Vasculitis Lecture

Process characterized by inflammation and necrosis of blood vessels. (most from unknown cause)

immune complexes damage vessel wall and WBCs. Complement responsible for damage. Affects small and medium muscular arteries, rarely large vessels. Older people b/c they take more drugs. Can cause necrosis. Tx: take them off the drug causing it, also maybe prednisone.

Must have 3 of 5 criteria: age > 16, medication taken at onset, palpable purpura, maculopapular rash, Biopsy of arteriole and venule show granulocytes in peri- or extra vascular area.

immune complex mediated damage. Affects small vessels (arterioles and venules). Usually occurs in kids/young from an infectious trigger. Usually just Palpable purpura on skin, bloody diarrhea, not too bad.

Must have 2 of 4 criteria : palpable purpura, Age < 20, bowel angina ( pain w/ bloody diarrhea), granulocytes in wall of arterioles or venules

Immunoglobulins that under go reversible precipitation in cold. Sx: acral cyanosis, ulceration( ears, palate), necrosis, Raynauds. small vessels (arterioles and venules)

Type 1- assc. with hemo malignancies (myeloma, lymphoma)
2 - assc with Hep C
3 - assc with other infxns, rheumatic diseases, inflammatory disorders.

Usually affects upper and lower resp. tract and kidneys. ELK triad=
E- ENT
L- Lung ( cavitary masses)
K- Kidney ( biopsy shows glomerulonephritis)

ANCA (+) mostly C( proteanase 3). Chronic inflammation ---> saddle nose deformity. Eye inflammation. Often infection triggered.


Must have 2 of 4 : nasal or oral inflammation, abnormal CxR (nodules, cavities, infiltrate), urinary sediment, granulomatous inflammation in wall, peri- or extravascular. Affects small and medium muscular arteries.

Alot of end organ problems not enough blood to testicles, kidneys, bowel. Livedo and subQ nodules present. Neuropathy (wrist drop). Aneurysm ! ( emphasized by Yunus). No Lung. ANCA (-)

Must have 3 of 10: weight loss >4kg, livedo reticularis, testicular pain, myalgias, mono/polyneuropathy, diastolic BP >90, increased BUN, HBV, arteriographic abnormality, PMNs present. Affects small and medium muscular arteries.

Mix between Wegeners and PAN in a person with asthma. Allergy type symptoms. U/L resp problems. nerve problems.

Must have 4 of 6 : asthma, eosinophilia > 10%, neuropathy, pulmonary infiltrates, paranasal sinus abnormality, extravascular eosinophils. Affects small and medium muscular arteries.

Childhood disease. Mucus skin and lymph node inflammation. Get strawberry tongue, skin peeling, big problem is w/ coronary arteries. Vessels weaken and burst case of kids having heart attacks.


Mus have 4 of 5: Fever @ least 5 days, bilateral conjunctival congestion, oropharyngeal involvement ( strawberry tongue), cervical lymphadenopathy, 1 or more of ( indurative edema of hands and feet, erythema of palms, desquamation of skin, transverse grooves in nails 2-3 months after.) Affects small and medium muscular arteries.

Usually in younger patients. Get pain/ cant move limbs. Diff BP in arms ( pulseless disease). Large vessels narrow in some places and balloon (aneurysm) in others.

3 of 6 : age < 40, claudication, decreased brachial pulse, BP >10mmHg diff between arms, bruit over subclavian, arteriogram abnormal (occlusion or narrow aorta) Large arteries

Usually much older >50 . Women twice as likely to get. Most common large vessel. General inflammation (hips shoulders), stiff Sx: Headache, jaw claudication, visual loss, weight loss, respiratory sx, elevated ESR, CRP, anemia, elevated Alkaline phosphatase, scalp necrosis. Often seen with PMR. Can get dilation of large vessels as well.

3 of 5 : age > 50, new headache, temporal artery tender or dcr. pulse, elevated westergren ESR >50mm/hr, artery inflitrate w/ infiltrate, granulomatous inflamm. , giant cells. Large arteries

Recurrent oral ulceration plus recurrent genital ulceration, eye lesions (inflammation), can cause venulitis.

skin lesions

Tx: not much bc usually just skin involved. Maybe some predinisone.

-Ischemic vascular changes ( gangrene, Raynauds, livedo)
-Glomerulonephritis
-mononeuritis multiplex
-abdominal or testicular pain

Worst of the 3 because they are much more sick/ losing organ function.

Tx: must use harsh drugs ** treat with cyclophosphamide (emphasized this in class)

Headache
Jaw/limb claudication
visual loss stroke

not so bad b/c even if inflamed they are large enough where blood is still getting through.

Tx:corticosteroids-> Prednisone

F. they can but almost none of them do.