Vasculitis Flashcards
Vasculitis is a heterogeneous group of disorders linked by what primary finding?
Inflammation within blood vessel walls; at least 20 forms os systemic vasculitis are recognized currently
Vasculitides are classified by what first?
Size of the blood vessel involved; Small = caps and post cap venules, medium = musc arteries/arterioles, large = aorta and its major branches
Additional considerations in classifications include what?
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
Different form of vasculitis have widely divergent profiles with regard to?
Age, gender, ethnicity
Associations between what have been recognized increasingly in recent years, there has also been progress in the area of what interactions?
Associations between genes and vasculitis and interactions between genes then the environment
Vasculitis mediated by immune complexes (ICs) includes a heterogeneous group of disorders linked by?
Inefficient or dysregulated clearance of ICs
The most common types of IC mediated vasculitis are what?
Hypersensitivity vasculitis, HSP, and mixed cryoglobulinemia; rarer forms are hypocomplementemic urticarial vasculitis and erythema elevatum diutinum
Connective tissue disorders such as what can be associated with IC Vasculitis?
SLE, Sjogren’s syndrome, and rheumatoid arthritis
What is the most prominent feature in the majority of cases of small blood vessels in IC Vasculitis
Cutaneous involvement, extracutaneous in some too
The classic cutaneous finding in small vessel vasculitis is what?
Palpable purpura, but a variety of other lesions include pustules, vesicles, urticaria, and small ulcerations
Direct immunofluorescence studies of involved blood vessels demonstrate what?
Characteristic types and patterns of Ig and complement deposition
HS Vasculitis usually results from what?
A reaction to a medication or an infection
HSP is associated with?
Purpura, arthritis, glomerulonephritis, and colicky abdominal pain; IgA deposition is found within BV walls
Cryoglobulinemic Vasculitis is most often seen with what? Why is it sometimes called mixed?
Long standing Hep C infection; the immunoreactants involved in the dz include both IgG and IgM
Granulomatosis w/ polyangiitis (GPA), microscopic polyangiitis (MPA), and allergic granulomatosis with polyangiitis (AGPA) are what?
Forms of vasculitis that affect small to medium sized vessels and share a number of clinical, pathologic features, and laboratory features
Animal models, in vitro studies, and clinical studies support what in most patients with GPA, MPA, or AGPA?
Pathogenic role for ANCAs
Testing for ANCA and more specific auto antibodies by immunoassay it useful for dx of small vessel vasc, what does it do for monitoring?
It’s controversial, don’t really know
GPA can affect any organ or tissue but has predilection for what? How is it most commonly associated with ANCA?
U/LRT and kidneys; Through imunofluorescence and positive testing for the proteinase-3 antigen
MPA can be distinguished from other forms of small vessel vasculitis by what?
Absence of granuloma formation and the predominance of perinuclear ANCA staining by IF and positive testing for the myeloperoxidase antigen
AGPA can be distinguished from other forms of small vessel vasculitis by what?
The basis of a prior hx of adult-onset asthma or allergic rhinitis and tissue eosinophilia with necrotizing vasculitis and extravascular granuloma formation
Primary angiitis of the CNS (PACNS) is defined as what?
Vasculitis confined only to the brain, meninges, or spinal cord
PACNS consists of subsets including what? How are they differentiated?
Granulomatous (GACNS) and atypical PACNS that are differentiated by histologic and imaging
Dx of PACNS is based on?
Compatible clinical features together with evidence from spinal fluid, brain and vascular imaging, and often brain biopsy
What else is central to the dx of PACNS?
Ruling out mimics that have similar clinical or angiographic appearance
Reversible cerebral vascoconstriction syndrome (RCVS) is what?
One of the most important mimickers of PACNS because it has treatment and outcome implications
Outcome of PACNS is?
Variable; highest disability and mortality rate in GACNS
What is Behcet’s dz?
Complex multisystem dz characterized by oral and genital ulcers and other systemic features
Diagnosis of Behcet’s is based on what?
International Criteria for Behcet’s Dz including oral apthae, genital apthae, ocular lesions, cutaneous lesions, and a positive pathergy test
Cutaneous lesions should display what for Behcet’s Dz?
Neutrophilic vascular reaction
Treatment of Behcet’s Dz is usually?
Based on the degree of systemic involvement and ranges from topical corticosteroids to thalidomide to systemic immunosuppressive agents and TNF inhibitors
Prognosis of Behcet’s dz is?
Variable, patients typically have high periods of exacerbations and remissions
