Chapter 7 Vasculitis

Question 1

What are two Large vessel vasculitides?

Answer 1

Temporal (Giant Cell) Arteritis

Takayasu Arteritis

Question 2

What type of vasculitis is Temporal Arteritis? What vessel does it usually affect?

Answer 2

Granulomatous vasculitis that classically involves branches of the carotid artery.

Question 3

What demographic does Temporal Arteritis affect?

Answer 3

Older adults (>50) usually women

Question 4

What are the presenting symptoms of temporal arteritis?

Answer 4

Headache (temporal artery involvment), visual disturbances (opthalmic artery involvment), jaw claudication. FLu like symptoms with joint and muscle pain (polymyalgia rheumatica).

Question 5

What is an irregular lab value seen in temporal arteritis?

Answer 5

ESR is elevated

Question 6

What does a biopsy reveal in temporal arteritis? What is important to keep in mind when taking a biopsy segment?

Answer 6

Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis. Must take long segment because lesions are segmental.

Question 7

What is the treatment of temporal arteritis. What major complication indicates early treatment even without confirmative diagnosis?

Answer 7

Treatment is corticosteroids. there is a high risk of blindness without treatment.

Question 8

What type of vasculitide is takayasu Arteritis?

Answer 8

Large vessel granulomatous vasculitis very similar to temporal vasculitis.

Question 9

What vessels does takayasu arteritis affect?

Answer 9

involves aortic arch at branch points.

Question 10

What demographic does takayasu arteritis affect?

Answer 10

adults (<50) classically young asian females

Question 11

What are the presenting symptoms of Takayasu arteritis?

Answer 11

visual and neurological symptoms with a weak or absent pulse in the upper extremity (pulseless disease)

Question 12

What lab value is elevated in takayasu arteritis?

Answer 12

ESR

Question 13

What is the treatment of takayasu arteritis?

Answer 13

Corticosteroids

Question 14

Name 3 medium vessel vasculitides?

Answer 14

Polyarteritis nodosa
Kawasaki disease
Buerger Disease

Question 15

What type of vasculitis is PAN? What type of arteries does it affect? which organs?

Answer 15

Medium vessel (muscular arteries supplying organs) necrotizing vasculitis. All organs except lungs.

Question 16

How does PAN present?

Answer 16

In young adults as hypertension (renal artery involvment), abdominal pain with melena (mesenteric artery), neurological disturbances, and skin lesions.

Question 17

What virus is PAN associated with?

Answer 17

HBV

Question 18

What demographic does kawasaki disease affect?

Answer 18

Asian children <4

Question 19

How does kawasaki disease present?

Answer 19

Nonspecific signs including fever, conjuctivitis, erythematous rash of palms and soles, and enlarge cervical lymph nodes.

Question 20

What is a major feared complication of kawasakis disease (which artery)

Answer 20

Coronary artery involvment is common and leads to risk for thrombosis with MI and aneurysm with rupture

Question 21

How is kawasaki disease treated?

Answer 21

Aspirin and IVIG, disease is self limiting

Question 22

What doe early lesions of PAN show?

Answer 22

transmural inflammation with fibrinoid necrosis

Question 23

What type of vasculitis is buerger disease? what body parts does it affect?

Answer 23

medium vessel vasculitis involving digits.

Question 24

How does beurger disease present?

Answer 24

ulceration, gangrene, and autoamputation of fingers and toes, raynaud phenomenon is often present.

Question 25

What risk factor is high associated with beurger disease? what does treatment involve?

Answer 25

Smoking, treatment is to stop smoking

Question 26

What type of vasculitis is Wegners Granulomatosis (granulomatosis with polyarteriitis)? what areas of the body are commonly invovled?

Answer 26

necrotizing small vessel vasculitis involving the nasopharynx, lungs, and kidneys.

Question 27

What is the classic presentation of Wegners granulomatosis?

Answer 27

middle aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral lung infiltrates, hematuria due to rapidly progressive glomerulonephritis. (presents with hematuria)

Question 28

What serum marker correlates with diease activity in Wegners granulomatosis?

Answer 28

c-ANCA

Question 29

What does biopsy reveal in Wegners granulomatosis?

Answer 29

Large necrotizing granulomas with adjacent necrotizing vasculitis

Question 30

What is the treament for Wegners granulomatosis? Does it work well?

Answer 30

cyclophosphamide and steroids, relapses are common

Question 31

What type of vasculitis is is microscopic polyangiitis? What organs does it involve?

Answer 31

Small vessel necrotizing vasculitis involving multiple organs, mainly lungs and kidney (similar to Wegners but without nasopahryngeal involvment)

Question 32

What serum marker correlates with disease activity in microscopic polyangiitis?

Answer 32

p-ANCA

Question 33

What type of Vasculitis is Churg-Strauss Syndrome and what organs does it primarily affect?

Answer 33

small vessel vasculitis with necrotizing granulomatous inflammation with eosinophils involving multiple organs especially the lungs and heart

Question 34

What findings are often present in Churg Strauss syndrome (clinical and laboratory)

Answer 34

Peripheral eosinophilia and asthma

Question 35

What serum marker correlates with disease activity in Churg strauss syndrome?

Answer 35

p-ANCA

Question 36

What type of vasculitis is Henoch schonlein purpura? what is the cause of it? what demographic is it most common in

Answer 36

Small vessel vasculitis due to IgA immune complex deposition mainly seen in children.

Question 37

How does Henoch Schonlein pupura present? what usually preceeds it?

Answer 37

palpable purpura on the buttocks and legs, GI pain and bleeding, and hematuria (IgA nephopathy) usually following an upper respiratory tract infection.

Question 38

How is Henoch schonlein purpura treated?

Answer 38

Disease is self-limited, but may recur; treat with steroids. if sever