Path 20- Vasculitis/tumors

Front 1

C-ANCA acts against

Back 1

PR3- proteinase 3 in neutrophils

Front 2

P-ANCA acts against

Back 2

MPO- myeloperoxidase

Front 3

C-ANCA indicative of

Back 3

Wegener Granulomatosis

Front 4

P-ANCA indicative of

Back 4

Microscopic Polyarteritis
Churg Strauss Syndrome

Front 5

Fragmentation of internal elastic lamina

Back 5

Giant cell arteritis (Temporal arteritis)

Front 6

Clinical symptoms:
1- Fever, fatigue, weight loss
2- Pain or headache along course of a tender, nodular, temporal artery
3- Vision changes
4- Myocardial ischemia, GI, neurologic changes
5- Polymyalgia rheumatica

Back 6

Giant cell arteritis

Front 7

Difference between Takayasu disease and temporal arteritis

Back 7

1- YOUNGER <40 yrs old Japanese women
2- Aortic arch affected

Front 8

Polyarteritis nodosa microscope

Back 8

Artery with segmental, transmural inflammation with fibrinoid necrosis

Front 9

Clinical Polyarteritis

Back 9

1) Skin lesions- purpura, livedo reticularis, Infarcts of tips of digits
2) Renal- HTN, failure
3) Peripheal neuropathy
4) Fever, weight loss, malaise
5) GI- Adbdominal pain, bleeding, pain
6 )Aneurysm
7) Hepatitis B
8) Testicular pain
Vessels at different (ALL) stages of inflammation
Remember doesn't affect lungs and NO glomerulonephritis!!!

Front 10

Major cause of death of Polyarteritis Nodosa

Back 10

Renal involvement

Front 11

What does polyarteritis nodosa NOT affect?

Back 11

Lungs!

Arterioles, venules, capillaries

Front 12

How is microscopic polyarteritis or polyangitis different from PAN? (4)

Back 12

1- Affects arterioles, capillaries, venules
2- Inflammatory lesions of the SAME stage
3- p-ANCA present in 80% of cases
4- Necrotizing glomerulonephritis and pulmonary capillaritis are common

Front 13

Leukocytoclastic vasculitis lesions are

Back 13

Purpuras

Front 14

Churg-Strauss Syndrome features

Back 14

Age- 50 years, SMALL VESSELS
1- Allergic rhinitis
2- Asthma
3- Eosinophila
4- P-ANCA

Front 15

Triad features in Wegener granulomatosis

Back 15

1- Necrotizing granulomatous vasculitis
2- Necrotizing granulomata of lungs and upper airways
3- Glomerulonephritis

Front 16

c ANCA and Wegener granulomatosis

Back 16

C-ANCA is positive in 90% of cases

Front 17

Wegener's granulomatosis

What is it?

Symptoms

Findings

Treatment

Back 17

Triad of:
1- Focal necrotizing vasculitis
2- Necrotizing granulomas in the lung and upper airway
3- Necrotizing glomerulonephritis
-Affects medium and small vessels

Symptoms- hemoptysis, hematuria, perforation of nasal septum

Findings- C-ANCA is strong marker

Front 18

Buerger's Disease (Thromboangiitis obliterans)

Pathogenesis
Clinical features

Back 18

Pathogenesis- Cigarette smoking causes endothelial cell damage due to direct cytotoxicity or hypersensitivity

Clinical
1) Raynaud
2) Pain in instep area
3) Chronic ulcers, gangrene of digits, feet

Front 19

What happens in Raynaud's Phenomenon?

Back 19

White, blue, and red response

Front 20

Primary Raynaud's Phenomenon is caused by

Back 20

Unknown

Front 21

Secondary Raynaud's Phenomenon is caused by

Back 21

Arterial insufficiency due to diseases such as SLE, SS, AS, or Buerger's Disease

Front 22

Difference between juvenile vs cavernous hemangiomas (port-wine stains)

Back 22

Cavernous hemangiomas do not regress

Front 23

Pyogenic granuloma

Back 23

Occurs after trauma in skin, gingival, or oral mucosa

Front 24

Cystic hygroma is what

Back 24

Cavernous lymphangioma, occurs in neck or axilla
-Associated with Turner's Syndrome

Front 25

One major cause of concern of Kawasaki disease

Back 25

Coronary vasculitis can cause thrombosis and aneurysm and lead to MI

Front 26

Giant cell arteritis affects what arteries (4)

Back 26

Aortic arch
Vertebral
Ophthalmic
Temporal

Front 27

Clinical features of Giant cell arteritis

Back 27

1- Constitutional symptoms (fever, fatigue, weight loss)
2- Tender, nodular. temporal artery
3- Ocular features- diplopia
4- Polymyalgia rheumatica

Front 28

What is Pulseless disease?

Back 28

Takayasu Disease

Front 29

First step when you suspect Takayasu Arteritis

Back 29

Aortic Arch Angiogram

Front 30

Giant cell arteritis vs Takayasu- how to tell the difference?

Back 30

Takayasu- under 40 yrs and pulselessness of upper extremities, affect branches of aortic arch more

Front 31

What does PAN NOT affect?

Back 31

Arterioles, capillaries, venules
Pulmonary vessels

No glomerulonephritis

Front 32

PAN

Back 32

1- Arterioles, capillaries, venules not affected
2- Different stages of inflammation seen
3- Segmental, transmural, fibrinoid necrosis
4- No association with ANCA
-Aneurysms, renal failure, heart, bowels, skin, testicle

Front 33

PAN is fatal if untreated

Back 33

Notes

Front 34

Differentiate PAN and Microscopic Polyangiitis

Back 34

Microscopic Polyarteritis affects
1) Arterioles, capillaries, venules, glomerulonephritis is possible
2) Pulmonary capillarities/glomerulonephritis
3) p-ANCA present
4) inflammatory stages are of the SAME stage (either acute, subacute, chronic, repair)

Front 35

Leukocytoclastic or hypersensitivity vasculitis (occurs in microscopic polyarteritis)

Back 35

Hypersensitivity reactions with skin lesions like purpura

Pauci immune- no or few immune deposits

Front 36

Pauci immune means

Back 36

No or few immune deposits

Front 37

Churg-Strauss Syndrome characteristics (4)

Back 37

1- Allergic Rhinitis
2- Asthma
3- Eosinophilia
4- P-ANCA positive

Front 38

How does Churg-Strauss kill someone?

Back 38

Coronary arteritis or myocardial infarction

Front 39

With multiple organ lesions, musculoskeletal, renal, GI, skin, peripheral NS...think about

Back 39

PAN

Front 40

4 things to remember about Wegener's

Back 40

1. necrotizing granulomatous vasculitis
2. necrotizing granulomata of tissues
3. glomerulonephritis ( focal or crescentic)
4. C-ANCA positive (90%)

Front 41

What is Mucocutaneous Lymph Node Syndrome?

Back 41

Kawasaki Syndrome

Front 42

Clincher for diagnosis for Kawasaki Disease

Back 42

Auto Abs to endothelial and smooth muscle cells

Front 43

What is the major concern for Kawasaki Disease?

Back 43

Coronary vasculitis can cause aneurysm formation, thrombosis, and lead to an MI

Front 44

Features of Kawasaki Disease (4)

Back 44

1- Skin- desquamating rash, erythema of palms and soles
2- Mucosa- conjuctival and oral erythema
3- Lymph nodes- cervical lymphadenopathy
4- Fever

Front 45

Buerger Disease affects what arteries?

Back 45

Tibial and Radial Arteries

Front 46

Tibial and Radial Arteries

Back 46

Buerger Disease

Front 47

Cigarette Smoking is a major risk factor in

Back 47

Buerger Disease (Thromboangiitis Obliterans)

Front 48

What is a cystic hygroma?

Back 48

Cavernous Lymphangioma- occur in the neck or axilla

Front 49

Kaposi Sarcoma is caused by

Back 49

Human Herpes Virus 8

Front 50

4 Types of Kaposi Sarcoma

Back 50

1- Chronic or classic
2- Lymphadenopathic- aggressive
3- Transplant Associated
4- AIDS associated

Front 51

Microscopic polyangiitis/polyarteritis is also called

Back 51

Hypersensitivity or leukocytoclastic vasculitis

Front 52

Churg-Strauss Syndrome is also called

Back 52

Allergic granulomatosis and angiitis

Front 53

Kawasaki Syndrome is also called

Back 53

Mucocutaneous Lymph Node Syndrome

Front 54

Buerger Disease is also called

Back 54

Thromboangiitis obliterans

Front 55

What is hemangiosarcoma, or an angiosarcoma?

Back 55

Malignant endothelial neoplasm
Occurs in the elderly
Involves skin, soft tissue, breast, liver
Associated with radiation, arsenic, thorotrast, and PVC (Polyvinyl chloride)

Front 56

When you see radiation, arsenic, thorotrast, and PVC think...

Back 56

Hemangiosarcoma

Front 57

CD 31, 34, cWF think

Back 57

Hemangiosarcoma

Front 58

Round nodules in subcutaneous tissue

Back 58

Bacillayr angiomatosis

Front 59

Main site of syphilitic aneurysm

Back 59

Thoracic aorta

Front 60

Cause of syphilitic aneurysm

Back 60

Treponema Pallidum

Front 61

Tree barking of intima of aorta

Back 61

Syphylitic aneurysm

Front 62

Most common cause of death of syphilitic aneurysm

Back 62

Cardiac failure due to valve incompetence

Front 63

Angiosarcoma

Back 63

Malignant endothelial
-Liver angiosarcoma associate with exposure to polyvinylchloride, arsenic, thorium dioxide

Front 64

Bacillary angiomatosis

Back 64

caused by Bartonella henselae, common in AIDS
-Round nodules in subcutaneous tissues
Microscope- Capillaries with eipthelioid endothelial cells

Front 65

Kaposi's sarcoma

Back 65

Malignant tumor arising from endothelial cells or primitive mesenchymal cells
-Human Herpes Virus 8
-Raised red-purple lesions that progress from flat-->plaque-->nodule that ulcerates

Front 66

Lymphangiosarcoma

Back 66

Malignancy of lymphatics
-Caused by long-standing chronic lymphedema (after modified radical mystectomy)

Front 67

Pyogenic granuloma

Back 67

Vascular, red pedunculated mass that ulcerates and bleeds easily
-Trauma and pregnancy

Front 68

Marfan's syndrome is a cause of

Back 68

Aortic dissection

Front 69

Monkenger's Arteriosclerosis

Back 69

Calcification of the media in extremities
-NO CLINICAL SIGNIFCANCE!!!