Classification of Vasculitis

Question 1

Large-sized vessels: aorta, renal artery

Answer 1

Granulomatous arteritis: giant cell or Takayasu arteritis

Question 2

Medium-sized vessels: renal artery, medium-sized vessels within the kidneys (interlobar artery, arcuate artery)

Answer 2

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.

Question 3

Medium-sized vessels: renal artery, medium-sized vessels within the kidneys (interlobar artery, arcuate artery)

Answer 3

Without mucocutaneous lymph node (MCLN) syndrome: PAN

With MCLN syndrome: Kawasaki disease

Question 4

Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)

Answer 4

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

Question 5

Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)

Answer 5

Systemic lupus erythematosus (SLE) or rheumatoid arthritis

Others: postinfectious, hypocomplementemic urticarial (anti-C1q) vasculitis

Question 6

Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)

Answer 6

Circulating ANCA with paucity of vascular or glomerular immunoglobulin staining:

Granulomas and no asthma: granulomatous polyangiitis (Wegener’s)

Question 7

Small-sized vessels: Smaller vessels within the kidneys (interlobar artery, arcuate artery, interlobar artery, arterioles)

Answer 7

Eosinophilia, asthma, and granuloma: eosinophilic granulomatosus with polyangiitis (Churg–Strauss)

No asthma or granulomas: microscopic polyangiitis

Question 8

Review

Large sized vasculitis let’s talk about Giant Cell Arteritis

Answer 8

The age of onset is typically over 15 years

Female to Male ratio is 4:1

Pathogenesis is Unknown

Question 9

Review

Large sized vasculitis let’s talk about Giant Cell Arteritis

Answer 9

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.

Question 10

Review

Large sized vasculitis let’s talk about Giant Cell Arteritis

Answer 10

Renal involvement is rare compared with Takayasu’s. Hypertension is possible.

Question 11

Review

Large sized vasculitis let’s talk about Giant Cell Arteritis

Answer 11

Pathology includes renal arteries which may be involved but significant disease is rare.

Question 12

Review

Large sized vasculitis let’s talk about Giant Cell Arteritis

Answer 12

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.

Question 13

Review

Large sized vasculitis let’s talk about Giant Cell Arteritis

Answer 13

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.

Question 14

Review

Large sized vasculitis: Let’s talk about Takayasu’s arteritis.

Answer 14

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.

Question 15

Review

Large sized vasculitis: Let’s talk about Takayasu’s arteritis.

Answer 15

Clinical manifestations include reduced pulses, vascular bruits, claudication, renal ischemia due to renal artery stenosis or aortic coarctation. Hypertension is also possible.

Question 16

Review

Large sized vasculitis: Let’s talk about Takayasu’s arteritis.

Answer 16

Pathology includes ischemic renal disease which is relatively common.
Glomerular sign is possible. Nodular Mesangial matrix expansion is seen. Mesangiolysis is also seen.

Question 17

Review

Large sized vasculitis: Let’s talk about Takayasu’s arteritis.

Answer 17

Treatment is the same as giant cell arteritis.

Question 18

Review

Large sized vasculitis: Let’s talk about Takayasu’s arteritis.

Answer 18

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.

Question 19

Review

Polyarteritis nodosa (PAN)

Answer 19

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.

Question 20

Review

Polyarteritis nodosa (PAN)

Answer 20

Pathogenesis is unknown; immune complex trigger for example hepatitis B was suggested but not confirmed.

There is absence of ANCA.

Question 21

Review

Polyarteritis nodosa (PAN)

Answer 21

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.

Question 22

Review

Polyarteritis nodosa (PAN)

Answer 22

There is no mucocutaneous lymph node involvement.

Question 23

Review

Polyarteritis nodosa (PAN)

Answer 23

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.

Question 24

Review

Polyarteritis nodosa (PAN)

Answer 24

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.

Question 25

Review

Polyarteritis nodosa (PAN)

Answer 25

Note light microscopy changes of involved vessels are indistinguishable from ANCA associated glomerulonephritis.

Question 26

Review

Polyarteritis nodosa (PAN)

Answer 26

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.

Question 27

Review

Kawasaki

Answer 27

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.

Question 28

Review

Kawasaki

Answer 28

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.

Question 29

Review

Kawasaki

Answer 29

Pathogenesis is not clear. Possible precipitating factors include infectious agent or environmental toxin. Both cell and antibody mediated mechanisms are possible.

Question 30

Review

Kawasaki

Answer 30

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.

Question 31

Review

Kawasaki

Answer 31

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.

Question 32

Review

Kawasaki

Answer 32

Pseudo-aneurysm formation and thrombosis may also occur.

Question 33

Review

Kawasaki

Answer 33

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.