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Vasculitis MLC
Main Vasculitides, their definitions, presentations, classifications and pathophysiological mechanisms
92
Pathology
Professional
10/30/2011

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Term
Giant-cell (temporal) arteritis
Definition
Granulomatous inflammation; frequently involves the temporal artery. Usually occurs in patients older than age 50 and is associated with polymyalgia rheumatica.
Term
Takayasu arteritis
Definition
Granulomatous inflammation usually occurring in patients younger than age 50
Term
Polyarteritis nodosa
Definition
Necrotizing inflammation typically involving renal arteries but sparing pulmonary vessels
Term
Kawasaki disease
Definition
Arteritis with mucocutaneous lymph node syndrome; usually occurs in children. Coronary arteries can be involved with aneurysm formation and/or thrombosis.
Term
Wegener granulomatosis
Definition
Granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vessels, including glomerular vessels. Associated with PR3-ANCAs.
Term
Churg-Strauss syndrome
Definition
Eosinophil-rich granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vessels. Associated with asthma and blood eosinophilia. Associated with MPO-ANCAs.
Term
Microscopic polyangiitis
Definition
Necrotizing small-vessel vasculitis with few or no immune deposits; necrotizing arteritis of small and medium-sized arteries can occur. Necrotizing glomerulonephritis and pulmonary capillaritis are common. Associated with MPO-ANCAs.
Term
Giant-cell (temporal) arteritis
Definition
Large arteries (Aorta, aortic arch vessels to head, neck, extremities)
Term
Takayasu arteritis
Definition
Large arteries (Aorta, aortic arch vessels to head, neck, extremities)
Term
Polyarteritis nodosa
Definition
Medium arteries (usually renal arteries, sparing pulmonary)
Term
Kawasaki disease - Vessel Size
Definition
Medium arteries - possibly coronary
Term
Wegener granulomatosis
Definition
Small arteries - including glomerular
Term
Polyarteritis Nodosa
Definition
Immune complex mediated medium vessel vasculitis
Term
Kawasaki Disease
Definition
Anti-endothelial cell antibody mediated medium vessel vasculitis
Term
Chrug-Strauss Syndrome
Definition
Small vessel vasculitis, few immune complexes, with eosinophilia, asthma and granulomas
Term
Chrug-Strauss Syndrome
Definition
Small vessel vasculitis without few immune complexes, with eosinophilia, asthma and granulomas
Term
Wegener Granulomatosis
Definition
Small vessel vasculitis with few immune complexes, granulomas, no asthma
Term
Microscopic Polyangiitis
Definition
Small vessel vasculitis with few immune complexes, without asthma or granulomas
Term
Henoch-Schonlein Purpura
Definition
Small vessel vasculitis with immune complexes of IgA
Term
Cryoglobulin Vasculitis
Definition
Small vessel vasculitis with immune complexes and cryoglobulin
Term
Kawasaki Disease
Definition
Medium vessel vasculitis with anti-endothelial cell antibodies
Term
Polyarteritis Nodosa
Definition
Medium vessel vasculitis with immune complexes
Term
Giant Cell (Temporal) Arteritis
Definition
Affects primarily arteries in the head
Term
Giant Cell (Temporal) Arteritis
Definition
Medial granulomatous inflammation that leads to elastic lamina fragmentation
Term
Giant Cell (Temporal) Arteritis
Definition
Inflammatory lesions are non-continuous, with stretches of relatively normal artery.
Term
Giant Cell (Temporal) Arteritis
Definition
Occurs predominantly in older age individuals, with vague symptoms or facial pain and headache, sometimes involving the eye
Term
Giant Cell (Temporal) Arteritis
Definition
Degenerated internal elastic lamina and nodular intimal thickening
Term
Takayasu Arteritis
Definition
Ocular disturbances and weakening of the upper extremity pulses
Term
Takayasu Arteritis
Definition
"Pulseless Disease"
Term
Takayasu Arteritis
Definition
Transmural fibrous thickening of the aorta, and luminal narrowing of the arotic arch vessels
Term
Takayasu Arteritis
Definition
Shares many attributes with giant-cell arteritis, and diagnosis is often on the basis of age being less than 50
Term
Takayasu Arteritis
Definition
Classically involves the aortic arch
Term
Takayasu Arteritis
Definition
Histological appearance is indistinguishable from giant-cell arteritis
Term
Takayasu Arteritis
Definition
Younger ages, non-specific symptoms, with extremity weakness and ocular disturbances
Term
Polyarteritis Nodosa
Definition
Medium artery vasculitis with segmental necrosis and without ANCA
Term
Polyarteritis Nodosa
Definition
Medium artery vasculitis sometimes associated with hepatitis B
Term
Polyarteritis Nodosa
Definition
Hepatitis B Antigen-Antibody complexes found in affected medium arteries
Term
Polyarteritis Nodosa
Definition
Segmental transmural necrotizing inflammation of small to medium-size arteries
Term
Polyarteritis Nodosa
Definition
Commonly involves medium arteries in the kidneys, heart and liver
Term
Giant Cell (temporal) Arteritis -or- Takayasu Arteritis
Definition
Infiltrate consists of CD4+ and CD8+ T-cells and multinucleated giant cells
Term
Polyarteritis Nodosa
Definition
Transmural inflammation of the arterial wall with a mixed infiltrate of neutrophils, eosinophils, and mononuclear cells and fibrinoid necrosis
Term
Polyarteritis Nodosa
Definition
Initial Fibrinoid necrosis followed by fibrous thickening of the wall of medium arteries
Term
Polyarteritis Nodosa
Definition
Medium sized artery disease, only part of the circumference of the vessel wall affected
Term
Polyarteritis Nodosa
Definition
Young person with severe hypertension due to renal artery vasculitis
Term
Kawasaki Disease
Definition
Acute, febrile, usually self-limited illness of infancy or childhood
Term
Kawasaki Disease
Definition
Child with fever and acute myocardial infact
Term
Giant-Cell (Temporal) Arteritis
Definition
Unexplained predilection for a single vascular site
Term
Kawasaki Disease
Definition
T-Cell cytokines and B-Cell autoantibodies injuring endothelial cells and smooth muscle cells of medium arteries
Term
Polyarteritis Nodosa
Definition
Predilection for branch points and partial circumference lesions
Term
Microscopic Polyangiitis
Definition
Necrotizing vasculitis that affects capillaries as well as arterioles and venules
Term
Microscopic Polyangiitis
Definition
Also called Leukoclastic vasculitis
Term
Microscopic Polyangiitis
Definition
All lesions tend to be the same age in a given patient
Term
Microscopic Polyangiitis
Definition
Necrotizing glomerulonephritis
Term
Microscopic Polyangiitis
Definition
Necrotizing glomerulonephritis and pulmonary involvment.
Term
Microscopic Polyangiitis
Definition
Necrotizing vasculitis of medium and small vessels with "pauci-immune" lesions
Term
Microscopic Polyangiitis
Definition
Segmental fibrinoid necrosis of media with focal transmural necrotizing lesions, but sparing of medium and larger arteries
Term
Microscopic Polyangiitis
Definition
Hematuria, palpable purpura and hemoptysis aftter a course penicillin
Term
Churg-Strauss Syndrome
Definition
"Allergic granulomatosis"
Term
Churg-Strauss Syndrome
Definition
Small vessel vasculitis associated with asthma, allergic rhinitis, lung infiltrates, peripheral hypereosinophilia and extravascular necrotizing granulomas
Term
Churg-Strauss Syndrome
Definition
Asthma, eosinophilia, and palpable purpura
Term
Churg-Strauss syndrome
Definition
Asthma, eosinophilia, and cardiomyopathy
Term
Churg-Strauss Syndrome
Definition
sometimes pANCA positive, can cause cardiomyopathy but not infarction
Term
Wegener Granulomatosis
Definition
Acute necrotising granulomas of upper respiratory tract, small-medium vessels, focal necrotizing crescentric glomerulonephritis
Term
Wegener Granulomatosis
Definition
Necrotizing granuloma, limited forms may be restricted to the respiratory tract
Term
Wegener Granulomatosis
Definition
PR3-ANCAs usually present
Term
Wegener Granulomatosis
Definition
Small vessel vasculitis affecting respiratory tract, cANCA positive (PR3)
Term
Wegener Granulomatosis
Definition
Ulcerative lesion of the nose
Term
Wegener Granulomatosis
Definition
Necrosis of glomerular capillary loops leading to parietal cell proliferation crescents
Term
Wegener Granulomatosis
Definition
Males affected more than females, average age around 40
Term
Wegener Granulomatosis
Definition
35 year old man with pneumonitis, bilateral nodular cavitary infiltrates and chronic sinusitis
Term
Thromboangiitis Obliterans (Buerger Disease)
Definition
Segmental, thrombosing, acute and chronic inflammation of medium and small arteries, often tibial and radial arteries
Term
Thromboangiitis Obliterans (Buerger Disease)
Definition
Male heavy cigarette smokers 20-40 years old
Term
Thromboangiitis Obliterans
Definition
Both genetic and smoking related
Term
Thromboangiitis Obliterans
Definition
Sharply segmental acute and chronic vasculitis of medium and small arteries, with thrombus and microabcesses
Term
Thromboangiitis Obliterans (Buerger Disease)
Definition
Inflammatory process uniquely extends into contiguous veins and nerves
Term
Thromboangiitis Obliterans (Buerger Disease)
Definition
Raunaud's Phenomenon, nodular phlebitis and pain in the medial foot
Term
Thromboangiitis Obliterans (Buerger Disease)
Definition
Foot pain induced by exercise and raunaud phenomenon
Term
Thromboangiitis Obliterans (Buerger Disease)
Definition
30 year old male with ulceration of the toes, pain at rest and history of smoking
Term
Cryoglobulinemia
Definition
Old man with hepatitis C and Raynaud phenomenon.
Term
Polyarteritis Nodosa
Definition
35 year old IV drug abuser with chronic hepatitis B, positive p-ANCA titer, blood pressure 230/140 and acute renal failure.
Term
Henoch–Schonlein purpura
Definition
12 year old recently recovered from upper respiratory infection now has abdominal pain, joint pain, palpable purpura of the lower extremities and hematuria.
Term
Giant Cell Arteritis
Definition
67 year old woman with recent pain chewing, headaches and blurred vision, with normal head CT and MRI.
Term
Wegener Granuloma - cANCA (PR3) 95% cases, likely contributive
Churg-Strauss - pANCA (MPO) ~50% cases, probably contributive when present
Microscopic Polyangiitis - pANCA associated hypothesis
Kawasaki - anti-entothelial hypothesis
Definition
Vasculitides that may involve autoantibody mediated mechanisms
Term
Giant Cell Arteritis
Takayasu Arteritis
Definition
The two major large vessel vasculitides
Term
Polyarteritis Nodosa
Definition
A strictly small-medium vessel vasculitis
Term
Giant Cell Arteritis
Takayasu Arteritis
Wegener Granuloma
Definition
Vasculitides with characteristically granulomatous lesions
Term
Kawasaki Disease
Definition
Affects large to medium, and sometimes small, arteries.
Term
Polyarteritis Nodosa
Definition
Affects small or medium muscular arteries, but not arterioles, capillaries, or venules.
Term
1) Polyarteritis Nodosa - "typically involving renal and visceral vessels but sparing the pulmonary circulation"
2) Takayasu Arteritis - about half of patients
3) Giant Cell - rare cases- "Giant Cell Aortitis"
4) Microscopic Polyangiitis - unlikely but possible (medium arteries)
5) Kawasaki unlikely but possible (medium arteries)
Definition
Vasculitides capable of producing renovascular hypertension, in order of likelihood
Term
Polyarteritis Nodosa
Kawasaki Disease
Definition
Medium vessel vasculitis usually involving transmural inflammation
Term
Wegener's Granulomatosis
Definition
cANCAs usually present
Term
Polyarteritis Nodosa
Definition
anti-myeloperoxidase antibodies present, associated with Hepatitis B
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