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
- Complications: Renovascular Hypertension
- *See Vascular: Renovascular Disease
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
