthma or allergies



2. How can you DDx Wegners from Goodpasture syndrome?


Both cause problems in the lung & kidney

Wegners is cANCA + and also involves the UPPER respiratory tract (nose bleeds etc)

Goodpasture's (anti-GBM) does NOT affect the URT



3. What is the antibody against in c-ANCA?

Which disease(s) does it cause?


Proteinase 3; cytoplasmic +; c-Anti-Neutrophil Cytoplasmic Ab

Wegner's (aka weCners) Granulomatosis



4. What is the antibody against in p-ANCA?

Which disease(s) does it cause?


Myeloperoxidase (MPO); perinuclear +; p-Anti-Neutrophil Cytoplasmic Ab

Churg Strauss/Eosinophilic Granulomatosis & microscopic polyangiitis

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