Vasculitis

Question 1

Vasculitis

Answer 1

Inflammation of blood vessel wall
Most cases are not infectious
Get nonspecific symptoms of inflammation (fever, fatigue, weight loss, myalgia)
Symptoms of organ ischemia due to inflammation causing narrowing or thrombosis

Question 2

Large Vessel Vasculitis
Medium
Small

Answer 2

L = involves aorta and its major branches
M = muscular arteries that supply organs
S = arterioles, capillaries, and venules

Question 3

Temporal (Giant Cell) Arteritis

Answer 3

Large vessel vasculitis that involves branches of carotid.
Most common form in OVER 50, usually females
Presents as headache (temporal Artery), visual disturbances (opthalmic A), and jaw claudication. Flu like symptoms with joint and muscle pain
ESR is elevated
Biopsy shows inflamed vessel wall with Giant cells and intimal fibrosis.
Treat with corticosteroids quickly to prevent blindness

Question 4

Takasayu Arteritis

Answer 4

Granulomatous vasculitis that involves aortic arch and its branch points.
adults YOUNGER than 50 (classically ASIAN females)
ESR is elevated
Visual and neurological symptoms with weakor absent pulses
Treat with corticosteroids

Question 5

Polyarteritis Nodosa

Answer 5

Necrotizing vasculitis involving multiple organs but NOT LUNGS
Presnts in young adults as hypertension (renal A), abdominal pain with melena (Mesenteric A), neuro disturbances and skin lessions
See HBsAg
Varying stages of disease are present. Fibroid necrosis heals with fibrosis = string of pearls
treat with corticosteroids and cyclophosphamide or else is fatal

Question 6

Kawasaki Disease

Answer 6

Asian children <4
Nonspecific signs of fever, conjunctivitis, ertheymatous rash of palms and soles, enlarged cervical lymph nodes
Coronary artery involvement is common and risk of thrombosis, MI, aneurysm with rupture
treat with Aspirin and IVIG

Question 7

Buerger Disease

Answer 7

Necrotizing Vasculitis nivolving digits in SMOKERS.
Presents with ulcerations, gangrene, autoamputation of fingers and toes.
Sometimes see Raynaud phenomenon

Question 8

Wegener Granulomatosis

Answer 8

Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
Classic = middle aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria to rapidly progressive glomulonephritis
c-ANCA correlates with disease
Large necrotizing granulomas with adjacent necrotizing vasculitis on biopsy
Treat with cyclophosphamide and steroids
relapses are common

Question 9

Microscopic Polyangiitis

Answer 9

Necrotizing vasculitis involving multiple organs especially LUNG and KIDNEY
Presents similar to Wegener nut nasopharyngeal involvement and granulomas are ABSENT
p-ANCA levels correlate with disease activity
Treat with cyclophosphamide and steroids
relapses are common

Question 10

Churg-Strauss Syndrome

Answer 10

Necrotizing granulomatous inflammation with EOSINIOPHILS involving multiple organs especially LUNGS and HEART
ASTHMA and peripheral EOSINOPHILS
p-ANCA correlates with disease activity

Question 11

Henoch-Schonlean Purpura

Answer 11

Vasculitis due to IgA complex deposition.
Most common vasculitis in children
Palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria
Usually occurs following an upper respiratory infection
Disease is self limited but may reoccur. Treat with steroids if necessary