Escolar Documentos
Profissional Documentos
Cultura Documentos
Definition
A heterogeneous group of disorders linked by
the primary finding of inflammation within
blood vessel walls
Mimics of vasculitis should be excluded
Components of a vasculitis diagnosis
1. Compatible clinical phenotype
2. Exclusion of mimics and secondary causes
3. Supported by specific serology (e.g. ANCA) or
radiology (e.g. angiography)
4. Confirmation by tissue biopsy
Type of vessels
Considerations in the Classifications of
Systemic Vasculitis
Size of predominant blood vessels affected
Pathologic features
Granulomatous inflammation
Immune complex deposition versus pauci-immune
histopathology
Linear staining along glomerular basement
membrane
Presence of antibodies : ANCA, anti-GBM Ab,
RF in serum
ANCA-associated Small-vessel
vasculitis
Small-vessel vasculitis is defined as vasculitis
that affects vessels smaller than arteries
1. Microscopic polyagiitis
2. Granulomatosis with polyangiitis (Wegeners
granulomatosis)
3. Eosinophilic granulomatosis with polyangiitis
(Churg Strauss syndrome)
What are ANCA?
Specific antibodies for antigens in cytoplasmic
granules of neutrophils and monocyte
lysosomes
What are ANCA?
ANCAs were originally described based on their
immunofluorescence patterns
cytoplasmic (c-ANCA) and perinuclear (p-ANCA)