(IV) Vasculitides

Question 1

1. What is the definition of vasculitis? What are the vascular consequences?

Answer 1

Inflammation and necrosis of a blood vessel with subsequent impairment of blood flow. Vessel
wall destruction ->perforation and hemorrhage into adjacent tissues. Thrombosis -> impairment of blood flow -> ischemia/infarction of dependent tissues. Complx: accelerated 2/2 ATH of the involved vessel, which contributes to
morbidity and mortality.

Question 2

Histologic features of vasculitis?

Answer 2

Infiltration of the vessel wall by neutrophils, mononuclear cells, and/or giant cells
• Fibrinoid necrosis (panmural destruction of the vessel wall)
• Leukocytoclasis (dissolution of leukocytes, yielding “nuclear dust”)
Perivascular infiltration is a nonspecific histologic finding & not diagnostic of vasculitis,

Question 3

immune mechanism does vasculitis occur?

Answer 3

Cell-mediated vasculitis:
GCA
Takayasu arteritis,
Primary CNS vasculitis

IC mediated vasculitis:
PAN
HSP
cryoglobulinemic vasculitis,
cut leukocytoclastic angiitis. 

ANCA-assoc vasculitides: 
(+/- cellular & humoral)
GPA
MPA
EGPA

Question 4

Cell-mediated vasculitis

Answer 4

Cell-mediated vasculitis:
GCA
Takayasu arteritis,
Primary CNS vasculitis

Question 5

IC med vasculitis

Answer 5

IC mediated vasculitis:
PAN
HSP
Cryoglobulinemic vasculitis,
Cut leukocytoclastic angiitis.

Question 6

AAV

Answer 6

ANCA-assoc vasculitides: 
(+/- cellular & humoral)
GPA
MPA
EGPA

Question 7

Abbrev?
GCA
PAN
HSP

GPA
MPA
EGPA

Answer 7

GCA
PAN
HSP

GPA
MPA
EGPA

Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss)

Question 8

Chapel Hill

Answer 8

Large
Med
Small
Variable
Single organ
Assoc with sys dz
Assoc with probably cause

Question 9

Large vessel

Answer 9

Takayasu & GCA

Question 10

Medium vessel

Answer 10

PAN & Kawasaki

Question 11

Small vessel

Answer 11

• IC-mediated*
• Anti-GBM
• Cryoglobulinemic vasculitis
• IgA vasculitis(HSP
• Hypocomplementemic urticarial (HUVS, anti-Clq vasculitis)
• ANCA(pauci-immune)*
• GPA
• MPA
• EPA

Question 12

Variable vessel

Answer 12

Bechet & Cogan

Question 13

Single Organ vasc

Answer 13

Cutaneous leukocytoclastic vasculitis
Cutaneous arteritis
Primary CNS
Isolated aortitis
Others

Question 14

vasc with sys dz

Answer 14

SLE
RA
Sarcoid & others

Question 15

Vasc with probable eitology

Answer 15

Hep C cryoglobulinemic vasculitis
Hep B vasculitis
Syphilis vasculitis
Drug-assoc IC vasculitis (HSR)
Drug-assoc ANCA vasculitis
Cancer vasculitis
Others

Question 16

Presentations

Answer 16

Large-:
limb claudication,
bruits, asymmetric BP, absence of pulses

• Medium:-: 
cutaneous nodules, 
ulcers, 
livedo reticularis, 
digital gangrene, mononeuritis multiplex, renovascular HTN

• Small: 
palpable purpura, 
urticaria, 
glomerulonephritis,
alveolar hemorrhage, 
scleritis

Question 17

PEARL

MCC Presentation for vasc

Answer 17

PEARL:
Headache or visual loss in the elderly->GCA

asymmetric pulses + bruits + <30 y/o ->Takayasu

mononeuritis multiplex->PAN

rapidly prog pulm–renal syn->AAV

palpable purpura->IC mediated vasculitis

Question 18

When to suspect vasc mimics??

Answer 18

Vasculitis mimics should be suspected when there is:
1. A new heart murmur (subacute bacterial endocarditis, SBE)

2. Necrosis of lower-extremity digits (cholesterol emboli)
3. Splinter hemorrhages (SBE)
4. Prominent liver dysfunction (hepatitis C)
5. Drug abuse (human immunodeficiency virus, HIV; hepatitis B/C; cocaine, etc.)
6. Prior diagnosis of neoplastic disease
7. Unusually high fever (SBE)
8. History of high-risk sexual activity (HIV)

Question 19

What disorders can mimic Large arteries?

Answer 19

Fibromuscular dysplasia, radiation fibrosis, neurofibromatosis type I, congenital coarctation of
aorta, genetic diseases (Marfan syndrome, Loeys–Dietz syndrome), syphilitic aortitis, IgG4 disease

Question 20

What disorders can mimic Medium arteries ?

Answer 20

Cholesterol emboli syndrome, atrial myxoma, fibromuscular dysplasia, lymphomatoid granulomatosis,
angioblastic T-cell lymphoma, thromboembolic disease, ergotism, type IV Ehlers–Danlos syndrome,
segmental arterial mediolysis, Grange syndrome, pseudoxanthoma elasticum

Question 21

What disorders can mimic Cerebral arteries ?

Answer 21

Reversible cerebral vasoconstrictive syndrome, reversible posterior encephalopathy syndrome,
cerebral amyloid angioapthy, CADASIL syndrome, Susac syndrome, progressive multifocal leukoencephalopathy,
Moyamoya disease, intravascular lymphoma, infections

Question 22

What disorders can mimic Small arteries ?

Answer 22

Infectious endocarditis, mycotic aneurysm with emboli, cholesterol microemboli syndrome, antiphospholipid
antibody syndrome, sepsis (gonococcal, meningococcal), ecthyma gangrenosum (Pseudomonas),
thrombotic thrombocytopenia purpura, cocaine, amphetamines, minocycline, hydralazine, HIV, hepatitis
C, amyloidosis, systemic rheumatic diseases (systemic lupus erythematosus, SLE; Sjögren’s syndrome), bacteremias
(SBE, Rickettsia), other systemic viral infections, common variable immunodeficiency, calciphylaxis,
livedoid vasculopathy (atrophie blanche), malignant atrophic papulosis (Degos disease)

Question 23

To rule out vasculitis mimics:,

Answer 23

consider blood cultures, viral hepatitis studies, HIV testing, urinary toxicology
screening, echocardiography, antinuclear antibody (ANA), rheumatoid factor (RF), antiphospholipid antibodies,
and/or angiography/magnetic resonance angiography, depending on the clinical situation

Question 24

Jaw claudication, visual loss, palpable, thickened,
tender temporal artery, or

Diminished temporal artery pulsation

Answer 24

Giant cell arteritis (GCA)

Question 25

Absent radial pulses, difficulty obtaining a blood pressure | in one arm

Answer 25

Takayasu arteritis or large artery involvement in GCA

Question 26

Sinus involvement, otitis media, scleritis

Answer 26

GPA (Wegener) or EGPA (Churg–Strauss syndrome)

Question 27

Hypertension, renal vascular involvement

Answer 27

Polyarteritis nodosa or Takayasu arteritis

Question 28

Asthma

Answer 28

EGPA (Churg–Strauss syndrome)

Question 29

Pulmonary–renal syndromes (hemoptysis and glomerulonephritis)

Answer 29

GPA (Wegener) and microscopic polyangiitis

Question 30

Testicular tenderness

Answer 30

Polyarteritis nodosa

Question 31

subcutaneous nodules, “punched-out” skin ulcers, livedo reticularis, digital gangrene

Answer 31

Medium-vessel vasculitis:

Question 32

palpable purpura, splinter hemorrhages, hemorrhagic macules, vesiculobullous lesions, and urticaria lasting >24 hours

Answer 32

Small-vessel vasculitis

Question 33

Primary systemic vasculitis never causes pancytopenia (must rule out SLE, B cell lymphoma, myeloma, leukemia)

Answer 33

Westergren ESR and CRP. ESR >100 mm/h and CRP >10 mg/dL in the absence of bacterial infection and widespread cancer should suggest vasculitis.

Question 34

c-ANCA

Answer 34

if against serine proteinase 3, usually GPA; sometimes MPA | highly specific for GPA with widespread systemic involvement >90%

Question 35

p-ANCA

Answer 35

if against myeloperoxidase, consider MPA and EGPA; sometimes GPA. If the p-ANCA is not against myeloperoxidase, inflammatory diseases other than vasculitis should be considered (inflammatory bowel disease, infections). In some patients, ANCAs may have predictive value for relapses and ongoing disease activity. ANCAs may be pathogenic as part of several simultaneous or sequential events.

Question 36

When are hepatitis serologies helpful when vasculitis is suspected?

Answer 36

The presence of hepatitis B surface antigen may be found in some patients (10% to 25%, depending on risk factors) with PAN. Hepatitis C antibodies are often found in patients with essential mixed cryoglobulinemic vasculitis and rarely in PAN.

Question 37

What is the role of tissue biopsy in the diagnosis of vasculitis and in what type of vasculitis might tissue biopsy be helpful?

Answer 37

Tissue biopsy is unquestionably the procedure of choice in the diagnosis of vasculitis. Some frequently approached biopsy sites are as follows: • Common sites: Skin, sural nerve (PAN, EGPA; only biopsy if abnormal electromyogram [EMG]/nerve conduction velocity [NCV]), temporal artery (GCA), muscle (PAN), kidney (GPA, MPA; rare to see vasculitis, usually see focal necrotizing glomerulonephritis with or without crescents), lung (GPA, MPA) • Less common sites: Testicle (PAN), rectum/gut, liver, heart, brain (primary CNS vasculitis), sinus (GPA)

Question 38

16. If tissue biopsy is not feasible, what alternative procedures can yield a diagnosis?

Answer 38

Angiography at the following sites may be helpful for diagnosis of certain types of vasculitis: • Abdomen (celiac trunk, superior mesenteric, and renal arteries) for diagnosis of PAN • Aortic arch for diagnosis of Takayasu arteritis and GCA with large-vessel involvement. • Extremities for diagnosis of Buerger disease • Cerebral sites for diagnosis of primary CNS vasculitis

Question 39

List two characteristic (but not diagnostic) angiographic features of vasculitis

Answer 39

Irregular tapering and narrowing & Aneurysms (“beading”)

Question 40

noninvasive tests can be used to determine vessel involvement in patients with vasculitis?

Answer 40

Doppler ultrasound of temporal arteries can localize the area of narrowing in GCA and help determine biopsy sites. • Magnetic resonance angiography of the aorta can reveal aortic wall thickening (enhancement with gadolinium indicates inflammation) and areas of stenosis in patients with Takayasu arteritis or GCA with large-artery involvement. • Positron emission tomography can reveal enhancement of the aortic and subclavian vessel wall if active inflammation occurs in patients with Takayasu arteritis and GCA