Vascular: Vasculitis & Arteriopathies

Temporal (Giant Cell) Arteritis

Basics

  • Large-Vessel Inflammatory Vasculitis
    • Particularly the Temporal Arteries
  • Long Segments of Smooth Stenosis
  • Most Common in Caucasians Age > 50

Complications

  • Headache
  • Scalp Tenderness
  • Jaw/Tongue Claudication (Specific)
  • Diplopia
  • Blindness
  • Aortic Aneurysm

Diagnosis

  • Gold Standard: Temporal Artery Bx
  • Requires ≥ 3 of 5 Criteria:
    • Age > 50 at Onset
    • New Headache
    • Temporal Artery Tenderness
    • ESR > 50 mm/h
    • Arterial Biopsy Showing Granulomatous Inflammations

Treatment

  • Primary Tx: Steroids
    • Low-Dose Aspirin (81 mg) Reduces Risk of Cardiovascular Events & Blindness

Takayasu Arteritis

Basics

  • Large-Vessel Inflammatory Vasculitis
    • Panarteritis Affecting All 3 Layers
  • Primarily Affects the Aorta & Large Branches
    • Can Also Affect Pulmonary Artery & Coronary Arteries
  • Most Common in Young (20-30’s) East-Asian Women

Phases

  • First Phase: Inflammatory Period
    • Fever
    • Headache
    • Myalgia
    • Arthralgia
  • Second Phase: Vessel Inflammation
    • Vessel Pain & Tenderness
    • Carotidynia: Pain Along Carotid Artery (Pathognomonic – 32%)
  • Third Phase: “Burned-Out” Period
    • Vessel Fibrosis
    • Aneurysmal Degeneration
  • *Diverse Presentation May Have Inflammation & Fibrosis Simultaneously

Complications

  • Hypertension
  • Syncope
  • TIA or Stroke
  • Visual Disturbances
  • Claudication
    • Upper Extremity More Common than Lower Extremity
  • Coronary Artery Disease
  • Aneurysms

Diagnosis

  • Generally a Diagnosis of Exclusion
  • Elevated ESR/CRP
  • CTA/MRA May Benefit
  • Gold Standard: Digital Subtraction Angiography

Treatment

  • Primary Tx: Steroids
  • Refractory Tx: Immunomodulators or Surgical Revascularization

Fibromuscular Dysplasia (FMD)

Basics

  • Nonatheromatous Noninflammatory Proliferative Process
  • Most Common in Women Age 20-60

Affected Arteries

  • Primarily Affects Medium-Sized Arteries
  • Can Be Seen in Any Artery
  • Most Common Sites:
    • Renal Artery – Most Common
    • Internal Carotid Artery
    • Vertebral Artery
    • Iliac Artery

Renal Artery FMD

Carotid FMD

  • Complications: Intractable Migraines, Pulsatile Tinnitus, Transient Ischemic Attack (TIA) or Stroke
  • Tx:
    • Asymptomatic: Medical Tx (Antiplatelet Tx)
    • Symptomatic: Angioplasty

Thromboangiitis Obliterans (Buerger’s Disease)

Basics

  • Nonatherosclerotic Segmental Obliterative Inflammatory Vasculitis
  • Affects Small-Medium Vessel Inflammation
  • Most Common in Young Male Smokers
  • See Corkscrew Collaterals

Complications

  • Digit Ischemia (Finger Gangrene)
  • Lower Extremity Claudication & Ulceration

Treatment

  • Primary Tx: Smoking Cessation

Raynaud’s Syndrome

Basics

  • Episodic Pallor/Cyanosis of the Fingers from Vasoconstriction of the Small Digital Arteries
    • Can Also Affect Toes
    • Can progress to Ulcer & Tissue Ischemia
  • Exaggeration of Normal Physiologic Response
  • Causes:
    • Cold Temperature
    • Emotional Stress
  • Most Common in Young Women

Definitions

  • Reynaud’s Disease – Primary/Idiopathic
  • Reynaud’s Phenomenon – Secondary Due to Other Associated Disease

Associated Disease

  • Vasculitis
  • Thromboangiitis Obliterans (Buerger’s Disease)
  • Temporal (Giant Cell) Arteritis
  • Scleroderma
  • Systemic Lupus Erythematosus
  • Rheumatoid Arthritis
  • Atherosclerosis
  • Malignancy
  • Vasopressors
  • Drugs – Cocaine/Amphetamines

Treatment

  • Initial Tx: Conservative Management
    • Avoid Cold or Aggravating Factors
    • Temperature Biofeedback
  • If Conservative Management Fails: Calcium Channel Blockers (Nifedipine/Diltiazem)
  • If Medical Management Fails: Botulinum Toxin Injection, Sympathetic Block or Sympathectomy

Radiation Arteritis

Basics

  • Arterial Inflammation from Radiation Exposure

Cause

  • Early (< 1 Year): Obliterative Endarteritis with Sloughing & Thrombosis
  • Late (1-10 Years): Fibrosis, Scarring & Stenosis
  • Late-Late (3-30 Years): Advanced Atherosclerosis