Vasculitis

Question 1

What are the three layers of the arterial wall?

Answer 1

Intima, media, adventitia

Question 2

What is the cause of vasculitis?

Answer 2

Etiology is normally unknown

Question 3

What are clinical features of vasculitis?

Answer 3

Nonspecific symptoms of inflammation (fever, fatigue, weight loss, myalgias) and organ ischemia due to luminal narrowing (due to fibrosis) and/or thrombosis.

Question 4

Name the primary divisions of the vasculitides and the general vessels involved

Answer 4

Large - aorta and its major branches, Medium - muscular arteries, Small - arterioles, capillaries, venules

Question 5

What is temporal (giant cell) arteritis? What type of vasculitis? What vessels are typically involved? Typical patient? Clinical presentation and associated artery? Describe biopsy. Tx and potential complications.

Answer 5

Large-vessel granulomatous vasculitis that classically involves branches of the carotid artery. Usually affects patients >50; generally women; Headache (temporal artery), visual disturbances (ophthalmic) jaw claudication, Flu-like Syx (Polymyalgia rheumatica), Increased ESR; Biopsy: inflamed vessel wall w/ giant cells and intimal fibrosis; Tx: Corticosteroids; High risk of blindness without treatment.

Question 6

What is an important consideration when taking a biopsy to Dx temporal arteritis?

Answer 6

You must take a long segment of vessel. The lesions are segmental so if you have a negative biopsy, it doesn’t rule out the disease.

Question 7

What is Takayasu Arteritis? Typical presentation? What is another name for TA? Why? Treatment?

Answer 7

Large vessel granulomatous vasculitis that involves aortic arch/branches. Presents in adults <50 (classically, young Asian women) as visual/neurologic Syx with weak/absent pulses in upper extremity (pulseless disease) ESR increased. Tx: Corticosteroids.

Question 8

What is Polyarteritis Nodosa? What organs are spared in this disease? What is the classic presentation? What is the textbook vascular presentation? Tx.

Answer 8

Medium vessel necrotizing vasculitis involving multiple organs. Lungs are spared. Young adult as HTN (renal artery involvement) abdominal pain with melena (mesenteric artery involvement), neuro disturbances, skin lesions. Associated with serum Hepatitis B surface Antigen. Fatal if not Tx. “String-of-pearls” from fibrosis. Tx: Corticosteroids and Cyclophosphamide.

Question 9

What is Kawasaki Disease? Classic presentation? Presenting symptoms? Which artery is commonly involved and what are potential complications? Tx?

Answer 9

Self-limiting medium vessel vasculitis that typically affects Asian children < 4 yo. Syx: fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph nodes. Coronary arteries involved which may lead to thrombosis (and MI) or aneurysm and rupture. Tx: aspirin and IVIG

Question 10

What is the mnemonic for Kawasaki Disease?

Answer 10

Asian kids on Kawasaki motorcycles. They drive the motorcycles with their hands and feet and when they drive it their heart is affected.

Question 11

What is Buerger Disease? Presenting Syx? What activity is it highly associated with? Tx?

Answer 11

Medium vessel necrotizing vasculitis involving digits. Syx: Ulceration, gangrene, autoamputation of fingers/toes, Raynaud’s Syndrome, Associated with heavy smoking; Tx: Smoking cessation

Question 12

What is Wegener’s Granulomatosis? What organs are involved? Classic presentation? Marker used to monitor disease? Biopsy findings? Tx?

Answer 12

Small vessel necrotizing vasculitis involving nasopharynx, lungs, kidneys; Presentation: Middle-aged male, w/ sinusitis/nasopharyngeal ulceration, hemoptysis, bilateral nodular lung infiltrates, hematuria due to glomerulonephritis; c-ANCA; Biopsy: Necrotizing granulomas with adjacent vasculitis Tx: cylcophosphamide, steroids

Question 13

Mnemonic for Wegener Granulomatosis

Answer 13

“C Disease” “Wecener’s” Body C - nasopharynx, lungs, kidney; c-ANCA, cyclophosphamide

Question 14

What is Microscopic Polyangiitis? What is the typical presentation? Marker of disease? Tx?

Answer 14

Small vessel necrotizing vasculitis involving multiple organs esp. lung/kidney; Same presentation as Wegener minus the nasopharynx and granulomas; p-ANCA; Tx:corticosteroids, cyclophosphamide

Question 15

What is Churg-Straus Syndrome? Associated marker? What other disease is on your differential? How do you differentiate?

Answer 15

Small vessel necrotizing granulomatous inflammation with eosinophilia affecting multiple organs esp heart/lung. Asthma commonly associated; p-ANCA; Microscopic Polyangiitis - differentiating information: granulomas, asthma, and eosinophilia in CS

Question 16

What is Henoch-Schoenlein Purpura? Of what population is it the most common vasculitis? What event does it commonly follow? Presenting Syx? Tx?

Answer 16

Generally self-limiting small vessel vasculitis due to IgA immune complexes; Children!; Palpable purpura on buttocks/legs, GI pain/bleeding, hematuria (due to IgA neuropathy); Follows upper resp. infection (A lot of IgA produced to protect mucosa). If severe Tx with steroids.

Question 17

Which large artery vasculitidies are granulomatous?

Answer 17

Takayasu and Giant Cell Arteritis

Question 18

What is the politically correct name for Wegener’s Granulomatosis?

Answer 18

Granulomatosis with Polyangitis

Question 19

What does c-ANCA stand for? What disease is it used to monitor?

Answer 19

cytoplasmic - Antineutrophil Cytoplasmic Antibodies, Wegener’s Granulomatosis (Granulomatosis with Polyangiitis)

Question 20

What does p-ANCA stand for? What diseases is it used to monitor?

Answer 20

Perinuclear - Antineutrophil Cytoplasmic Ab; Microscopic polyangiitis, Churg-Strauss Syndrome

Question 21

Mnemonic to remember microscopic polyangiitis?

Answer 21

Same as wegeners minus the nasopharynx involvement and the granulomas

Question 22

What are the 3 Syx of Behcet’s disease?

Answer 22

Oral aphthous ulcers, genital ulcers, uveitis

Question 23

What vessels are frequently affected in Behcet’s disease?

Answer 23

Large and small vessel vasculitis.

Question 24

A if a person with an autoimmune disease (Sjoegren’s or SLE) develops a purpuric skin rash, what they probably got?

Answer 24

Hypersensitivity vasculitis due to depositions of immune complexes

Question 25

What is another name for Kawasaki disease?

Answer 25

Mucocutaneous lymph node syndrome

Question 26

What two diseases are Raynaud Syndrome Associated with?

Answer 26

SLE and Scleroderma

Question 27

What do purpura and glomerulonephritis in a child suggest?

Answer 27

Henoch-Schoenlein purpura

Question 28

What type of vasculopathy is observed in Scleroderma?

Answer 28

Subintimal fibromuscular thickening