Vasculitis

Question 1

Temporal (giant cell) arteritis

Answer 1

Generally elderly females.
Unilateral headache (temporal artery), jaw claudication
May lead to irreversible blindness use to ophthalmic artery occlusion
Associated with poly myalgia rheumatic a

Most commonly affects branches of carotid artery
Focal granulomatous inflammation
Increased ESR
Treat with high-dose corticosteroids

Question 2

Takayasu’s arteritis

Answer 2

Asian females < 40 years of age
Pulse less disease (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances

Granulomatous thickening of aortic arch, proximal great vessels
Increased ESR
Treats with corticosteroids

Question 3

Polyarteritis nodosa

Answer 3

Young adults
Hep B seropositivity in 30% of patients
Fever, weight loss, malaise, headache
GI: abdominal pain, melena
Hypertension, neurological dysfunction, cutaneous eruptions, renal damage

Typically involves renal and visceral vessels
Immune-complex mediated
Transmural inflammation of arterial wall with fibrinoid necrosis
Lesions of different ages
Many aneurysms and constrictions on arteriograpm
Treat with corticosteroids, cyclophosphamide

Question 4

Kawasaki disease

Answer 4

Asian children < 4 years of age
Fever, cervical lymphadenopathy, conjunctival injection, changes in lips or oral mucosa (strawberry tongue), hand-foot erythema, desquamating rash

May develop coronary aneurysms: MI, rupture
Treat with IV immunoglobulin and aspirin

Question 5

Beurger’s disease (thromboangiitis obliterans)

Answer 5

Heavy smokers < 40 years of age
Intermittent claudication may lead to gangrene, auto amputation of digits, superficial nodular phlebitis
Raynaud’s often present

Segmental thrombosing vasculitis
Treat with smoking cessation

Question 6

Microscopic polyangitis

Answer 6

Necrotizing vasculitis commonly involving lung, kidneys, skin with pauci-immune glomerulonephritis and palpable purpura

No granulomas
p-ANCA
Treat with cyclophasphamide and corticosteroids

Question 7

Wegener’s (granulomatosis with polyangitis)

Answer 7

Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis
Lower respiratory tract: hemoptysis, cough, dyspnea
Renal: he matures, red cell casts

Triad:
- focal necrotizing vasculitis
- necrotizing granulomas in lung and upper airway
- necrotizing glomerulonephritis
c-ANCA
Chest x-Ray: large nodular densities
Treat with cyclophasphamide and corticosteroids

Question 8

Churg-Strauss syndrome

Answer 8

Asthma, sinusitis, palpable purpura, peripheral neuropathy (wrist/foot drop). Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis)

Granulomatous, necrotizing vasculitis with eosinophilia
p-ANCA
Elevated IgE level

Question 9

Henoch-Schonlein purpura

Answer 9

Most common childhood systemic vasculitis
Often follows URI
Classic triad:
- skin: palpable purpura on buttocks/legs
- arthralgia
- GI: abdominal pain, melena, multiple lesions of same age

Vasculitis secondary to IgA complex deposition
Associated with IgA nephropathy