Vasculitis

Question 1

What are the three layers of the arterial wall?

Answer 1

Endothelial intima, Smooth muscle media and connective tissue adventitia

Question 2

What are the clinical features of vasculitis?

Answer 2

1. Nonspecific symptoms of inflammation (fever, fatigue, weight loss and myalgias)
2. Symptoms of organ ischemia - due to luminal narrowing or thrombosis of the inflamed vessels

Question 3

Which vessels are involved in large vessel vasculitis?

Answer 3

Aorta and major branches

Question 4

Which vessels are involved in medium vessel vasculitis?

Answer 4

Muscular arteries that supply organs

Question 5

Which vessels are involved in small vessel vasculitis?

Answer 5

Arterioles, capillaries, venules

Question 6

A patient presents with headache to your clinic. On examination, she has visual deficits and has problem chewing food. She also appears to have flu like symptoms. What does she have? What is causing her symptoms? What lab values are elevated?

Answer 6

She has Temporal (Giant Cell) Arteritis which is a granulomatous vasculitis that classically involves branches of the carotid artery. Headache is due to temporal artery involvement, visual disturbances are due to opthalmic artery involvement. ESR is elevated.

Question 7

What disease commonly presents with Temporal (Giant Cell) Arteritis?

Answer 7

Polymyalgia Rheumatica which is joint and muscle pain.

Question 8

What is seen on biopsy of Temporal (Giant Cell) Arteritis? How is it treated?

Answer 8

Inflamed vessel wall with giant cells and intimal fibrosis. Lesions are segmental and negative biopsy does not exclude disease. Corticosteroids. High risk of blindness without treatment.

Question 9

What is seen on biopsy of Temporal (Giant Cell) Arteritis? How is it treated?

Answer 9

Inflamed vessel wall with giant cells and intimal fibrosis. Lesions are segmental and negative biopsy does not exclude disease. Corticosteroids. High risk of blindness without treatment.

Question 10

A 30 year old Japanese teacher comes with vision problems. Neuro exam shows positive findings. When palpating, you have a hard time finding a pulse on her upper extremities. What does she have? Which vessels are involved? What lab value is elevated and how would you treat?

Answer 10

Takayasu arteritis which is a granulomatous vasculitis that classically involves the aortic arch at branch points. Presnts in adults (

Question 11

A 24 year old basket ball player comes in with abdominal pain. He mentions that he has been having really black stools. Exam is notable for hypertension, neurologic disturbances. He also has multiple skin lesions. What does he have? What causes his symptoms? Which organs are not involved? What lab value is elevated and how would you treat?

Answer 11

Polyarteritis Nodosa which is a necrotizing vasculitis involving multiple organs. Lungs are spared. HTN due to renal artery involvement, abdominal pain with melena due to mesenteric artery involvement. Associated with serum HBsAg. Treatment is corticosteroids and cyclophosphmide. Fatal if not treated.

Question 12

How does polyarteritis nodosa present on imaging? What is causing this phenomena?

Answer 12

String of pearls appearance on imaging. Lesions of varying stages are present with early lesions that consist of transmural inflammation with fibrinoid necrosis that eventually heal with fibrosis to form the string portion.

Question 13

How does polyarteritis nodosa present on imaging? What is causing this phenomena?

Answer 13

String of pearls appearance on imaging. Lesions of varying stages are present with early lesions that consist of transmural inflammation with fibrinoid necrosis that eventually heal with fibrosis to form the string portion.

Question 14

A 3 year old chinese boy is brought to the clinic with elevated fever. His conjuctiva appears inflammed, and PE shows enlarged cervical lymph nodes. He also has an erythematous rash on his palms and soles. What does he have? Which vessles are involved? What does this increase the risk of? How is it treated? Is the disease self limited?

Answer 14

He has Kawasaki Disease which classically affects asian children

Question 15

A patient presents with ulceration, gangrene and autoamputation of fingers and toes. He is also a heavy smoker. He has Raynaud phenomena on examination. What does he have and how is he treated?

Answer 15

Buerger disease which is a necrotizing vasculitis involving digits. Smoking cessation.

Question 16

A patient presents with ulceration, gangrene and autoamputation of fingers and toes. He is also a heavy smoker. He has Raynaud phenomena on examination. What does he have and how is he treated?

Answer 16

Buerger disease which is a necrotizing vasculitis involving digits. Smoking cessation.

Question 17

A 50 year old male presents with sinusitis. He complains he has been throwing up blood, and has noted a tinge of blood in his urine. What is causing this presentation and what organs are involved? What values are elevated? What is seen on biopsy and how is he treated?

Answer 17

He has Wegner Granulomatosis that involves nasopharynx, lungs and kidneys. Hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis. Serum c-ANCA levels correlate with disease activity. Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis. Treatment is cyclophosphamide and steroids.

Question 18

A 50 year old male presents with hemoptysis. He has noted a tinge of blood in his urine. What is causing this presentation and what organs are involved? What values are elevated? What is seen on biopsy and how is he treated?

Answer 18

He has Microscopi Polyangitis that involves lungs and kidneys. Hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis. Serum P-ANCA levels correlate with disease activity. Biopsy reveals no granulomas. Treatment is cyclophosphamide and steroids.

Question 19

What is Churg-Strauss syndrome? What two conditions are often present? What values are elevated?

Answer 19

Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart. Asthma and peripheral eosinphilia are often present. Serum p-ANCA.

Question 20

p-ANCA, granulomas, lung, kidneys is which disease?

Answer 20

Churg-Strauss Syndrome

Question 21

p-ANCA, no granulomas, lung, heart is which disease?

Answer 21

Microscopic Polyangitis

Question 22

p-ANCA, no granulomas, lung, heart is which disease?

Answer 22

Microscopic Polyangitis

Question 23

A patient presents with GI pain. Physical exam shows palpable purpura on buttocks and legs. He also has GI bleeding and hematuria. What is causing this? What might have triggered it? How is it managed?

Answer 23

Henoch-Schonlein Purpura which is a vasculitis due to IgA immune complex deposition. Most common vasculitis in children. Hematuria due to IgA nephropathy. Usually occurs following upper respiratory tract infection. Disease is self limited but treat with steroids if severe.