Classification of Vasculitis Flashcards
Large-sized vessels: aorta, renal artery
Granulomatous arteritis: giant cell or Takayasu arteritis
Medium-sized vessels: renal artery, medium-sized vessels within the kidneys (interlobar artery, arcuate artery)
Necrotizing arteritis: polyarteritis nodosa (PAN) or Kawasaki disease.
NOTE: microaneurysms in PAN may resemble small grapes or “beads on a chain” within the kidneys on angiogram.
Medium-sized vessels: renal artery, medium-sized vessels within the kidneys (interlobar artery, arcuate artery)
Without mucocutaneous lymph node (MCLN) syndrome: PAN
With MCLN syndrome: Kawasaki disease
Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)
Immune complex deposits in vessel walls:
Cryoglobulins: cryoglobulin deposits often affecting both skin and glomeruli
IgA-dominant deposits (HSP): vasculitis involving skin, gut, and glomeruli, with associated arthritis/arthralgias
Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)
Systemic lupus erythematosus (SLE) or rheumatoid arthritis
Others: postinfectious, hypocomplementemic urticarial (anti-C1q) vasculitis
Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)
Circulating ANCA with paucity of vascular or glomerular immunoglobulin staining:
Granulomas and no asthma: granulomatous polyangiitis (Wegener’s)
Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)
Eosinophilia, asthma, and granuloma: eosinophilic granulomatosus with polyangiitis (Churg–Strauss)
No asthma or granulomas: microscopic polyangiitis
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Large sized vasculitis let’s talk about Giant Cell Arteritis
The age of onset is typically over 15 years
Female to Male ratio is 4:1
Pathogenesis is Unknown
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Large sized vasculitis let’s talk about Giant Cell Arteritis
Clinical Manifestations include headaches, temporary artery tenderness, blindness, deafness, jaw claudication, tongue dysfunction, reduced pulses, extremity claudication. More than 50% who have polymyalgia rheumatica - which is stiffness and aching of back, hip, and shoulders.
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Large sized vasculitis let’s talk about Giant Cell Arteritis
Renal involvement is rare compared with Takayasu’s. Hypertension is possible.
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Large sized vasculitis let’s talk about Giant Cell Arteritis
Pathology includes renal arteries which may be involved but significant disease is rare.
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Large sized vasculitis let’s talk about Giant Cell Arteritis
Treatment is the same for both Giant cell and Takayasu’s arteritis and they include glucocorticoids such as prednisolone 1 mg per kilogram per day for one month followed by slow taper over several months. Persistent disease requires prolonged glucocorticoid plus or minus cytotoxic agents such as cyclophosphamide.
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Large sized vasculitis let’s talk about Giant Cell Arteritis
Patients with Giant Cell Arteritis should also be on the low dose aspirin to reduce thrombotic risks.
Surgical bypass or angioplasty may be required when disease is quiescent.
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Large sized vasculitis: Let’s talk about Takayasu’s arteritis.
Age of onset is 10 to 20 years old and it’s very rare after the age of 50.
Female to male ratio is 9:1.
Pathogenesis is unknown.
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Large sized vasculitis: Let’s talk about Takayasu’s arteritis.
Clinical manifestations include reduced pulses, vascular bruits, claudication, renal ischemia due to renal artery stenosis or aortic coarctation. Hypertension is also possible.
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Large sized vasculitis: Let’s talk about Takayasu’s arteritis.
Pathology includes ischemic renal disease which is relatively common.
Glomerular sign is possible. Nodular Mesangial matrix expansion is seen. Mesangiolysis is also seen.
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Large sized vasculitis: Let’s talk about Takayasu’s arteritis.
Treatment is the same as giant cell arteritis.
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Large sized vasculitis: Let’s talk about Takayasu’s arteritis.
Treatment is the same for both Giant cell and Takayasu’s arteritis and they include glucocorticoids such as prednisolone 1 mg per kilogram per day for one month followed by slow taper over several months. Persistent disease requires prolonged glucocorticoid plus or minus cytotoxic agents such as cyclophosphamide.
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Polyarteritis nodosa (PAN)
The age of onset is 40 to 60 years of age
Epidemiology there is a 1:1 female to male ratio.
There is no race predilection.
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Polyarteritis nodosa (PAN)
Pathogenesis is unknown; immune complex trigger for example hepatitis B was suggested but not confirmed.
There is absence of ANCA.
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Polyarteritis nodosa (PAN)
Clinical manifestations include fever, malaise, weight loss, arthralgias, myalgias, skin ulcers, purpura, libido reticularis, infarction, ulceration, mononeuritis multiplex, abdominal pain, blood in stools, flank pain, hematuria, hypertension, life-threatening retroperitoneal bleed due to arterial rupture which is rare, testicular pain, stroke, confusion, cardiomyopathy, and pericarditis.
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Polyarteritis nodosa (PAN)
There is no mucocutaneous lymph node involvement.
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Polyarteritis nodosa (PAN)
Pathology - there is renal artery involvement such as interlobar, arcuate, and interlobular arteries. Involvement of capillaries, arterials, venous beds to exclude polyarteritis nodosa. Nodular inflammatory lesions and aneurysms in arteries are seen.
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Polyarteritis nodosa (PAN)
Acute arterial lesions can be seen and they include segmental transmural fibrinoid necrosis plus or minus leukocyte infiltration. Chronic changes you would see arterial wall erosions from necrotizing inflammation into surrounding perivascular tissues leading to appearance of enlarged lumen, hence “pseudo-aneurysm” and propensity for thrombosis and rupture.
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Polyarteritis nodosa (PAN)
Note light microscopy changes of involved vessels are indistinguishable from ANCA associated glomerulonephritis.
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Polyarteritis nodosa (PAN)
Treatment
if there is no hepatitis B you give Glucocorticoids plus or minus cytotoxic agents such as cyclophosphamide. Glucocorticoids may be adequate if benign such as younger age, no cardiac, gut, or renal involvement. If hepatitis B positive you give high-dose Glucocorticoids tapered for over two weeks followed by antiviral therapy plus or minus plasma exchange.
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Kawasaki
Kawasaki is also a medium-sized vasculitis.
Age of onset is young children, peaks at age 1, and is typically less than five years old.
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Kawasaki
Epidemiology it’s more common in Asians and Polynesians than Caucasians and Blacks. Occasionally it’s an endemic or epidemic pattern, or it can be sporadic.
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Kawasaki
Pathogenesis is not clear. Possible precipitating factors include infectious agent or environmental toxin. Both cell and antibody mediated mechanisms are possible.
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Kawasaki
Clinical manifestations include mucocutaneous lymph node syndrome where you see fevers, mucosal inflammation, swollen, red or strawberry tongue, polymorphous erythemaous rash, indurative edema of extremities, erythema of palms, desquamation from tips of digits, conjunctival injection, lymphadenopathy, coronary arteritis and possible myocardial infarction can be seen.
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Kawasaki
Renal arteritis is uncommon. Pathology is small and medium arteritis with necrotizing inflammation, frequently involving coronaries and renal arteries such as the inter-lobar, arcuate, and interlobular arteries.
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Kawasaki
Pseudo-aneurysm formation and thrombosis may also occur.
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Kawasaki
Treatment is aspirin and IVIG.
Disease is typically self-limited.
Recurrence is rare if properly treated.
Note - glucocorticoids may increase risk of coronary artery aneurysms.
