Vasculitis & Angitis Lecture Powerpoint

Question 1

2 key large vessel vasculitis

Answer 1

Takayasu arteritis

Giant cell arteritis

Question 2

2 key medium vessel vasculitis

Answer 2

Polyarteritis nodosa

Kawasaki disease

Question 3

Key ANCA positive small vessel vasculitis (3)

Answer 3

• microscopic polyangitis
• granulomatosis with polyangitis (wegeners)
• eosinophilic granulomatosis with polyangitis

Question 4

Key Immune complex small vessel vasculitis (2)

Answer 4

• Anti glomerular basement membrane dz

- IgA vasculitis

Question 5

Vasculitis etiology and pathogenesis

Answer 5

Antigenic stimulation results in immune complex formation, autoantibody production and or cell mediated immunity. Formation of pathogenic immune complexes, antiendothelial antibody mediated vascular injury, cell mediated immune resposne with granuloma formation

Question 6

Giant cell arteritis definition

Answer 6

Granulomatous vasculitis of medium to large arteries most often temporal artery or opthalmic arteries, can also involve arteries that originate from aortic arch

Question 7

Giant cell arteritis clinical presentation (9)

Answer 7

• age >50
• headache
• systemic symptoms
• polymyalgia rheumatica (muscle aches in proximal musculature of upper and lower extremities)
• visual symptoms
• jaw or limb claudication
• markedly elevated sed rate
• tender or swollen artery
• temporal cell biopsy shows thickening with multinucleated giant cells and skip lesions

Question 8

Giant cell arteritis treatment options (3)

Answer 8

• high dose prednisone followed by slow taper
• tocilizumab
• methotrexate

Question 9

Polymyalgia rheumatica (PMR) definition and treatment (1)

Answer 9

Proximal upper and lower limb myalgias present for at least 1 month in patients over 50, sed rate elevated (over 50) upon exclusion of other conditions and see excellent response to moderate dosage of prednisone

Question 10

Giant cell arteritis and polymyalgia rheumatica etiology

Answer 10

Unknown

Question 11

Polymyalgia rheumatica (PMR) clinical presentation (4)

Answer 11

• good health prior to onset
• low grade fever and weight loss
• begins in one area and then involves symmetrical and proximal muscles of upper and lower extremities
• morning stiffness common

Question 12

Polyarteritis nodosa

Answer 12

Medium size blood vessel vascularity, necrotizing panarteritis (involving all 3 layers of blood vessel) often presenting with skin, joints, peripheral nerves, gut, and kidney involvement, see panmural necrotizing lesions with predilection for vessel bifurcation but distinct from ANCA associated vasculitis (microscopic polyangitis)

Question 13

Polyarteritis nodosa clinical presentation (8)

Answer 13

• peak age 40-60
• fever, milaise
• skin purpura or ulcerations
• renal disease (proteinuria and hypertension)
• neuropathy (foot or wrist drop***)
• GI bleeding due to ischemia
• elevated ESR, anemia, thrombocytosis
• beaded appearance on angiogram esp. at bifurcation

Question 14

Polyarteritis nodosa (2) treatment

Answer 14

• high dose prednisone and taper

- cyclophosphamide when severe

Question 15

Eosinophilic granulamatosis with polyangitis (EGPA) definition

Answer 15

ANCA associated vasculitis often begins with prodromal period of months to years of rhinitis, polyposis, asthma, 2nd stage of eosinophilia, eosiniphilic pneumonia or gastroenteritis, followed by 3rd phase which is frank systemic vasculitis

Question 16

Eosinophilic granulomatosis with polyangitis (EPGA) treatment options (1)

Answer 16

-prednisone 40-60 mg daily

Question 17

Granulomatosis with polyangitis (wegener’s granulomatosis) definition and treatment options (3)

Answer 17

Positive c-ANCA necrotizing granulomas of respiratory tract upper and lower, glomerulonehprhitis, and joints/other organs, limited movement initlaly and more generalized dz eventually, requires biopsy for diagnosis, treated with steroids and cyclophosphamide or rituximab

Question 18

Microscopic polyangiitis and treatment (3)

Answer 18

Small vessel pauci immune necrotizing vasculitis onsetting age of 40-60 treated with high dose prednisone or cyclophosphamide and rituximab

Question 19

IgA vasculitis (Henoch schonlein purpura) definition

Answer 19

Multisystem disorder characterized by palpable purpura (common on lower extremities and buttocks), abdominal pain, and hematuria, peak incidence ages 3-15 but can affect any age, self limited course but an relapse, just need supportive care via glucocorticoids

Question 20

Leukocytoclastic vasculitis (hypersensitivty vasculitis)

Answer 20

Biopsy finding of small blood vessels usually in the skin typically having skin develop characteristic palpable purpura at the same stage, often idiopathic but can be due to serum sickness or if IgA then considered henoch schonlein purpura

Question 21

Think of ___ when 2 or more seemingly unrelated organ systems are involved in pathology

Answer 21

vasculitis