(IV) Vasculitides Flashcards Preview

Rheum Secrets 3E > (IV) Vasculitides > Flashcards

Flashcards in (IV) Vasculitides Deck (40):
1

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

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.

2

Histologic features of vasculitis?

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,

3

immune mechanism does vasculitis occur?

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

4

Cell-mediated vasculitis

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


5

IC med vasculitis

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


6

AAV

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

7

Abbrev?
GCA
PAN
HSP

GPA
MPA
EGPA

GCA
PAN
HSP

GPA
MPA
EGPA

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

8

Chapel Hill

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

9

Large vessel

Takayasu & GCA

10

Medium vessel

PAN & Kawasaki

11

Small vessel

***IC-mediated***
-Anti-GBM
-Cryoglobulinemic vasculitis
-IgA vasculitis(HSP
-Hypocomplementemic urticarial (HUVS, anti-Clq vasculitis)


***ANCA(pauci-immune)***
-GPA
-MPA
-EPA

12

Variable vessel

Bechet & Cogan

13

Single Organ vasc

Cutaneous leukocytoclastic vasculitis
Cutaneous arteritis
Primary CNS
Isolated aortitis
Others

14

vasc with sys dz

SLE
RA
Sarcoid & others

15

Vasc with probable eitology

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

16

Presentations

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

17

PEARL
MCC Presentation for vasc

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

18

When to suspect vasc mimics??

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)

19

What disorders can mimic Large arteries?

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

20

What disorders can mimic Medium arteries ?

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

21

What disorders can mimic Cerebral arteries ?

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

22

What disorders can mimic Small arteries ?

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)

23

To rule out vasculitis mimics:,

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

24

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

Diminished temporal artery pulsation

Giant cell arteritis (GCA)

25

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

Takayasu arteritis or large artery involvement in GCA

26

Sinus involvement, otitis media, scleritis

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

27

Hypertension, renal vascular involvement

Polyarteritis nodosa or Takayasu arteritis

28

Asthma

EGPA (Churg–Strauss syndrome)

29

Pulmonary–renal syndromes (hemoptysis and glomerulonephritis)

GPA (Wegener) and microscopic polyangiitis

30

Testicular tenderness

Polyarteritis nodosa

31

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

Medium-vessel vasculitis:

32

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

Small-vessel vasculitis

33

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

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.

34

c-ANCA

if against serine proteinase 3, usually GPA; sometimes MPA



highly specific for GPA with widespread systemic involvement
(>90%)

35

p-ANCA

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.

36

When are hepatitis serologies helpful when vasculitis is suspected?

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.

37

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

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)

38

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

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

39

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

Irregular tapering and narrowing & Aneurysms (“beading”)

40

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

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