Vascular Pathology Flashcards
Vasculitis - basics
A. Inflammation of the blood vessel wall
1. Arterial wall is comprised of three layers: endothelial intima, smooth muscle media, and connective tissue adventitia (Fig. 7.1).
B. Etiology is usually unknown; most cases are not infectious.
C. Clinical features include
1. Nonspecific symptoms of inflammation (e.g., fever, fatigue, weight loss, and myalgias)
2. Symptoms of organ ischemia - due to luminal narrowing or thrombosis of the inflamed vessels
D. Divided into large-, medium-, and small-vessel vasculitides
1. Large-vessel vasculitis involves the aorta and its major branches.
2. Medium-vessel vasculitis involves muscular arteries that supply organs.
3. Small-vessel vasculitis involves arterioles, capillaries, and venules.
LARGE-VESSEL VASCULITIS
A. Temporal (Giant Cell) Arteritis
1. Granulomatous vasculitis that classically involves branches of the carotid artery 2. Most common form of vasculitis in older adults (> 50 years); usually affects
females
3. Presents as headache (temporal artery involvement), visual disturbances (ophthalmic artery involvement), and jaw claudication. Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica) are often present. ESR is elevated.
4. Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis (Fig. 7.2).
i. Lesions are segmental; diagnosis requires biopsy of a long segment of vessel, and a negative biopsy does not exclude disease.
5. Treatment is corticosteroids; high risk of blindness without treatment
B. Takayasu Arteritis
1. Granulomatous vasculitis that classically involves the aortic arch at branch points
2. Presents in adults < 50 years old (classically, young Asian females) as visual and neurologic symptoms with a weak or absent pulse in the upper extremity (‘pulseless disease’). ESR is elevated. 3. Treatment is corticosteroids.
Temporal (Giant Cell) Arteritis
A. Temporal (Giant Cell) Arteritis
1. Granulomatous vasculitis that classically involves branches of the carotid artery 2. Most common form of vasculitis in older adults (> 50 years); usually affects
females
3. Presents as headache (temporal artery involvement), visual disturbances
(ophthalmic artery involvement), and jaw claudication. Flu-like symptoms with
joint and muscle pain (polymyalgia rheumatica) are often present. ESR is
elevated.
4. Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis (Fig. 7.2).
i. Lesions are segmental; diagnosis requires biopsy of a long segment of vessel,
and a negative biopsy does not exclude disease. 5. Treatment is corticosteroids; high risk of blindness without treatment
Takayasu Arteritis
- Granulomatous vasculitis that classically involves the aortic arch at branch
points 2. Presents in adults < 50 years old (classically, young Asian females) as visual and
neurologic symptoms with a weak or absent pulse in the upper extremity
(‘pulseless disease’). ESR is elevated. 3. Treatment is corticosteroids.
Medium vessel vasculitis
polyarteritis nodosa
Kawasaki disease
buerger disease
Polyarteritis Nodosa
- Necrotizing vasculitis involving multiple organs; lungs are spared.
- Classically presents in young adults as hypertension (renal artery involvement),
abdominal pain with melena (mesenteric artery involvement), neurologic disturbances, and skin lesions. Associated with serum HBsAg - Lesions of varying stages are present. Early lesion consists of transmural
inflammation with fibrinoid necrosis (Fig. 7.3); eventually heals with fibrosis, producing a ‘string-of-pearls’ appearance on imaging - Treatment is corticosteroids and cyclophosphamide; fatal if not treated
Kawasaki Disease
- Classically affects Asian children < 4 years old
- Presents with nonspecific signs including fever, conjunctivitis, erythematous
rash of palms and soles, and enlarged cervical lymph nodes - Coronary artery involvement is common and leads to risk for (1) thrombosis
with myocardial infarction and (2) aneurysm with rupture. - Treatment is aspirin and IVIG; disease is self-limited.
Buerger Disease
- Necrotizing vasculitis involving digits
- Presents with ulceration, gangrene, and autoamputation of fingers and toes;
Raynaud phenomenon is often present. - Highly associated with heavy smoking; treatment is smoking cessation.
SMALL-VESSEL VASCULITIS
Wegener Granulomatosis
Microscopic Polyangitis
Churg-Strauss Syndrome
Henoch- Schonlein Purpura
Wegener Granulomatosis
- Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys 2. Classic presentation is a middle-aged male with sinusitis or nasopharyngeal
ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due
to rapidly progressive glomerulonephritis. - Serum c-ANCA levels correlate with disease activity.
- Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis
(Fig. 7.4). - Treatment is cyclophosphamide and steroids; relapses are common.
Microscopic Polyangiitis
- Necrotizing vasculitis involving multiple organs, especially lung and kidney
- Presentation is similar to Wegener granulomatosis, but nasopharyngeal
involvement and granulomas are absent. 3. Serum p-ANCA levels correlate with disease activity. 4. Treatment is corticosteroids and cyclophosphamide; relapses are common.
Churg-Strauss Syndrome
- Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart
- Asthma and peripheral eosinophilia are often present.
- Serum p-ANCA levels correlate with disease activity .
Henoch-Schönlein Purpura
- Vasculitis due to IgA immune complex deposition; most common vasculitis in
children 2. Presents with palpable purpura on buttocks and legs, GI pain and bleeding, and
hematuria (IgA nephropathy); usually occurs following an upper respiratory
tract infection 3. Disease is self-limited, but may recur; treated with steroids, if severe
Hypertension -basics
A. Increased blood pressure; may involve pulmonary (see chapter 9) or systemic
circulation B. Systemic HTN is defined as pressure ≥ 140/90 mm Hg (normal ≤ 120/80 mm Hg);
divided into primary or secondary types based on etiology
PRIMARY HTN
A. HTN of unknown etiology (95% of cases)
B. Risk factors include age, race (increased risk in African Americans, decreased risk in
Asians), obesity, stress, lack of physical activity, and high-salt diet.