Vasculitis Flashcards
in vasculitis, there is inflammation and necrosis of the ____
in vasculitis, there is inflammation and necrosis of the blood vessels, including arteries, veins and capillaries
list the vasculitis disorders that are immune complex-mediated
- immune complex-mediated
- hepatitis B or C virus-mediated
- SLE and rheumatoid arthritis
- drug induced
describe the vasculitis disorders that are ANCA-mediated
- ANCA-mediated
- Wegener’s granulomatosis
- Churg-Strauss
list the vasculitis disorders that are direct antibody attack-mediated
- direct antibody attack-mediated
- Kawasaki disease (anti-endothelial cells antibodies)
describe the vasculitis disorders that are cell-mediated
- cell-mediated
- allograft organ rejection
___ is the most common type of vasculitis
explain this vasculitis
giant cell (temporal) arteritis (GCA)is the most common type of vasculitis
- affects mainly the arteries in the head
- aorta (giant cell aortitis)
- temporal
- vertebral
- ophthalmic → can lead to blindness
- T-cell mediated immunologic rxn against elastin (?)
describe the histology of giant cell arteritis
- focal, nodular thickening with reduction of lumen
- granulomatous inflammation of intima and inner media
- giant cells, mononuclear cells
- fragmentation of IEL
describe diagnosis of GCA
- diagnosis:
- elevated ESR
- temporal artery biopsy
- 2-3 cm. segment, multiple section
- elastic trichrome stain
- association with polymyalgia rheumatica
describe the treatment of giant cell arteritis
- treatment:
- steroids, analgesics
describe Takayasu arteritis
- granulomatous vasculitis of medium and large arteries with narrowing and obliteration of lumen
- most commonly affects arch of aorta with narrowing or virtual obliteration of the origins of the great vessels arising in the arch
- can also involve pulmonary, coronary and renal arteries
describe the clinical features of Takayasu arteritis
- most common in females < 40 yrs
- ocular changes: visual disturbances, retinal hemorrhages, blindness
- progressive diminution of upper limb pulses with coldness or numbness of fingers → pulseless disease
- low BP in upper limb
- neurologic defects → dizziness, focal weakness or complete hemiparesis
which disease shows the following:
progressive diminution of upper limb pulses with coldness or numbness of fingers → pulseless disease
Takayasu arteritis
describe what is seen in the image
Takayasu disease
aortic arch angiogram showing reduced flow of contrast material into the great vessels and narrowing of the brachiocephalic, carotid and subclavian arteries
Takayasu arteritis
- cross-sections of the right carotid artery demonstrating marked intimal thickening and luminal narrowing
describe what is seen in the image
Takayasu arteritis
- histo illustrating destruction and fibrosis of the arterial media associated with mononuclear infiltrates and giant cells (arrows)
describe polyarteritis nodosa
- systemic vasculitis
- transmural necrotizing inflammation of small or medium-sized muscular arteries
- typically involves renal and visceral arteries and spares the pulmonary circulation (PAN = Pulmonary Arteries Not affected)
- arterioles, capillaries and venules are not affected
30% of PAN patients also have _____
30% of PAN patients also have Hep B
describe the gross morphology of PAN and the order in which organs are affected
- gross:
- distribution of lesions in descending order of frequency → kidney, heart, liver, GIT (lungs NOT involved)
- lesions have a predilection for branching points
describe the histo morphology of PAN
all stages of activity may coexist in different vessels or even within the same vessel which is a characteristic feature
- histo:
- acute stage: transmural inflammation, fibrinoid necrosis of the inner half of the vessel wall
- later stages: inflammation is replaced by fibrous thickening of the vessel wall
describe the image
PAN
- associated with segmental fibrinoid necrosis and thrombotic occlusion of a small artery. note that part of the vessel (arrow) is uninvolved
list complications of PAN
- weakening of arterial wall owing to inflammatory process may cause aneurysmal dilatation or localized rupture
- impairment of perfusion causing ulcerations, infarcts, ischemic atrophy, or hemorrhages in the areas supplied by these vessels
describe Kawasaki disease
- arteritis involving the large, medium and small arteries (often coronary arteries)
- also called mucocutaneous lymph node syndrome (MCLNS)
- usually in young children and infants
- leading cause of acquired heart disease in children in the US
in Kawasaki disease there are ___ against ____ leading to acute vasculitis
in Kawasaki disease there are autoantibodies against endothelium, smooth muscle cells leading to acute vasculitis
describe the clinical features of Kawasaki disease
describe the treatment of Kawasaki disease
describe the morphology of Kawasaki disease
- lesions may range from:
- severe destruction of all constituents of the wall by a segmental necrotizing process, with moderate fibrinoid change and dense infiltrate of inflammatory cells, to
- mild changes involving intima only
- acute vasculitis may lead to coronary aneurysm formation and associated thrombosis with MI
describe Buerger disease (Thromboangiitis obliterans)
- Buerger disease (thromboangiitis obliterans)
- characterized by segmental, thrombosing, acute and chronic inflammation of medium-sized and small arteries
- mainly the tibial and radial arteries and sometimes secondarily extending to veins and nerves of the extremities
in Buerger disease (thromb. obliterans), the ___ and ___ arteries are mainly affected
in Buerger disease ( thromb . obliterans), the tibial and radial arteries are mainly affected
the main predisposing factor for Buerger disease is _____ and mainly affects ____ (3 populations)
the main predisposing factor for Buerger disease is cigarette smoking (HS reaction to tobacco → direct endothelial injury) and mainly affects people from India, Israel and Japan
describe the clinical features of Buerger disease
- affects males most commonly (<35 yrs)
- superficial nodular phlebitis, cold insensitivity (Reynaud’s), claudication pain on the instep, severe progressive pain (due to neural involvement) on exercise and at rest
- chronic ulceration of the toes, feet or finger may appear followed by gangrene
describe the morphology seen in Buerger disease
- sharply segmental acute and chronic vasculitis: medium and small-sized arteries of extremities
- acute and chronic inflammation permeating the arterial walls, accompanied by thrombosis of the lumen
describe microscopic polyangiitis (hypersensitivity or leukocytoclastic vasculitis)
- necrotizing vasculitis of capillaries, arterioles and small venules
- segmental fibrinoid necrosis of media with focal transmural necrotizing lesions - PMNs
- no infarcts - small size vessels
- absence of granulomatous response
- all organs affected including lungs
- all lesions same age
microscopic polyangiitis may be caused by hypersensitivity to drugs, like ____ and infections by _____
microscopic polyangiitis may be caused by hypersensitivity to drugs, like penicillin and infections by Strep
patients with microscopic polyangiitis test positive for ____ aka _____ and there is absence of ____
patients with microscopic polyangiitis test positive for MPO-ANCA aka p-ANCA and there is absence of IgG (pauci-immune)
describe the clinical features of microscopic polyangiitis
- hemoptysis, hematuria, proteinuria
- blood in the stool
- cutaneous purpura
- respond to removal of the offending agent
describe the triad seen in granulomatosis with polyangiitis (Wegener Granulomatosis)
- triad:
- acute necrotizing granulomas of upper and lower resp. tract
- focal necrotizing or granulomatous vasculitis affecting small to medium-sized vessels
- renal disease in the form of focal or necrotizing, often crescentic glomerulonephritis
patients with GPA/Wegeners test positive for ____
patients with GPA/ Wegeners test positive for c-ANCA
describe the clinical features of GPA/WG
- M>F
- persistent pneumonitis with bilateral nodular and cavitary infiltrates
- hemoptysis
- chronic sinusitis
- mucosal ulceration of the nasopharynx
- evidence of renal disease (hematuria, acute renal failure)
- 80% of untreated patients die within 1 year
describe Churg-Strauss Syndrome
- allergic granulomatosis and angiitis
- allergic rhinits, bronchial asthma, peripheral eosinophilia
- positivity for p-ANCA
summarize the small blood vessel vasculitis conditions
