StepWise USMLE
vascular pathology · systemic

Vessels · Inflammation · Remodeling

Vasculitis · arteriosclerosis · aneurysms · venous disease · vascular neoplasms

🫀 Vasculitides

  • Systemic inflammatory vessel disorders — heterogenous presentation
  • Categorised by predominant vessel size (large / medium / small)
  • Vasculitis may be primary or secondary to infection, drugs, or connective tissue disease
  • Large vessel
    • Takayasu arteritis – age <50, aortic arch involvement, granulomatous inflammation, blindness possible
    • Giant cell (temporal) arteritis – older adults, headache, jaw claudication, vision loss; inner media granulomas; steroids & anti-TNF
  • Medium vessel
    • Kawasaki disease – children, mucocutaneous + cervical LAD; coronary aneurysms; IVIG therapy
    • Polyarteritis nodosa – young adults (M>F), HBV association, renal involvement can be fatal; spares lungs
  • Small vessel
    • ANCA-associated
      • Granulomatosis with polyangiitis (Wegener) – lung/upper airway granulomas, GN; PR3-ANCA (+)
      • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) – asthma, eosinophilia, MPO-ANCA; cardiac death possible
    • Immune complex–mediated – anti-GBM disease, IgA vasculitis (Henoch-Schönlein)
  • Thromboangiitis obliterans (Buerger) – thrombosis predominant, young male smokers, distal extremity ulcers

❄️ Raynaud phenomena

  • Primary Raynaud – young women, episodic vasospasm (fingers/toes/nose/ears) triggered by cold/stress; blanching → cyanosis
  • Secondary Raynaud – underlying arterial insufficiency (e.g. scleroderma/CREST)
  • Secondary Raynaud often asymmetric, may progress to digital ulcers

🧬 Arteriosclerosis

  • Monckeberg medial calcific sclerosis – media calcification of medium muscular arteries (femoral, radial), asymptomatic, x-ray finding
  • Arteriolosclerosis (small arteries/arterioles)
    • Hyaline – pink glassy thickening; benign HTN, diabetes, aging
    • Hyperplastic – concentric “onion skin”; malignant HTN
  • Atherosclerosis – large/medium elastic & muscular arteries; chronic inflammatory process
    • Fatty streak (reversible) – lipid-laden macrophages (foam cells)
    • Stable plaque – dense fibrous cap, small lipid core, chronic ischemia
    • Vulnerable plaque – thin cap, large lipid core, dense inflammation → rupture/embolism
Risk factorImpact
Hyperlipidemia↑ LDL, ↑ oxidation
HypertensionEndothelial injury
SmokingOxidative stress, vasospasm
DiabetesAdvanced glycation end products
Obesity / sedentaryInsulin resistance
  • Complications: MI, CVA, atheroemboli (TIA, renal infarcts), aneurysm, PVD, mesenteric occlusion

⬆️ Hypertension

  • Persistent diastolic >80 or systolic >130 mmHg with end-organ damage
  • Malignant (accelerated) – systolic >180 / diastolic >120; retinal hemorrhages, exudates, papilledema; medical emergency
  • Epidemiology – 25% US adults; African Americans at higher risk; 95% essential (idiopathic)
  • End-organ damage
    • Hyaline arteriolosclerosis (arterioles)
    • Concentric LV hypertrophy → CHF
    • Accelerated atherosclerosis, MI, aneurysm, CVA, renal failure
  • Malignant HTN: 2-year mortality >90% if untreated (renal failure, stroke, heart failure)

🫧 Aneurysms & AV fistulas

  • Aneurysm – localised dilatation due to media weakness; complications: thrombosis, embolism, compression, rupture
  • Atherosclerotic – abdominal aorta (below renal arteries), HTN association; >6 cm → 50% rupture in 10y; >5 cm surgical
  • Syphilitic – ascending aorta, tertiary syphilis; obliterative endarteritis of vasa vasorum → aortic regurgitation
  • Aortic dissection – intimal tear, media degeneration (cystic medial necrosis); severe tearing pain; Marfan / HTN
  • Berry aneurysm – circle of Willis (congenital)
  • Microaneurysms – HTN, diabetes
  • Arteriovenous fistula – direct artery–vein connection (congenital/trauma); high-output failure, rupture risk
Intimal tear→ Blood enters media→ Dissection plane→ Compression / rupture

🩸 Venous disease

  • Deep vein thrombosis – iliac/femoral/popliteal; unilateral swelling, warmth, erythema; Homan sign; Doppler ultrasound; PE risk
  • Varicose veins – dilated tortuous veins; lower extremity (superficial), pregnancy; esophageal varices (portal HTN), hemorrhoids
  • Venous insufficiency – edema, hyperpigmentation, ulcers (gaiter area); female > male, age-related
ConditionKey feature
DVTUnilateral leg pain/swelling, PE
Varicose veinsSuperficial, pregnancy, hemorrhoids
Venous insufficiencyEdema, stasis dermatitis, ulcers

🧫 Vascular neoplasms

  • Benign
    • Hemangioma – capillary/cavernous, most common infant tumour, may regress
    • Hemangioblastoma – von Hippel-Lindau (cerebellum, retina, RCC)
    • Glomus tumour – painful subungual nodule
  • Malignant
    • Kaposi sarcoma – HHV8; endothelial spindle cells, slit-like spaces, extravasated RBCs
      • Classic (European) – lower extremity plaques in elderly
      • Transplant-associated – immunosuppression, may regress
      • African – children/young men, lymphatic spread
      • AIDS-associated – aggressive, skin/GI/lungs, chemotherapy responsive
    • Angiosarcoma – high mortality; skin, breast, liver; vinyl chloride / arsenic association (liver)
  • Port‑wine stain (vascular malformation) does not regress; nevus flammeus nuchae (“stork bite”) regresses