Vasculotides

Question 1

Polyarteritis Nodosa

Answer 1

Affected arteries are medium-small and show a sharply segmental, necrotizing inflammation

Path: Destruction of arterial median and internal elastic elamina leads to replacement w/ scar tissue and nodular thickenings
*(String of pearls appearance on imaging)

-Affects the kidneys, GI, liver, and heart; sx assoc. w/ these areas (renal vasculitis, melena) impaired perfusion can lead to ulcerations, infarcts, and atrophy of affected areas

Diagnosis: Vessel biopsy
Tx: Corticosteroids and cyclophosphamide
*Can be fatal w/o tx

Question 2

Kawasaki Syndrome

Answer 2

-Affects large-small vessels (*Most often the coronary) and is the #1 causes of acquired heart disease in CHILDREN; resembles polyarteritis nodosa but has transmural inflammation and less fibrinoid necrosis and can also lead to an aneurysm

Congestied Conjunctiva 
Rash 
Associated lymphadenopathy
(S) Changes in lipS 
Hands (edema) 
and
BURN (erythema) 

Tx: Aspirin/IVIG (only time you give aspirin to kids)

Question 3

Microscopic Polyangiitis

Answer 3

Similar to PAN but affects small vessels and all lesions tend to be the same age; typically due to hyperimmune response w/ possible complex deposition (only sometimes) producing segmental fibrinoid, non-granulomatous inflammation

***MPO-ANCA present

Signs and sx depend on vascular bed involved

Tx: Cyclophosphamide and corticosteroids

Question 4

Giant Cell Arteritis

Answer 4

Granulomatous, segmental inflammation of the media affecting large to small vessels (most commonly assoc. w/ temporal and opthalamic arteries); will see intimal destruction on histology and thickened media

-Affected artery is nodular and tender to palpation; diagnose w/ biopsy of artery

***Medical emergency requiring tx in order to prevent blindness

Tx: Corticosteroids

Question 5

Churg-Strauss Syndrome

Answer 5

Small vessel vasculitis assoc. w/ TRIAD

1. Allergic rhinitis
2. Bronchial asthma
3. Eosinophilia

Lesions have intra- and extra-vascular granulomas and pts. present w/ palpable purpura, GI bleeding, and renal involvement
-May have assoc. MPO-ANCA

Question 6

Granulomatosis w/ Polyangiitis

Answer 6

CLASSIC TRIAD

1. Respiratory tract involvement (necrotizing granulomas of URT and LRT that presents w/ ulcerations)
2. Focal necrotizing granulomas of medium-small vessels commonly in the lungs
3. Renal disease (focal necrotizing glomerulonephritis presenting w/ hematuria, proteinuria)
   
   • Can be limited to just respiratory tract

***Assoc. w/ PR3-ANCA (good marker that will increase in relapse)

Question 7

Thromboangiitis obliterans

Answer 7

Segmental, necrotizing inflammation of medium-small arteries that commonly affects radial and tibial arteries; lumen shows thrombosis w/ microabscesses filled w/ PMNs and surrounded by granulomatous inflammation

***ASSOC W/ MALE, HEAVY SMOKERS (commonly assoc. w/ tobacco hypersensitivity)

Early signs: Instep claudication, cold insensitivity in hands, and superficial nodular phlebitis

Later: Ulceration of extremities, gangrene

*Smoking cessation early is highly beneficial