Inflammatory diseases of the Aorta
VASCULITIDES
1. For practical purposes, the vasculitides can be classified on the basis of the size of the vessel(s) involved and will be presented accordingly in the following sections
2. Specific cause can be identified in only a minority of cases
3. Can be divided into infectious and noninfectious groups, with the latter much more extensive
4. There is a correlation between the type of antineutrophil cytoplasmic autoantibodies (ANCA) and the specific vasculitis syndrome
5. cANCA (antiproteinase 3) is more often seen in active Wegener disease
6. pANCA (antimyeloperoxidase) is more often seen in microscopic Polyangiitis, primary glomerular disease (idiopathic crescentic glomerulonephritis) and in Churg-Strauss syndrome
Takayasu Arteritis
- Takayasu Arteritis Clinical aspects.
1. Onset of disease usually before the age of 50
2. Predominantly affects women
3. More prevalent in Asia than in the US and Europe
4. Disease manifestation is variable and includes decreased/absent pulses in upper extremities, renovascular hypertension
- Takayasu Arteritis Gross exam.
1. Involvement of aorta, aortic arch branches, pulmonary arteries, and occasionally coronary arteries
2. May involve only a portion (thoracic more often affected than abdominal) or the entire aorta
3. Stenosing lesions more common than aneurysms
4. Aorta is thickened, sometimes with superimposed thrombosis
- Takayasu Arteritis Microscopic exam.
1. Chronic granulomatous inflammation predominantly affecting the media and adventitia
2. Inflammatory cells are composed of lymphocytes, plasma cells and macrophages, giant cells are commonly present
3. Intima shows reactive hyperplasia
4. In chronic lesions, media shows destruction and fragmentation of elastic lamellae and adventitia often markedly fibrotic
Giant Cell Arteritis (GCA)
- Giant Cell Arteritis Clinical aspects.
1. Onset of disease usually after the age of 50
2. Female predominance
3. May be but not always associated with temporal arteritis
4. Clinical manifestations include temporal headache, visual disturbances, jaw or tongue claudication, scalp tenderness, polymyalgia rheumatica
5. Aortic aneurysms are late complications of the disease
6. Temporal artery biopsy may be performed when GCA is associated with cranial arteritis and visual loss is a serious complication of the disease
- Giant Cell Arteritis Gross exam.
1. Preferentially involves the thoracic aorta
2. Aortic wall may be thickened with “tree-barking” of the intima and superimposed atherosclerosis
- Giant Cell Arteritis Microscopic exam.
1. Areas of medial necrosis bordered by mononuclear cells and giant cells
2. Proliferation of vasa vasorum with surrounding mononuclear inflammatory cells (lymphocytes, plasma cells, macrophages)
3. Reactive intimal hyperplasia
4. Adventitia with mild inflammation and mild fibrotic thickening
5. Histopathology sometimes indistinguishable between systemic (aortic involvement in GCA) and isolated aortitis
6. Temporal arteritis
- Granulomatous inflammation in the media consisting of macrophages and lymphocytes
- Giant cells are present in most cases, but not a prerequisite for the diagnosis of temporal arteritis
- Disruption of the internal elastic lamina associated with the inflammation
Other rare causes of large-vessel vasculitis
1. Behcet’s disease
2. Rheumatoid arthritis
3. Ankylosing spondylitis
4. Cogan’s syndrome
5. Relapsing polychondritis
6. IgG4-related disorders
7. Behcet’s disease
8. Rheumatoid arthritis
9. Ankylosing spondylitis
10. Cogan’s syndrome
11. Relapsing polychondritis
12. IgG4-related disorders
