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Cardio > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vascultis?

A

heterogenous group of uncommon (primarily autoimmune), occasionally life-threatening diseases having in common inflammation of blood vessels

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2
Q

What are the 2 major divisions of vasculitis?

A

1) infectious

2) non-infectous (primary)

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3
Q

What are the 3 most common causes of infectious vasculitis?

A

fungal- aspergillus
bacterial- pseudomonas
viral- CMV

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4
Q

How might giving steroid therapy to a patient with infectious vasculitis kill them?

A

steroids are anti-inflammatory and giving steroids to a patient with an infection will kill off all the things needed to mount an immune response to get rid of the infection

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5
Q

What is giant cell arteritis?

A

temporal arteritis - multinucleated giant cell attack on calcified internal elastic lamina in arteries or calcified media of thoracic aorta

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6
Q

What are the primary immune cells in arteries?

A

immature dendritic cells

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7
Q

How do dentritic cells get activated in he blood vessels?

A

antigens from pathogens or calcifications litigate TLRs which stimulate the PAMP/DAMP pathway whcih activates intracellular TRAM that stimulates NFkB to enter the nucleus and increase transcription of pro-inflammatory cells

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8
Q

What is the major dendritic receptor in adventitial cells?

A

TLR-4

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9
Q

Activated dendritic cells have what 3 functions?

A

1) Produce IL-12 and IL-18
2) Release lots of IFN-gamma, IL-2, and IL-6 that recruit inflammatory cells and increase T cell proliferation
3) Release “homing chemokines”

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10
Q

What is a “homing chemokine”? What does it do?

A

CCL19 and CCL21 bind to CCR7 and “trap” dendritic cells in the vessel

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11
Q

Progression of immune response in giant cell arteritis depends on what two cell types?

A

Th1

Th17

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12
Q

What does Th1 do to progress giant cell arteritis?

A

Release IL-12 and IFN-gamma that upregulates VEGF and PDGF which leads to lumen stenosis and an inflammatory loop where VEGF stimulates production of IFN-gamma that leads to giant cells and granuloma formation

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13
Q

How does Th17 progress giant cell arteritis?

A

release IL-1, -6, and -17 which create a proinflammatory environment that recruits activated monocytes to the arterial wall

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14
Q

What is released by macrophages and SM cells to destroy elastin and lead to matrix degeneration, intimal hyperplasia, and lumenal narrowing?

A

MMP-2 and MMP-9

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15
Q

What is the epidemiology of giant cell (temporal) arteritis?

A

Common, primarily in elderly white females (>50) of Northern European descent

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16
Q

What are the most common symptoms of giant cell arteritis?

A 
headache
Visual disturbances--> can lead to blindness
Jaw claudicaiton
Swollen, tender artery
Scalp tenderness
FEVER
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17
Q

What vessels are affected in giant cell arteritis?

A

Involves temporal and ophthalmic arteries and thoracic aorta

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18
Q

What inflammatory disease is typically associated with temporal arteritis?

A

polymyalgia rheumatica (40% of cases)

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19
Q

How do you diagnose temporal arteritis?

A

high ESR (over 40 mm/hour) and positive biopsy (of temporal artery)

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20
Q

How do you treat temporal arteritis?

A

steroids (sometimes aspirin)

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21
Q

What is Takayasu Arteritis?

A

Granulomatous vasculitis of aortic arch at branch points with transmural scarring and thickening and severe luminal narrowing of branches (VERY similar to giant cell arteritis)

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22
Q

What factor is key in diagnosing Takayasu arteritis versus temporal arteritis?

A

AGE - same diagnosis and treatment, but Takayasu arteritis occurs in patients younger than 50 y/o

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23
Q

What are the symptoms of Takayasu arteritis

A

ocular disturbances and marked weakening of pulses in upper extremities, neurological problems

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24
Q

What is polyarteritis nodosa?

A

Vasculitis of small or medium-sized muscular arteries that typically involves renal and visceral vessels and spares pulmonary circulation. Healing of the lesions leaves behind some fibrosis

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25
Q

Polyarteritis Nodosa is commonly associated with what?

A

Hepatitis B immune complex formation

26
Q

What is unique about the morphology of polyarteritis nodosa?

A

see lesions at all stages of activity suggesting ongoing & recurrent pathogenic insults

27
Q

What are the signs/symptoms of polyarteritis nodosa?

A

HTN (renal involvement), melena and abdominal pain, neurologic disturbances, skin lesions (SYSTEMIC SYSTEMS)

28
Q

What would a skin biopsy of polyarteritis nodosa show?

A

fibrinoid necrosis (NOT FIBRIN), inflammatory cells that surround the vessels and all the wall through the wall (transmural)

29
Q

Who gets polyarteritis nodosa?

A

middle aged men

30
Q

Why is polyarteritis nodosa a must-not-miss diagnosis?

A

because it is 90% treatable with immunosuppression (cyclophosphamide) and 100% fatal if non-treated

31
Q

What leads to death in polyarteritis nodosa?

A

renal failure

32
Q

What is Kawasaki disease?

A

(mucocutaneous lymph node syndrome) an acute childhood primary vasculitis of medium arteries (especially coronaries)

33
Q

Who gets Kawasaki disease?

A

1-2 year old children, slightly more boys, an more common in Japanese

34
Q

What are the symptoms of Kawasaki disease?

A 
persistent high fever
conjunctivitis
mucosal and skin erythema
edema (palms and soles with peeling)
erythematous maculopapular skin rash
cervical lymphadenopathy
strawberry tongue
35
Q

What are the complications of Kawasaki disease?

A

arterial rupture
MI
death

36
Q

What is the proposed pathogenesis in Kawasaki disease?

A

aberrant immune reaction to a ubiquitous RNA virus that can become persistent in genetically predisposed individuals

37
Q

What is the proposed treatment for Kawasaki disease?

A

aspirin with IV Ig but usually self-limited

38
Q

What is Microscopic Polyangitis?

A

Necrotizing vasculitis that generally affects capillaries and is a feature of immune disorders like Henoch-Schonlein purpura, antibody responses to drugs, etc.

39
Q

What is the immunologic cause of microscopic polyangitis?

A

MPO-ANCA lysosomal granule constituent involved in free-radical generation

40
Q

What organs are typically affected by microscopic polyangitis?

A

kidney and lungs

41
Q

What are the major complications of microscopic polyangitis?

A

necrotizing glomerulonephritis and pulmonary capillaritis

42
Q

How do you treat microscopic polyangitis?

A

immmunosuppression and removal of offending agent.

43
Q

What is the other name of Wenger’s Granulomatosis?

A

granulomatosis with polyangiitis

44
Q

What is granulomatosis with polyangiitis?

A

Cell-mediated hypersensitivity response directed against inhaled infectious or environmental agents.

45
Q

What is the immunologic cause of granulomatosis with polyangiitis?

A

Tissue injury driven by PR3-ANCA

46
Q

What does a biopsy of granulomatosis with polyangiitis look like?

A

biopsy shows large necrotizing granulomas and geographic necrosis with basophilic debris (nuclear dust)

47
Q

What organs are affected by granulomatosis with polyangiitis?

A

nasopharynx, lung, kidney

48
Q

What is the typical presentation of granulomatosis with polyangiitis?

A

40 y/o man with pneumonitis, sinusitis, mucosal ulcerations, renal disease

49
Q

What are the signs and symptoms of granulomatosis with polyangiitis?

A

Triad: granulomas of lung and URT, vasculitis, glomerulonephritis.

50
Q

How do you treat granulomatosis with polyangiitis?

A

cyclophosphamide and steroids

51
Q

What is Churg-Strauss Syndrome?

A

Hyperresponsiveness to normally innocuous allergic stimulus

52
Q

What is the immunologic cause of Churg-Strauss Syndrome?

A

MPO-ANCAs

53
Q

What are the signs and symptoms of Churg-Strauss Syndrome?

A

asthma, eosinophilia, necrotizing granulomatous inflammation!

54
Q

What are the complications of Churg-Strauss symtome?

A

cardiomyopathy from cardiac involvemnt, palpable purpura, renal disease, GI bleeds

55
Q

What are the possible immunologic causes of vasculitis?

A

Immune complex deposition
ANCAs
Anti-endothelial cell antibodies
Autoreactive T cells

56
Q

What are the 2 types of vasculitis with immune complex eposition?

A
  • Drug-hypersensitivity vasculitis (Ab’s against drug modified proteins)
  • Vasculitis secondary to infections (Ab to microbial constituents form immune complexes that circulate and deposit in vascular lesions)–> ex. polyarteritis nodosa
57
Q

What do ANCAs do?

A

directly activate neutrophils, stimulating the release of ROS and proteolytic enzymes leading to endothelial cell injury.

58
Q

What is PR3-ANCA? When do you see it?

A

azurophilic granule constituent that shares homology with numerous microbial peptides (in Wenger granulomatosis)

59
Q

What is MPO-ANCA? When do you see it?

A

lysosomal granule constituent involved in free-radical generation—associated with microscopic polyangiitis and C-S syndrome

60
Q

When do you see anti-endothelial cell antibodies?

A

Kawasaki disease

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