14. Vasculitis

Question 1

Vascultits

Answer 1

Blood vessel wall inflammation

2 common pathogenic mechanisms

Immune mediated
Direct invasion by pathogen

Question 2

Noninfectious immune complex associated vasculutis

Answer 2

Immune complex deposition- of circulating antigen-antibody complexes

Vascular injury due to recruitment of inflammatory cells \

ANCA -can present on neutrophils cause release of enzymes and ROS that damage endothelium

Question 3

Pathogenesis of vascular lesions in ANCA vasculitis

Answer 3

1. Cytokines prime neutrophils and present ANCA
2. Nuetriophils take in ANCA antigen and present on its cell surface
3. Release proteolytic enzymes Causing vasilitis
4. Acute inflammation is replaced with macro, lymphocyes scarring and fibrosis

Question 4

Giant cell arthritis

Answer 4

Non ANCA associated

Granulamtomatous inflammation

PAthogenesis:

1. Activation of T cells
2. Formation of multinuceliated giant cells
3. Amplification cascades leading to inflammation
4. Vascular remodeling and occulsion = ischemia and necrosis

Clinical features:

Fever, fatigue, headache
More prevent in aging adults, can experience vision loss

Question 5

Polyarteritis nodosa (PAN)

Answer 5

NOT ANCA

Idiopathic

Necrotizing vasculaities in small and medium arteries mostly in kidney

Pathogenesis:

1. Thickening of inflamed vessel walls - intimal proliferation
2. Luminal narrowing
3. Thrombosis of affected areas

Clinical features:
Multisystemic
Renal arteries will affect blood flow, likely causing HT

Question 6

Granulomatosis with poly angst is (widener granulmatosis)

Answer 6

ANCA associated

TRiad: ENT

Pathogenesis:
MPO target 10% of cases
Proteinase 3 (PR3) observed in 80-90%, increased ANCA associated PR3

Clinical features

Fever, anorexia, weight loss, ENT manifestations