Vasculitis

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Introduction & Classification - Vessel Vandals Intro

  • Vasculitis: Inflammation of blood vessel walls → vessel damage (stenosis, occlusion, aneurysm).
  • Chapel Hill Consensus Conference (CHCC) 2012 Classification:
Vessel SizeVasculitis TypeKey Examples
LargeLarge Vessel Vasculitis (LVV)Giant Cell Arteritis (GCA; Age >50 yrs), Takayasu Arteritis (TA) 📌 'Giant Takayasu'
MediumMedium Vessel Vasculitis (MVV)Polyarteritis Nodosa (PAN), Kawasaki Disease (KD)
SmallSmall Vessel Vasculitis (SVV)ANCA-Associated (AAV): MPA, GPA, EGPA
Immune Complex: IgA Vasculitis, Cryoglobulinemic Vasculitis

⭐ Giant Cell Arteritis (GCA) is the most common systemic vasculitis in individuals aged >50 years in Western countries.

Pathophysiology & Etiology - Inflammation Ignition

  • Immune Mechanisms: Vessel inflammation driven by:
    • Type II (antibody-mediated cytotoxicity).
    • Type III (immune complex deposition).
    • Type IV (cell-mediated immunity).
  • ANCA (Anti-Neutrophil Cytoplasmic Antibodies): Key in pauci-immune types.
    • PR3-ANCA (c-ANCA): Targets Proteinase 3; associated with GPA.
      • 📌 c-ANCA/PR3 ~ GPA ('C' for 'Ceiling' - affects upper respiratory/sinuses).
    • MPO-ANCA (p-ANCA): Targets Myeloperoxidase; linked to MPA, EGPA.
  • Common Triggers:
    • Infections: HBV (PAN), HCV (cryoglobulinemia).
    • Drugs: e.g., Hydralazine, Propylthiouracil.
    • Malignancies. Classification of vasculitis

⭐ PR3-ANCA (formerly c-ANCA) is highly specific for Granulomatosis with Polyangiitis (GPA).

Vasculitis TypeVessel SizeKey DemographicsClinical HallmarksDiagnostic Markers
GCALarge>50 yrs, F>MTemporal headache, jaw claudication, visual loss, PMRESR >50, CRP ↑, Temporal artery biopsy (granuloma)
Takayasu ArteritisLarge<40 yrs, Asian F"Pulseless disease", claudication, BP discrepancy, aortic archESR/CRP ↑, Angiography (MRA/CTA)
PANMediumMid-age, M>F, Hep B (10-30%)Renal (no GN), HTN, skin (livedo), neuropathy, GI; spares lungs. 📌 "Beads on string" (angio)pANCA neg, Biopsy (necrotizing), Angio (microaneurysms)
Kawasaki DiseaseMediumChildren <5 yrs, Asian📌 CRASH & Burn (Conjunctivitis, Rash, Adenopathy, Strawberry tongue, Hand/foot) + Fever ≥5d. Coronary aneurysms.Clinical, ESR/CRP ↑, Echo (coronary)
GPA (Wegener's)SmallAdults (mean 40-55)Triad: Upper/Lower resp. tract (sinusitis, nodules, hemoptysis), Kidneys (GN)c-ANCA (PR3-ANCA) +, Biopsy (necrotizing granulomas)
IgA Vasculitis (HSP)SmallChildren (peak 4-6 yrs), post-URITetrad: Palpable purpura, Arthritis, Abd pain, Renal (IgA nephropathy)Clinical, Biopsy (skin/kidney): IgA deposition

Management Principles - Dousing the Flames

Goals of Treatment:

  • Induce remission rapidly.
  • Maintain long-term remission.
  • Prevent disease relapse and minimize organ damage.

General Therapeutic Arsenal:

  • Corticosteroids: e.g., Prednisone (initial dose often 1 mg/kg/day).
  • Immunosuppressants:
    • Cyclophosphamide (CYC)
    • Azathioprine (AZA)
    • Methotrexate (MTX)
    • Mycophenolate Mofetil (MMF)
    • Rituximab (RTX)

Severe ANCA-Associated Vasculitis (AAV) Strategy:

  • Induction: Typically Cyclophosphamide (CYC) or Rituximab (RTX) combined with high-dose corticosteroids.

⭐ Rituximab is increasingly used as an alternative to cyclophosphamide for induction of remission in severe ANCA-associated vasculitis, particularly in relapsing disease or if cyclophosphamide is contraindicated.

High‑Yield Points - ⚡ Biggest Takeaways

  • Giant Cell Arteritis: Elderly (>50 yrs); temporal artery involvement; jaw claudication, visual loss; prompt steroids.
  • Takayasu Arteritis: Young females; "Pulseless disease"; affects aortic arch & branches; discrepant BP.
  • Buerger's Disease: Strongly linked to heavy smokers; affects distal extremity vessels; claudication, gangrene.
  • Polyarteritis Nodosa (PAN): Affects medium arteries; Hepatitis B association; renal/mesenteric ischemia; spares lungs.
  • Kawasaki Disease: Primarily in children; risk of coronary artery aneurysms; treat with IVIG & aspirin.
  • GPA (Wegener's): c-ANCA positive; involves respiratory tract & kidneys; characterized by granulomas.

Practice Questions: Vasculitis

Test your understanding with these related questions

A male child presented with arthralgia and abdominal pain. On examination, there was palpable purpura over the lower limbs. There is a past history of upper respiratory tract infection prior to the onset of presenting symptoms. Which of the following is the treatment for this condition?

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Flashcards: Vasculitis

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Pulsating hematoma is associated with _____ aneurysm.

TAP TO REVEAL ANSWER

Pulsating hematoma is associated with _____ aneurysm.

false (true/false)

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