Vasculitis Pathology Flashcards
Vasculitis
- heterogenous group of disease
- mostly autoimmune
- mostly treatable
- some life threatening
Signs and Symptoms of Vasculitis
- fever, myalgias, arthralgias, malaise
- palpable purpura, focal skin necrosis, ulveration
- livedo reticularis, urticaria, myositis
- peripheral neuropathy, GI ulcers/perforation
- intussusception
- pancreatitis, hemoptysis, nodular pulmonary infiltrates, hematuria, proteinuria, organ ischemia, organ infarction
Palpable Purpura
erythematous tender skin nodules
<1cm
Pathology of Temporal (Giant Cell) Arteritis
Inflammation
1) segmental
2) transmural
3) granulomatous w/multinucleate giant cells centering on internal elastic lamina (destroying int)
- lymphocytes, intimal thickening, cell proliferation, luminal stenosis
Temporal Arteritis Symptoms
- Headache (75%)
- Swollen tender artery (50%)
- Scalp tenderness (50%)
- Visual disturbances (50%)
- Jaw claudication (50%)
fever, malaise, weight loss
Pathology of Takayasu Arteritis
Inflammation
1) segmental
2) transmural
3) necrotizing
4) loosely granulomatous with multinucleate giant cells
- lymphocytes, intimal and adventitial thickening, medial loss of elastic fibers, thinning, luminal stenosis
- can have aneurysm*
Polyarteritis Nodosa Pathology
1) segmental
2) transmural
3) nodular arteritis
-with fibrinoid necrosis (most characteristic)
-neutrophils (acute), intimal thickening, cell proliferation and degeneration, luminal stenosis
+/- thrombosis, occlusion, rupture - especially branchpoints
Later: chronic inflammation, fibroblasts, scaring +/- aneurysms
-lesions at different phases at same time
Kawasaki disease Pathology
-endothelial necrosis
+transmural inflammation with neutrophils, lymphocytes, wall necrosis, aneurysms, thrombosis
aneurysms are characteristic (tunica media)
-no blood test, “clinical diagnosis” H&P
Pathology of Granulomatosis with Polyangiitis
Wegeners
-Necrotizing granulomatous vasculitis
of both arteries and veins in upper and lower
respiratory tracts and kidneys
-“Geographic” areas of necrosis
with basophilic debris (blue on H&E stain)
and palisaded histiocytes around them
(like rheumatoid nodules)
-95% have anti-neutrophil cytoplasmic
autoantibodies against proteinase-3
(ANCA-PR3) C-ANCA
Wegeners Upper Respiratory Pathology
- mucosal ulcers
- necrotizing lesions
- destructive of nasal cartilage leading to saddle nose deformity
Wegeners Lung Pathology
-necrotizing lesions (largest and most advanced=cavitating)
Wegeners Kidney Pathology
- severe necrotizing vasculitis of interlobar artery
- can have glomerulonephritis
Allergic Granulomatosis with Polyangiitis
Churg Sgrouss Syndrome
-Triad of asthma, eosinophilia, and vasculitis
Wageners + eosinophils
Burgers Disease Pathology
caused by smoking
Acute: segmental transmural actue inflammation without necrosis
+thrombosis +granulomas +giant cells
Chronic: nonspecific organization and recanalization of thrombos
+neovascularization +fibrosis
Hypersensitivity Angiitis Pathology
(leukocytoclastic vasulitis)
-Infiltration of smallest blood vessels (capillaries and post-capillary venules) by neutrophils with breakdown (leukocytoclasia) dispersing nuclear dust
+/- fibrinoid necrosis, thrombosis, rupture, hemorrhage
Later: lymphocytic infiltration
all lesions in same phase
