Vasculitis

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Vasculitis Basics - Vessel Wall Woes

  • Inflammation & necrosis of blood vessel walls, leading to downstream tissue ischemia and potential organ damage.
  • Pathogenesis is often immune-mediated via immune complexes, anti-neutrophil cytoplasmic antibodies (ANCAs), or pathogenic T-cells.
  • Classified by the predominant vessel size involved.

⭐ Most vasculitides present with nonspecific constitutional symptoms like fever, myalgia, arthralgia, and fatigue.

Histology of blood vessel wall layers

Large-Vessel - Aortic Arch Archons

  • Giant Cell (Temporal) Arteritis

    • Age > 50; affects branches of carotid artery.
    • Symptoms: Unilateral headache, jaw claudication, tender temporal artery.
    • Assoc: Polymyalgia Rheumatica (PMR).
    • Path: Granulomatous inflammation, fragmented internal elastic lamina; ↑ ESR, CRP.
    • Tx: High-dose corticosteroids STAT to prevent blindness.
  • Takayasu Arteritis

    • Age < 50 (typically young Asian women).
    • Symptoms: "Pulseless disease" → weak upper extremity pulses, fever, night sweats, arthritis.
    • Path: Granulomatous thickening of aortic arch & proximal great vessels.

High-Yield: Unilateral vision loss in GCA is an ophthalmic emergency due to anterior ischemic optic neuropathy (AION). It can become bilateral and irreversible within hours.

Giant Cell Arteritis Histopathology

Medium-Vessel - Beaded & Fiery

  • Polyarteritis Nodosa (PAN)

    • Segmental, transmural necrotizing vasculitis.
    • Associated with Hepatitis B/C in ~30% of cases.
    • Affects renal & visceral arteries; spares pulmonary circulation.
    • Creates microaneurysms, giving a "string-of-pearls" or "rosary bead" appearance on angiography.
    • Presents with fever, abdominal pain, myalgias, and hypertension.
  • Kawasaki Disease

    • Affects Asian children < 4 years old.
    • 📌 CRASH & Burn: Conjunctivitis, Rash, Adenopathy, Strawberry tongue, Hand-foot changes + Fever >5 days.

    ⭐ High risk of coronary artery aneurysms. Treat urgently with IVIG and aspirin.

Kawasaki Disease: Symptoms and Cardiac Complications

Small-Vessel - ANCA's Angry Armada

  • Pauci-immune necrotizing vasculitis: Characterized by little to no immune complex deposition on vessel walls. All are associated with Anti-Neutrophil Cytoplasmic Antibodies (ANCA).
FeatureGranulomatosis w/ Polyangiitis (GPA)Microscopic Polyangiitis (MPA)Eosinophilic GPA (EGPA)
ANCAc-ANCA (anti-PR3) >80%p-ANCA (anti-MPO) ~70%p-ANCA (anti-MPO) ~50%
Key OrgansUpper respiratory (sinusitis, otitis), Lower respiratory (nodules, hemoptysis), Kidneys (RPGN)Lungs, Kidneys (RPGN). No nasopharyngeal involvement.Lungs, Heart, Skin, Nerves (mononeuritis multiplex). Late-stage renal.
HistologyGranulomatous inflammationNon-granulomatousEosinophilic infiltrates, granulomas
UniqueNasopharyngeal destructionNo granulomasAllergic rhinitis, asthma, peripheral eosinophilia

⭐ ANCA vasculitides cause a pauci-immune (literally "few-immunes") glomerulonephritis. Biopsy shows necrotizing glomerulonephritis with crescents but lacks significant Ig/C3 deposition on immunofluorescence.

Pauci-immune crescentic glomerulonephritis histology

High‑Yield Points - ⚡ Biggest Takeaways

  • Giant Cell Arteritis: Most common vasculitis in adults >50; presents with headache, jaw claudication, and risk of blindness. Associated with high ESR.
  • Kawasaki Disease: Affects children <5; presents with fever & CRASH symptoms. Can lead to coronary artery aneurysms.
  • Polyarteritis Nodosa: Necrotizing vasculitis of medium arteries, strongly associated with Hepatitis B, and characteristically spares the lungs.
  • GPA (Wegener's): Triad of sinus, lung, and kidney disease; positive for c-ANCA.
  • Thromboangiitis Obliterans: Seen in heavy smokers; can lead to gangrene of digits.

Practice Questions: Vasculitis

Test your understanding with these related questions

A 72-year-old man presents to his primary care provider complaining of fatigue, mild headache, and discomfort with chewing for roughly 1 week. Before this, he felt well overall, but now he is quite bothered by these symptoms. His medical history is notable for hypertension and hyperlipidemia, both controlled. On examination, he is uncomfortable but nontoxic-appearing. There is mild tenderness to palpation over his right temporal artery, but otherwise the exam is not revealing. Prompt recognition and treatment can prevent which of the following feared complications:

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Flashcards: Vasculitis

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_____ is a form of arteriosclerosis that damages large- and medium-sized vessels due to buildup of cholesterol plaques

TAP TO REVEAL ANSWER

_____ is a form of arteriosclerosis that damages large- and medium-sized vessels due to buildup of cholesterol plaques

Atherosclerosis

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