vasculitis

Question 1

Describe Giant Cell Arteritis

Answer 1

Most common form of vasculitis among older individuals in the US and Europe

T cell-mediated response against vessel wall antigens that drive proinflammatory cytokine production

Typically occurring in the temporal, vertebral or ophthalmic arteries

Question 2

Discern Giant cell arteritis from Takayasu arteritis

Answer 2

Giant cell arteritis occurs in people > 50 y/o

Pulseless Disease typically occurs in people < 50y/o

• can cause syncope and angina due to the disparity in blood pressure

Question 3

Describe the morphology seen on biopsy of Giant cell arteritis and Pulseless Disease

Answer 3

medial granulomatous inflammation centered on the internal elastic lamina that produces elastic lamina fragmentation

Question 4

Another name for Kawasaki Disease

Answer 4

Mucocutaneous Lymph Node Syndrome

Question 5

Clinical Presentation of Kawasaki disease

Answer 5

Conjunctivitis

Rash

Adenopathy of cervical lymph nodes

Strawberry tongue

Hand/ feet edema

5+ days of fever

Question 6

what is polyarteritis nodosa

Answer 6

systemic vasculitis of small-medium sized arteries, typically involving the renal and visceral vessels and sparing the pulmonary circulation

Question 7

Risk of Kawasaki Disease

Answer 7

predilection for coronary aneurysms

Question 8

clinical presentation of PAN

Answer 8

rapidly accelerating HTN in adults 45-65 y/o

fever/ malaise

infarction causing: renal, abd or neurologic impairment

rash/ ulcerations/ nodules

PAN RAN a fever

Question 9

Diagnostics of PAN

Answer 9

30% of patients have chronic HepB with antigen complex deposition

negative ANCA

Question 10

morphology of PAN

Answer 10

vessels at various stages of acute and chronic segmental transmural necrotizing inflammation of small-medium sized arteries

acute stage fibrinoid necrosis

Question 11

how is PAN treated

Answer 11

cyclophosphamide

glucocorticoids

Question 12

Another name for Thromboangitis Obliterans

Answer 12

Buerger Disease

Question 13

Describe Buerger Disease

Answer 13

segmental thrombosing acute and chronic inflammation of the small-medium arteries with occasional secondary extension into the veins and nerves of the extremities

Question 14

Clinical presentation of Thromboangitis obliterans

Answer 14

Men >35y/o

smoker

intermittent claudication

necrosis of digits/ amputation

Raynaud phenomenon

chronic extremity ulcerations/ gangrene

Question 15

morphology of Buerger Disease

Answer 15

nonatherosclerotic segmental occlusion of arteries

Question 16

Another name or Granulomatosis with polyangiitis

Answer 16

Wegner Disease

Question 17

what is Wegner Disease

Answer 17

T cell-mediated hypersensitivity in response to innocuous microbial or other environmental agents

Question 18

Clinical Presentation of Granulomatosis with polyangiitis

Answer 18

Looks like PAN wit respiratory involvement

fever/ malaise

rash/ nodules

adenopathy of cervical lymph nodes

neuro/renal/abd and respiratory involvement

Necrotizing granulomas within the respiratory tract

chronic otitis media and mastoiditis

rapidly progressive glomerulonephritis

Question 19

DIagnostics of Wegner Disease

Answer 19

PRE-ANCA/cANCA

multiple bilateral cavitating nodular lesions on CXR

Question 20

Morphology of Granulomatosis with polyangiitis

Answer 20

granulomas with geographic patterns of central necrosis and accompanying vasculitis

-surrounded by a zone of proliferating fibroblasts associated wit giant cels ad leukocyte infiltrate

During late stages, there is extensive necrotizing granulomatous involvement of the parenchyma, leading to alveolar hemorrhage

Question 21

Another name or Churg-Strauss

Answer 21

Eosinophilic granulomatosis with polyangiitis

Question 22

what is Eosinophilic granulomatosis with polyangiitis

Answer 22

small vessel necrotizing vasculitis with hyperresponsiveness to allergic stimuli and leukotriene receptor antagonists in asthmatics

Question 23

clinical presentation of Eosinophilic granulomatosis with polyangiitis

Answer 23

severe allergic asthma/ sinusitis

palpable purpura

peripheral neuropathy

cardiomyopathy with eosinophilic infiltrates

Question 24

diagnostics of Churg-Strauss

Answer 24

MPO-ANCA/ p-ANCA

eosinophilia

increased IgE

Question 25

Morphology of Churg Straus

Answer 25

vessels with various stages of inflammation, eosinophils, necrotizing granulomas, and fibrinoid necrosis ## Footnote **similar to PAN but with eosinophils and granulomas**

Question 26

what is microscopic polyangiitis

Answer 26

necrotizing vasculitis that generally affects capillaries, small arterioles, and venules

Question 27

other names for microscopic polyangiitis

Answer 27

hypersensitivity vasulitis leukocytoclastic vasculitis

Question 28

clinical presentation of microscopic polyangiitis

Answer 28

HTN necrotizing glomerulonephritis palpable purpura pulmonary capillaritis hemoptysis hematuria proteinuria ad pain

Question 29

Diagnostics of microscopic polyangiitis

Answer 29

MPO-ANCA/p-ANCA

Question 30

Morphology of microscopic polyangiitis

Answer 30

lesions appear to be the same age, but without granulomatous inflammation. segmental fibrinoid necrosis o the media and focal transmural necrotizing lesions **similar to PAN, but spares medium and large arteries**

Question 31

Most common vasculitis in children

Answer 31

IgA Vasculitis

Question 32

another name for IgA Vasculitis

Answer 32

Henoch-Schonlein Purpura

Question 33

Clinical presentation of IgA vasculitis

Answer 33

child with palpable purpura in lower limbs arthralgias colicky abd pain, vomiting, melena hematuria spontaneous recovery in 4 months **Associated with Buerger disease**

Question 34

how do you treat IgA Vasculitis

Answer 34

NSAIDS Glucocorticoids IV hydration Supportive care

Question 35

what is cryoglobulinemic vasculitis

Answer 35

vasculitis due to **mixed** IgG and IgA deposition

Question 36

Clinical symptoms of cryoglobulinemic vasculitis

Answer 36

**fatigue** **arthralgias** **palpable purpura** glomerulonephritis peripheral neuropathy

Question 37

associations of cryoglobulinemic vasculitis

Answer 37

viral infection Hep C

Question 38

treatment for cryoglobulinemic vasculitis

Answer 38

glucocorticoids cyclophosphamide Hep C treatment (IFN-alpha/ ribavirin)

Question 39

clinical presentation of Bechet Disease

Answer 39

Middle eastern/ Japanese mucosal ulcers uveitis erythema nodosum

Question 40

Diagnostics of Bechet disease

Answer 40

associated with HLA-B51 cross-reacts wit TH17 microbes

Question 41

treatment for Bechet disease

Answer 41

glucocorticoids

Question 42

Compare/ Contrast Primary and Secondary Raynaud phenomenon

Answer 42

**Primary:** * presents as exaggerated central and local vasomotor responses to cold or emotion * typically occurring in young women * s_ymmetrically affects the extremities_ with no structural changes to the vessel wall * Chronically appears as atrophy, ulcers and ischemic gangrene **Secondary** * presents as vascular insufficiency due to arterial disease * typically occurs with SLE, scleroderma, Buerger Disease, and atherosclerosis with _asymmetric involvement of the extremities_ * worsens over time