Vasculitis Flashcards
Basic principles of vasculitis
Inflammation of the blood vessel wall
- arterial wall is comprised of 3 layers = endothelial intima, smooth muscle media and connective tissue adventitia
- etiology is usually unknown –> most cases are not infectious
Clinical features
- non-specific symptoms of inflammation –> fever, fatigue, weight loss and myalgias
- symptoms of organ ischemia –> due to luminal narrowing or thrombosis of the inflamed vessels
Divided into large, medium and small vessel vasculitides
- large vessel vasculitis –> involves the aorta and its major branches
medium vessel vasculitis –> involves muscular arteries that supply organs
- small vessel vasculitis –> involves arterioles, capillaries and venules
Temporal (giant cell) arteritis
Granulomatous vasculitis that classically involves branches of the carotid artery
- Large cell vasculitis
- most comon form of vasculitis in older adults (>50 years) - usually affects females
- presents as headache (temporal artery involvement), visual disturbances (opthalmic artery involvement), and jaw claudication
- flu like symptoms with join and muscle pain (polymyalgia rheumatica) are often presents
- ESR is elevated
Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis
- lesions are segmental –> diagnosis requires biopsy of a long segment of vessel
- negative biopsy does not exclude disease
Treatment is corticosteroids
- high risk of blindness without treatment
Takayasu arteritis
Granulomatous vasculitis that classically involves the aortic arch at branch points
- large vessel vasculitis
- presents in adults corticosteroids
Polyarteritis nodosa
Necrotizing vasculitis involving multiple organs –> lungs are spared!
- medium vessel vasculitis
- 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
- eventually heals with fibrosis, producing a string of pears appearance on imaging
Treatment –> corticosteroids and cyclophosphamide
- fatal if not treated
Kawasaki disease
Classically affects asian children aspirin and IVIG; disease is self limited
Buerger disease
Necrotizing vasculitis involving digits
- medium vessel vasculitis
- presents with ulceration, gangrene and autoamputation of fingers and toes
- Raynaud phenomenon is often presents
Highly associated with heavy smoking –> treatment is smoking cessation
Wegener granulomatosis
Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
- Small vessel vasculitis
- classic presentation is a middle aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates and hematuria due to a rapidly progressive glomerulonephritis
- serum c-ANCA levels correlate with disease activity
- biopsy reveals a large necrotizing granulomas with adjacent necrotizing vasculitis
Treatment –> cyclophosphamide and steroids
- relapses are common
Microscopic polyangiitis
Necrotizing vasculitis involving multiple organs, especially lungs and kidneys
- small vessel vasculitis
- presentation is similar to wegener granulomatosis, but nasopharyngeal involvement and granulomas are absent
- serum p-ANCA levels correlate with disease activity
Treatment –> corticosteroids and cyclophosphamide
- relapses are common
Churg-strauss syndrome
Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart
- small vessel vasculitis
- asthma and peripheral eosinophilia are often present
- serum p-ANCA levels correlate with disease activity
Henoch schonlein purpura
Vasculitis due to IgA immune complex deposition, most common vasculitis in kids
- small vessel vasculitis
- presents with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy)
- usually occurs following an upper resp tract infection
- disease is self limited, but may recur
Treated with steroids, if severe
Hemangioma
Benign tumor comprised of blood vessels
- commonly present at birth, orten regresses during childhood
- most often involves skin and liver
Angiosarcoma
Malignant proliferation of endothelial cells
- highly aggressive
- common sites include skin, breast and liver
- liver angiosarcoma –> associated with exposure to polyvinyl chloride, arsenic and thorotrast
Kaposi sarcoma
Low grade malignant proliferation of endothelial cells
- associated with HHV 8
- presents as purple patches, plaques and nodules on the skin, may also involve visceral organs
Classically seen in…
- older eastern european males –> tumor remains localized to skin, tx = surgical removal
- AIDS –> tumor spreads early; tx = antiretroviral agents to boost immune system
- transplant recipients –> tumor spreads early; tx = decreasing immunosuppression
