Vascular Pathology 2 - Vasculitis, Vessel Hyperreactivity, Veins and Lymphatics, Vascular Tumors, Flashcards
The 2 classifications of vasculidities and the reason distinction is important for tx.
Infectious (pathogenic) and noninfectious (immunologic injury).
Noninfectious can be treated with immunosuppressive therapy.
What is immune complex vasculitis?
Deposition of antigen-antibody complexes in vascular walls»_space; inciting inflammatory reaction
What cases can immune complex vasculitis be seen in?
Systemic immunologic Diseases - SLE
Drug hypersensitivity
Secondary exposure of infectious agent
What are antineutrophil cytoplasmic antibodies?
ANCA (antibodies) reactant with cytoplasmic granules found in neuts, monocytes, and endothelial cells.
What was formerly called c-ANCA?
What was formerly called p-ANCA?
Anti-proteinase3 (Wegners Granulomatosis)
Anti-myeloperoxidase (Microscopic Polyangiitis)
What do ANCAs do?
Activate neuts, which release ROS.
Name the (2) large vessel vasculitides - both Granulomatous Disease
Giant Cell Arteritis
Takayasu Arteritis
Name the medium vessel vascilitides (3).
1) Immune complex mediated - PAN
2) Anti-endothelial cell bodies - Kawasaki disease.
3) Bechet Disease (small and medium vessels)
Name the small vessel vasculitides (4)
1) Microscopic polyangiitis
2) Churg-Strauss Syndrome
3) Thromboangiitis Obliterans (small and medium vessels)
4) Bechet Disease (small and medium vessels)
What am I?
An elderly female presents with HA ad unilateral temporal pain with firm nodules on palpation. She may complain of blindness. You see giant cell granuloma. Joints may hurt, but not caused by arthritis.
Giant cell arteritis
What is the most common vasculitis among older patients?
Giant cell arteritis
What large vessel arteritis is associated with fragmented elastic lamina and medial/intimal thickening of the temporal arteries?
Giant cell arteritis
What tx does giant cell arteritis respond well to?
Steroids
What large vessel arteritis is associated with fragmented elastic lamina and medial/intimal thickening of the aortic arch (esp. carotid and subclavian)?
Takayasu arteritis
What age group does Takayasu Arteritis affect?
People less than 50yo.
What arteritis may present with weak pulse and low BP in upper extremities?
Takayasu arteritis
What is the “pulseless disease”?
What is the main artery involved, what other 3 may also be involveD?
Main - Aortic arch
Other - Pulmonary, coronary, renal arteries
What arteritis am I?
1/3 of patients with this have chronic hep B, usually young adult.
PAN
What vessels are affected in PAN, what vessels are not affected?
Involved: Renal vessels > heart > liver > GI
Not involved: Pulmonary vessels
Tx for PAN
immunosuppressive therapy
What am I?
A 3yo with an MI, rash, and cervical LN involvement. What else is this called?
Kawasaki Disease
“Mucocutanous lymph node syndrome”
What artery does Kawasaki often involve?
Coronary arteries
What 2 things are given to children with Kawasaki to lower risk of coronary event?
IV-Ig and Aspirin
What symptoms (other than coronary involvement) are associated with Kawasaki
Skin.
Erythema of conjuctiva, oral mucosa, palms, and soles
Rash
Cervical LN enlargement
What vessels does microscopic polyangiitis most often affect?
Renal glom and lung capillaries
What ANCA is microscopic polyangiitis associated with?
MPO-ANCA
What is another name for microscopic polyangiitis?
leukocytoclastic vasculitis
Describe the necrosis in microscopic polyangiitis.
Segmental necrotizing inflammation with fibrinoid necrosis and many apoptotic neuts.
What 3 main things is Churg-Strauss Syndrome associated with?
Allergic rhinitis, asthma, and hypereosinophila