Vasculitis Nicholls Flashcards
What are some signs and symptoms of vasculitis in general?
Fever, myalgia, arthralgia, malaise
- Also palpable Purpura (these are just erythematous tender skin nodules), focal skin necrosis and ulceration
- Livedo reticularis (this is a purplish discoloration of the skin due to dilation of the blood vessels,
- Urticaria
What is the key to diagnosis of vasculitis?
Distribution of organ involvement and histopathologic features
What are the common causes of infectious vasculitis?
Fungal (aspergillus)
Bacterial (Pseudomonas)
Viral (cytomegalovirus)
In temporal giant cell arteritis, what are the Giant cells doing?
THey attack the internal elastic Lamina and destroy it.
- You get intimal thickening that can lead to stenosis
Clinical manifestations of TGCA
- headache in 75% of pts
- swollen tender artery
- scalp tenderness
- visual disturbances
- jaw claudication
Peripheral neuropathy most common with
polyarteritis nodosa
Nodular pulmonary infiltrates most associated with
Granulomatosis with polyangiitis
Infectious vasculitis may cause
mycotic aneurysm
Inflammation in TGCA is
Segmental, Transmural, granulomatous
TGCA affects arteris of what size?
Medium
Why is it important that TGCA is segmental?
Biopsies may be false negatives
Why is is important that TGCA is transmural?
More easily narrows the lumen…..whereas atherosclerosis takes 50 years to narrow your coronary, TGCA takes just a few years.
Describe the inflammation of Takayasu Arteritis
Segmental, Transmural, loosely grnulomatous, and …..Necrotizing
Takayasu Arteritis can involve the aorta and cause
dissection–> rupture –> hemopericardium– > DEAD
Basically Cardiac Tamponade (bunch of blood in the pericardium)
Polyarteritis Nodosa inflammation is:
Segmental, transmural, NODULAR
What is the most characteristic feature of polyarteritis nodosa
Fibrinoid necrosis
Acute phase of PN
- neutrophils, intimal thickening, cell proliferation and degeneration, luminal stenosis
- Also may see: thrombosis, occlusion, rupture…all of which occur at BRANCHPOINTS of the artery
Later phases of PN
Chronic inflammation, fibroblasts, scarring
Unlike temporal arteritis, aneurysms are …. in PN
fairly common
Lesions are at………..phases at the same time in PN
DIFFERENT
Livedo reticularis can be a feature of
PN….basically just looks like a net like rash…its dilated blood vessels so that makes sense
Kawasaki disease features
Endothelial necrosis + transmural inflammation with neutrophils, lymphocytes, wall necrosis,and aneurysms
Aneurysms are most characteristic of which vasculitis?
Kaawasaki
What tests do we use for Kawasaki disease?
There is no blood test. It is a clinical diagnosis based on history and physical
Kawasaki’s disease is most common at what age?
1
Describe Granulomatosis with polyangiitis
Necrotizing granulomatous vasculitis of both ARTERIES AND VEINS in the UPPER AND LOWER RESP TRACT ANS KIDNEYS
Histology of granulomatosis with polyangiitis looks like
Geographic areas of necrosis with basophillic debris, BLUED
ANCA-PR3
Granulomatosis with Poly
Signs and symptoms of granulomatosis with Poly
Mucosal ulcers, necrotizing lesions, Nasal cartillage destruction
Most common site of involvement on presentation with GP
URT
GP in the kidney?
Can have severe necrotizing vasculitis of the interlobar arteries
May also have glomerulonephritis
What is Churg Strauss?
Allergic granulomatosis with polyangitis
Churg Straus Triad
Asthma , eosinophilia, vasculitis
Pathology of Churg Strauss?
Wegeners + Eosinophils
Brueger dz
Acute buergers: segmental transmural acute inflammation without necrosis + thromboses
+ granulomas + giant cells
Chronic: organization of the thromous and fibrosis
Beurgers is related to?
Smoking!! They must stop to prevent progression
Hypersensitivity angiitis
Affects VERY SMALL vessels
- Neutrophils infiltrate and break down dispersing nuclear dust
In Hypersensitivity angiitis, pts have leasions that are…..
ALL in the same phase
Most common cause of Hypersensitivity angiitis in kids?
URT…skin involvement, joint involvement, GI involvement, Renal involvement
In adults the most common cause of hypersensitivity vasculitis is?
DRUGS…Sulfas, penicillin, cephalosporins, diuretics, phenytoin, allopurinol
