Vasculitis - Pathoma

Question 1

What is the definition of vasculitis?

Answer 1

inflammation of the blood vessel wall

Question 2

What is the intima composed of in a blood vessel?

Answer 2

endothelial cells sitting on a basement membrane

Question 3

What is the etiology of most cases of vasculitis?

Answer 3

Unknown (most cases are not infectious)

Question 4

What are the two types of symptoms that present in vasculitis?

Answer 4

1. Nonspecific (fever, fatigue, weight loss, myalgias, etc.)

2. Sx of organ ischemia (organ dependent, thrombosis, glomerulonephritis, etc.)

Question 5

What are the three types of large vessel vasculitis?

Answer 5

1. Temporal (Giant cell) Arteritis
2. Takayasu Arteritis
3. Buerger Disease

Question 6

What is the most common form of vasculitis in older adults (>50yoa)?

Answer 6

Temporal (Giant cell) Arteritis

usually affects females

Question 7

What large vessels does Temporal Arteritis affect?

Answer 7

Branches of the carotid artery

Question 8

What are the classic symptoms of Temporal Arteritis?

Answer 8

• Headache (temporal artery)
• Visual disturbances (ophthalmic artery)
• Jaw claudication
• Flu-like symptoms with joint and muscle pain (polyrheumatic myalgia)

Question 9

What is the classic lab result in Temporal Arteritis?

Answer 9

Elevated ESR (>100)

Question 10

What is seen with biopsy in Temporal Arteritis?

Answer 10

Inflammed vessel wall with giant cells and intimal fibrosis

-granulomatous vasculitis

Question 11

What is the treatment for Temporal Arteritis?

Answer 11

Corticosteroids ASAP! (risk of blindness)

Question 12

How is Takayasu Arteritis different than Temporal Arteritis?

Answer 12

-Presents in adults

Question 13

What are the symptoms of Takayasu Arteritis?

Answer 13

• Visual and neurologic symptoms

- Weak or absent pulse in an upper extremity (“pulseless disease”)

Question 14

What lab is elevated in Takayasu Arteritis?

Answer 14

ESR

Question 15

What is the treatment for Takayasu Arteritis?

Answer 15

Corticosteroids

Question 16

What are the two types of medium vessel vasculitis?

Answer 16

1. Polyarteritis nodosa

2. Kawasaki’s Disease

Question 17

What kind of vessels are affected in medium vessel vasculitis?

Answer 17

muscular arteries that supply organs (e.g. renal arteries)

Question 18

What type of necrotizing vasculitis involves most organs, BUT spares the lungs?

Answer 18

Polyarteritis Nodosa

Question 19

What symptom is present if a patient has Polyarteritis Nodosa of the renal artery?

Answer 19

Hypertension

Question 20

What symptom is present if a patient has Polyarteritis Nodosa of the mesenteric artery?

Answer 20

Abdominal pain with melena

Question 21

What type of vasculitis often has a positive serum antibody for hepatitis B (HBsAg)?

Answer 21

Polyarteritis Nodosa

Question 22

What are the classic symptoms of Polyarteritis Nodosa?

Answer 22

• HTN
• Abdominal pain with melena
• Neurologic disturbances
• Skin lesions

Question 23

What classic vessel lesions are present on imaging in Polyarteritis Nodosa?

Answer 23

“String-of-pearls”

-due to transmural inflammation (dilation) with characteristic fibrinoid necrosis (nodes)

Question 24

What is the treatment of Polyarteritis Nodosa?

Answer 24

Corticosteroids and Cyclophosphamide

fatal if not treated

Question 25

What is the typical presentation of a patient with Kawasaki's Disease?

Answer 25

- Asian children under age 4 | - Nonspecific symptoms: fever, conjunctivities, rash of palms and soles, enlarged cervical lymph nodes

Question 26

What is the preferential artery involved with Kawasaki's Disease?

Answer 26

CORONARY ARTERY! - thrombosis with MI - aneurysm with rupture

Question 27

What is the treatment for Kawasaki's Disease?

Answer 27

- Aspirin (prevents TXA2 & platelet aggregation) - IVIG - Disease is self-limited

Question 28

What type of vasculitis is a necrotizing vasculitis that involves the digits and presents with ulceration, gangrene, and autoamputation of fingers and toes?

Answer 28

Buerger's Disease

Question 29

What lifestyle factor is highly associated with Buerger's Disease?

Answer 29

Smoking => Tx is to stop smoking!

Question 30

What unique condition is often associated with Buerger's disease?

Answer 30

Raynaud Phenomenon | -vasospasm cuts off blood supply to fingers (white => blue => red)

Question 31

What are the four types of small vessel vasculitis?

Answer 31

1. Wegener's granulomatosis 2. Microscopic polyangiitis 3. Churg-Strauss Syndrome 4. Henoch-Schonlein Purpura

Question 32

What type of vasculitis is a necrotizing granulomatosis vasculitis that involves the nasopharynx, lungs, and kidneys?

Answer 32

Wegener's granulomatosis

Question 33

What is the typical presentation of Wegener's granulomatosis?

Answer 33

Middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis, nodular lung infiltrates, and hematuria.

Question 34

What unique lab test is positive in Wegener's granulomatosis?

Answer 34

c-ANCA | serum antineutropil cytoplasmic antibodies

Question 35

What is the key finding on biopsy in the setting of Wegener's Granulomatosis?

Answer 35

Large necrotizing granulomas with adjacent necrotizing vasculitis

Question 36

What is the key treatment of Wegener's Granulomatosis?

Answer 36

Corticosteroids + Cyclophosphamide | relapses are common

Question 37

How is Microscopic Polyangiitis different from Wegener's Granulomatosis?

Answer 37

- Involves lung and kidney ONLY (no nasopharyngeal involvement) - No granulomas - see p-ANCA on lab findings

Question 38

What type of small vessel vasculitis involves necrotizing granulomatous vasculitis with eosinophils?

Answer 38

Churg-Strauss Syndrome

Question 39

What are the three features of Churg-Strauss Syndrome that help you differentiate it from Microscopic Polyangiits when there is a +p-ANCA?

Answer 39

``` CS: -granulomas -asthma -peripheral eosinophilia MP: -no Hx of asthma -no asthma ```

Question 40

What small vessel vasculitis is due to IgA immune complex deposition and is the most common vasculitis in kids?

Answer 40

HSP | Henoch-Schonlein Purpura

Question 41

What is the most common clinical findings of HSP?

Answer 41

* **PALPABLE purpura on the buttocks and legs - hematuria (IgA nephropathy) - GI pain and bleeding - post-URI

Question 42

How do you treat HSP?

Answer 42

- Self-limited | - If severe, steroids help