Pathoma - Vasculitis

Question 1

What are the large-vessel vasculitides?

Answer 1

Temporal (Giant cell) arteritis

Takayasu arteritis

Question 2

What are the medium-vessel vasculitides?

Answer 2

Think: cook a COW in a PAN to get a MEDIUM BURGER

Kawasaki Disease

Polyarteritis Nodosa

Buerger Disease

Question 3

What are the small-vessel vasculitides?

Answer 3

Wegener Granulomatosis

Microscopic polyangiitis

Churg-Strauss Syndrome

Henoch-Schonlein Purpura

Question 4

What vessels are affected in Temporal (Giant cell) Arteritis?

Answer 4

Branches of the carotid artery

Question 5

Presentation of Temporal (Giant cell) Arteritis?

Answer 5

Depends on artery involved:

Headache –> temporal a.

Visual disturbances –> ophthalmic a.

Jaw claudication

Question 6

What is polymyalgia rheumatica and what disease is it involved in?

Answer 6

Flu-like symptoms with joint and muscle pain

Presentation of Temporal (Giant cell) Arteritis

Question 7

Describe biopsy in Temporal (Giant cell) Arteritis

Answer 7

Focal granulomatous inflammation:

Giant cells and intimal fibrosis (between intima and media)

Lesions are segmental so biopsy requires long segment and may be negative even with disease

Question 8

Treatment and importance of treatment in Temporal (Giant cell) Arteritis

Answer 8

Corticosteroids

High risk of blindness without treatment

Question 9

Vessels involved in Takayasu arteritis?

Answer 9

Branches of the aortic arch

Question 10

Presentation of Takayasu arteritis

Answer 10

Visual and neuro symptoms with weak or absent pulse in upper extremities (e.g. pulseless disease)

Question 11

Treatment of Takayasu arteritis

Answer 11

Corticosteroids

Question 12

What are the different populations affected by Giant cell vs Takayasu arteritis

Answer 12

Giant cell: adults > 50 y/o (usually elderly females)

Takayasu: adults < 50 y/o (usually young Asian female)

Question 13

What organs are affected in polyarteritis nodosa?

Answer 13

Multiple organs with sparing of the lungs

Question 14

Presentation of polyarteritis nodosa

Answer 14

HTN - renal artery

Abd pain with melena - mesenteric a.

Neuro disturbances

Skin lesions

Question 15

What vasculitis is serum HBsAg associated with?

Answer 15

Polyarteritis nodosa

Question 16

What is the appearance of polyarteritis nodosa on imaging

Answer 16

String-of-pearls appearance

Early lesion weaken the wall leading to aneurism

Late lesions heal with fibrosis

Question 17

Histology of polyarteritis nodosa

Answer 17

Transmural inflammation with fibrinoid necrosis (super pink)

Question 18

What population is typically affected by Kawasaki Disease?

Answer 18

Asian children < 4 y/o

Question 19

Presentation of Kawasaki Disease

Answer 19

Nonspecific signs that may appear as viral infection: fever, conjunctivitis, erythematous rash of palms and soles

Question 20

What vessel is often involved in Kawasaki Disease?

Answer 20

Coronary artery (thrombosis with MI or aneurysm with rupture)

Question 21

Treatment of Kawasaki Disease

Answer 21

Aspirin (to prevent thrombosis) and IVIG

Question 22

Presentation of Buerger Disease

Answer 22

Necrotizing vasculitis of digits (ulceration, gangrene, autoamputation of fingers and toes)

Question 23

What is the major risk factor of Buerger Disease

Answer 23

Smoking

Question 24

Treatment of Buerger Disease

Answer 24

Smoking cessation

Question 25

Affected organs/tissues in Wegener’s granulomatosis

Answer 25

Nasopharynx, lungs, and kidney

Triad:

Focal necrotizing vasculitis

Necrotizing granulomas in the lung and upper airway

Necrotizing glomerulonephritis

Question 27

Positive serum marker in Wegener’s granulomatosis

Answer 27

c-ANCA

Question 28

Treatment of Wegener’s granulomatosis

Answer 28

Cyclophosphamide and corticosteroids

Question 29

Difference in presentation between Wegener’s granulomatosis and Microscopic Polyangiitis

Answer 29

Both involve lung and kidney, but Microscopic Polyangiitis does not involve nasopharynx and granulomas are absent

Question 30

Positive serum marker in Microscopic Polyangiitis

Answer 30

p-ANCA

Question 31

Treatment of Microscopic Polyangiitis

Answer 31

Cyclophosphamide and corticosteroids

Question 32

Histology of Churg-Strauss Syndrome

Answer 32

Granulomatous inflammation with eosinophils

Question 33

How to differentiate between Churg-Strauss and Microscopic polyangiitis

Answer 33

Churg-Straus has granulomas, asthma, and peripheral eosinophilia

Question 34

Positive serum marker in Churg-Strauss

Answer 34

p-ANCA

Question 35

Cause of vasculitis in Henoch-Schonlein Purpura

Answer 35

IgA immune complex deposition

Question 36

What is the most common vasculitis in children?

Answer 36

Henoch-Schonlein Purpura

Question 37

Presentation of Henoch-Schonlein Purpura

Answer 37

Palpable purpura on buttocks and legs

Question 38

Disease often preceding Henoch-Schonlein Purpura

Answer 38

URI

(recall that Henoch-Schonlein Purpura is caused by IgA deposition)