Vasculitis

Question 1

Vasculitis is a heterogeneous group of disorders linked by what primary finding?

Answer 1

Inflammation within blood vessel walls; at least 20 forms os systemic vasculitis are recognized currently

Question 2

Vasculitides are classified by what first?

Answer 2

Size of the blood vessel involved; Small = caps and post cap venules, medium = musc arteries/arterioles, large = aorta and its major branches

Question 3

Additional considerations in classifications include what?

Answer 3

Demographics, organ tropism, presence or absence of granulomatous inflammation, participation (or lack of) immune complexes, finding of auto antibodies, detection of infections associated with some vasculitides

Question 4

Different form of vasculitis have widely divergent profiles with regard to?

Answer 4

Age, gender, ethnicity

Question 5

Associations between what have been recognized increasingly in recent years, there has also been progress in the area of what interactions?

Answer 5

Associations between genes and vasculitis and interactions between genes then the environment

Question 6

Vasculitis mediated by immune complexes (ICs) includes a heterogeneous group of disorders linked by?

Answer 6

Inefficient or dysregulated clearance of ICs

Question 7

The most common types of IC mediated vasculitis are what?

Answer 7

Hypersensitivity vasculitis, HSP, and mixed cryoglobulinemia; rarer forms are hypocomplementemic urticarial vasculitis and erythema elevatum diutinum

Question 8

Connective tissue disorders such as what can be associated with IC Vasculitis?

Answer 8

SLE, Sjogren’s syndrome, and rheumatoid arthritis

Question 9

What is the most prominent feature in the majority of cases of small blood vessels in IC Vasculitis

Answer 9

Cutaneous involvement, extracutaneous in some too

Question 10

The classic cutaneous finding in small vessel vasculitis is what?

Answer 10

Palpable purpura, but a variety of other lesions include pustules, vesicles, urticaria, and small ulcerations

Question 11

Direct immunofluorescence studies of involved blood vessels demonstrate what?

Answer 11

Characteristic types and patterns of Ig and complement deposition

Question 12

HS Vasculitis usually results from what?

Answer 12

A reaction to a medication or an infection

Question 13

HSP is associated with?

Answer 13

Purpura, arthritis, glomerulonephritis, and colicky abdominal pain; IgA deposition is found within BV walls

Question 14

Cryoglobulinemic Vasculitis is most often seen with what? Why is it sometimes called mixed?

Answer 14

Long standing Hep C infection; the immunoreactants involved in the dz include both IgG and IgM

Question 15

Granulomatosis w/ polyangiitis (GPA), microscopic polyangiitis (MPA), and allergic granulomatosis with polyangiitis (AGPA) are what?

Answer 15

Forms of vasculitis that affect small to medium sized vessels and share a number of clinical, pathologic features, and laboratory features

Question 16

Animal models, in vitro studies, and clinical studies support what in most patients with GPA, MPA, or AGPA?

Answer 16

Pathogenic role for ANCAs

Question 17

Testing for ANCA and more specific auto antibodies by immunoassay it useful for dx of small vessel vasc, what does it do for monitoring?

Answer 17

It’s controversial, don’t really know

Question 18

GPA can affect any organ or tissue but has predilection for what? How is it most commonly associated with ANCA?

Answer 18

U/LRT and kidneys; Through imunofluorescence and positive testing for the proteinase-3 antigen

Question 19

MPA can be distinguished from other forms of small vessel vasculitis by what?

Answer 19

Absence of granuloma formation and the predominance of perinuclear ANCA staining by IF and positive testing for the myeloperoxidase antigen

Question 20

AGPA can be distinguished from other forms of small vessel vasculitis by what?

Answer 20

The basis of a prior hx of adult-onset asthma or allergic rhinitis and tissue eosinophilia with necrotizing vasculitis and extravascular granuloma formation

Question 21

Primary angiitis of the CNS (PACNS) is defined as what?

Answer 21

Vasculitis confined only to the brain, meninges, or spinal cord

Question 22

PACNS consists of subsets including what? How are they differentiated?

Answer 22

Granulomatous (GACNS) and atypical PACNS that are differentiated by histologic and imaging

Question 23

Dx of PACNS is based on?

Answer 23

Compatible clinical features together with evidence from spinal fluid, brain and vascular imaging, and often brain biopsy

Question 24

What else is central to the dx of PACNS?

Answer 24

Ruling out mimics that have similar clinical or angiographic appearance

Question 25

Reversible cerebral vascoconstriction syndrome (RCVS) is what?

Answer 25

One of the most important mimickers of PACNS because it has treatment and outcome implications

Question 26

Outcome of PACNS is?

Answer 26

Variable; highest disability and mortality rate in GACNS

Question 27

What is Behcet’s dz?

Answer 27

Complex multisystem dz characterized by oral and genital ulcers and other systemic features

Question 28

Diagnosis of Behcet’s is based on what?

Answer 28

International Criteria for Behcet’s Dz including oral apthae, genital apthae, ocular lesions, cutaneous lesions, and a positive pathergy test

Question 29

Cutaneous lesions should display what for Behcet’s Dz?

Answer 29

Neutrophilic vascular reaction

Question 30

Treatment of Behcet’s Dz is usually?

Answer 30

Based on the degree of systemic involvement and ranges from topical corticosteroids to thalidomide to systemic immunosuppressive agents and TNF inhibitors

Question 31

Prognosis of Behcet’s dz is?

Answer 31

Variable, patients typically have high periods of exacerbations and remissions