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CRRAB Week 5 - WLB > Vasculitis - Pathoma > Flashcards

Vasculitis - Pathoma Flashcards

1
Q

What is the definition of vasculitis?

A

inflammation of the blood vessel wall

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2
Q

What is the intima composed of in a blood vessel?

A

endothelial cells sitting on a basement membrane

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3
Q

What is the etiology of most cases of vasculitis?

A

Unknown (most cases are not infectious)

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4
Q

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

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

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

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5
Q

What are the three types of large vessel vasculitis?

A
  1. Temporal (Giant cell) Arteritis
  2. Takayasu Arteritis
  3. Buerger Disease
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6
Q

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

A

Temporal (Giant cell) Arteritis

usually affects females

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7
Q

What large vessels does Temporal Arteritis affect?

A

Branches of the carotid artery

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8
Q

What are the classic symptoms of Temporal Arteritis?

A
  • Headache (temporal artery)
  • Visual disturbances (ophthalmic artery)
  • Jaw claudication
  • Flu-like symptoms with joint and muscle pain (polyrheumatic myalgia)
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9
Q

What is the classic lab result in Temporal Arteritis?

A

Elevated ESR (>100)

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10
Q

What is seen with biopsy in Temporal Arteritis?

A

Inflammed vessel wall with giant cells and intimal fibrosis

-granulomatous vasculitis

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11
Q

What is the treatment for Temporal Arteritis?

A

Corticosteroids ASAP! (risk of blindness)

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12
Q

How is Takayasu Arteritis different than Temporal Arteritis?

A

-Presents in adults

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13
Q

What are the symptoms of Takayasu Arteritis?

A
  • Visual and neurologic symptoms

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

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14
Q

What lab is elevated in Takayasu Arteritis?

A

ESR

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15
Q

What is the treatment for Takayasu Arteritis?

A

Corticosteroids

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16
Q

What are the two types of medium vessel vasculitis?

A
  1. Polyarteritis nodosa

2. Kawasaki’s Disease

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17
Q

What kind of vessels are affected in medium vessel vasculitis?

A

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

18
Q

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

A

Polyarteritis Nodosa

19
Q

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

A

Hypertension

20
Q

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

A

Abdominal pain with melena

21
Q

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

A

Polyarteritis Nodosa

22
Q

What are the classic symptoms of Polyarteritis Nodosa?

A
  • HTN
  • Abdominal pain with melena
  • Neurologic disturbances
  • Skin lesions
23
Q

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

A

“String-of-pearls”

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

24
Q

What is the treatment of Polyarteritis Nodosa?

A

Corticosteroids and Cyclophosphamide

fatal if not treated

25
Q

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

A
  • Asian children under age 4

- Nonspecific symptoms: fever, conjunctivities, rash of palms and soles, enlarged cervical lymph nodes

26
Q

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

A

CORONARY ARTERY!

  • thrombosis with MI
  • aneurysm with rupture
27
Q

What is the treatment for Kawasaki’s Disease?

A
  • Aspirin (prevents TXA2 & platelet aggregation)
  • IVIG
  • Disease is self-limited
28
Q

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

A

Buerger’s Disease

29
Q

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

A

Smoking => Tx is to stop smoking!

30
Q

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

A

Raynaud Phenomenon

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

31
Q

What are the four types of small vessel vasculitis?

A
  1. Wegener’s granulomatosis
  2. Microscopic polyangiitis
  3. Churg-Strauss Syndrome
  4. Henoch-Schonlein Purpura
32
Q

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

A

Wegener’s granulomatosis

33
Q

What is the typical presentation of Wegener’s granulomatosis?

A

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

34
Q

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

A

c-ANCA

serum antineutropil cytoplasmic antibodies

35
Q

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

A

Large necrotizing granulomas with adjacent necrotizing vasculitis

36
Q

What is the key treatment of Wegener’s Granulomatosis?

A

Corticosteroids + Cyclophosphamide

relapses are common

37
Q

How is Microscopic Polyangiitis different from Wegener’s Granulomatosis?

A
  • Involves lung and kidney ONLY (no nasopharyngeal involvement)
  • No granulomas
  • see p-ANCA on lab findings
38
Q

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

A

Churg-Strauss Syndrome

39
Q

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

A 
CS:
-granulomas
-asthma
-peripheral eosinophilia
MP:
-no Hx of asthma
-no asthma
40
Q

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

A

HSP

Henoch-Schonlein Purpura

41
Q

What is the most common clinical findings of HSP?

A
  • **PALPABLE purpura on the buttocks and legs
  • hematuria (IgA nephropathy)
  • GI pain and bleeding
  • post-URI
42
Q

How do you treat HSP?

A
  • Self-limited

- If severe, steroids help

CRRAB Week 5 - WLB (5 decks)

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