Vasculitides Flashcards

1
Q

What is the most common arteritis in adults?

A

temporal (giant cell) arteritis

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2
Q

What is the most common arteritis in children?

A

Henoch-Schönlein purpura

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3
Q

What is temporal (giant cell) arteritis?

What vessels does it typically effect and what changes occur in the vessel?

A

granulomatous, large vessel vasculitis

Vessels:

-carotid branches (cranial arteries) , particularly temporal artery

Changes:

  • segmental granulomatous changes, multinucleated giant cells
  • fibrosis
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4
Q

What is the clinical presentation of temporal (giant cell) arteritis?

A

Constitutional Sx:

  • fever, malaise, weight loss
  • headache (temporal A.; actually occurs along artery which may also be TTP)
  • visual changes (ophthalmic A.); **sudden loss of vision**
  • jaw claudication (facial A.)
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5
Q

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

A
  • more common in females
  • older adults (>50)
  • european descent
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6
Q

What are diagnostic features of temporal (giant cell) arteritis?

A

-segmental granulomatous changes noted on temporal A. biopsy (because of segemental nature, false negative biopsy can occur)

-elevated ESR

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7
Q

What is the treatment and prognosis for temporal (giant cell) arteritis?

A

-corticosteroids upon ***suspicion of disease*** to prevent blindness (even before biopsy)

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8
Q

What are major complications of temporal (giant cell) arteritis?

A
  • sudden onset, potentially permanent blindness; this is why you treat upon suspicion before biposy confirmation
  • aortic aneurysm
  • ischemic stroke
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9
Q

What other condition is often present with temporal (giant cell) arteritis?

A

-polymyalgia rheumatica (arthralgia and myalgia)

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10
Q

What is Takayasu arteritis?

What vessels does it typically effect and what changes occur in the vessel?

A

granulomatous, large vessel vasculitis

Vessels:

-aortic arch at branching points (crainal and upper ext As.)

Changes:

  • smooth tapered stenosis
  • granulomatous changes of aortic arch, multinucleated giant cells
  • fibrosis
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11
Q

What is the epidemiology of Takayasu arteritis?

A
  • more common in females
  • adolescents to mid adults (15-45)
  • more common Asians (Takayasu is Japanese -> Asian)

“young asian women”

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12
Q

What is the clinical presentation of Takayasu arteritis?

A

Constitutional Sx:

  • fever, malaise, weight loss
  • decreased radial/brachial pulses (“pulseless disease”)
  • vision changes; “copper-wiring” retinopathy
  • HTN (renal A.)
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13
Q

What are diagnostic features of Takayasu arteritis?

A
  • smooth stenosis on angiography
  • elevated ESR
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14
Q

What is the treatment and prognosis for Takayasu arteritis?

A
  • corticosteroids
  • typically regresses but aquired permanent changes (vision and neuro deficits) will remain
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15
Q

What is the main differentiating factor between temporal arteritis and Takayasu arteritis?

A

age; takayasu <50 and temporal >50

  • very similar with constitutional s/x, visual changes, and neuro s/x
  • very similar histology
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16
Q

What is polyarteritis nodosa?

What vessels does it typically effect and what changes occur in the vessel?

A

necrotizing systemic medium vessel arteritis

Vessels:

  • muscular, medium arteries suppling most organs
  • ***lungs spared***

Changes:

  • transmural inflammation w/ fibrinoid necrosis (early) -> fibrous aneurysms (“string of pearls”; hence nodosa) (late)
  • multiple, diffuse lesions (hence polyarteritis) found in varying stages
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17
Q

What is the clinical presentation of polyarteritis nodosa?

A

Constitutional Sx:

-fever, malaise, weight loss

Symptoms are manifestaions of organ ischemia** and **variable

  • ulcers and gangrene
  • HTN (renal A.)
  • abdominal pain with melena (mesenteric As.)
  • mononeuritis multiplex (foot drop)
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18
Q

What is the epidemiology of polyarteritis nodosa?

A

-more common in males

-young adults

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19
Q

What are diagnostic features of polyarteritis nodosa?

A
  • multiple microaneurysms “string of pearls” on angiography
  • fibrinoid necrosis on biopsy

-positive hepatitis B

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20
Q

What other condition is often present with polyarteritis nodosa?

A

hepatitis B infection; positive for HBsAg (hepaitits B surface Ag

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21
Q

What is the treatment and prognosis for polyarteritis nodosa?

A
  • corticosteroids
  • cyclophosphamide

**fatal if not treated**

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22
Q

What is Kawasaki disease?

What vessels does it typically effect and what changes occur in the vessel?

A

necrotizing medium vessel vasculitis

Vessels:

-muscular, medium arteries (of significance are coronary As.)

Changes:

  • similar to PAN, less severe
  • mostly insignificant other than in cornary As.
  • transmural inflammation w/ fibrinoid necrosis -> aneurysms
  • thrombosis (following vessel damage)
23
Q

What is the clinical presentation of Kawasaki disease?

A

CRASH and BURN

  • Conjunctivitis
  • Rash (trunk)
  • Adenopathy (cervical)
  • Strawberry” tongue (bright red and bumpy)
  • Hands and feet (rash)
  • burn -> fever

very similiar in appearance to a viral infection

24
Q

What is the epidemiology of Kawasaki disease?

A
  • children (<5)
  • most common in Asians
25
Q

What are diagnostic features of Kawasaki disease?

A
  • fever >5 days + 4/5 CRASH s/x
  • echocardiogram to assess coronary arteries
26
Q

What is the treatment and prognosis for Kawasaki disease?

A
  • aspirin to prevent thrombosis of coronary As. (**aspirin is not normally given to children, esepcially when febrile, so this is unusual**)
  • IVIG
  • self limited though can have lasting cardiac damage
  • increased risk of MI
27
Q

What is thromboangiitis obliterans (Buerger disease)?

What vessels does it typically effect and what changes occur in the vessel?

A

medium vessel vasculitis

Vessels:

-muscular, medium arteries (particularly those of the extremities)

Changes:

  • smooth, tapering occlusion of vessels
  • formation of “corkscrew” collaterals
  • segemental thrombosis
28
Q

What is the clinical presentation of thromboangiitis obliterans (Buerger disease)?

A

Early triad:

  • Raynaud phenomenon
  • superficial thrombophlebitis (migratory)
  • claudication (cramping)

Late:

-ulceration, gangrene, and autoamputation of fingers/toes

29
Q

What is the epidemiology of thromboangiitis obliterans (Buerger disease)?

A

-young men

***only seen in smokers***

30
Q

What are diagnostic features of thromboangiitis obliterans (Buerger disease)?

A
  • segmental, smooth tapering lesions of distal vessels of extremities
  • corkscrew” collaterals
31
Q

What is the treatment and prognosis for thromboangiitis obliterans (Buerger disease)?

A
  • no effective treatment
  • smoking cessation stops sympsoms
  • aquired damage will persist
32
Q

What is granulomatosis with polyangitis (Wegener granulomatosis)?

What vessels does it typically effect and what changes occur in the vessel?

A

granulomatous and necrotizing small vessel vasculitis

Vessels:

-small vessels (of significance are upper resp, lower resp, and kidney)

Changes:

-lesion have central necrosis with peripheral granulomas

33
Q

What is the clinical presentation of granulomatosis with polyangitis (Wegener granulomatosis)?

A

Triad of upper respiratory, lower respiratory, and renal

Upper respiratory:

  • rhinitis/sinusitis
  • ulcerations -> septal perforation
  • saddle nose

Lower respiratory:

  • hemoptysis
  • nodular infiltrates (pneumonia-like s/x)

Renal:

  • rapidly progressive glomerulonephritis
  • hematuria
34
Q

What is the epidemiology of granulomatosis with polyangitis (Wegener granulomatosis)?

A
  • more common in males
  • older adults
35
Q

What are diagnostic features of granulomatosis with polyangitis (Wegener granulomatosis)?

A
  • biopsy shows necrotic, granulomatous vasculitis
  • positive c-ANCA (autoantibody)
  • nodular lesions on CXR
36
Q

What is the treatment and prognosis for granulomatosis with polyangitis (Wegener granulomatosis)?

A

-corticosteroids

(reduce ANCA)

  • cyclophosphamide
  • methotrexate
  • rituximab
  • relapses are common
  • lethal without treatment
37
Q

What is Henoch-Schönlein purpura?

What vessels does it typically effect and what changes occur in the vessel?

A

IgA immune complex mediated small vessel vasculitis

Vessels:

-small vessels

Changes:

-necrosis with IgA deposition

38
Q

What is the clinical presentation of Henoch-Schönlein purpura?

A

Tetrad

  1. palpable purpura; lower extremities and buttocks (100%)
  2. arthritis (75%)
  3. abdominal pain (60%)
  4. renal disease; IgA nephropathy (50%)
39
Q

What is the epidemiology of Henoch-Schönlein purpura?

A

-mostly children (<10)

40
Q

What are diagnostic features of Henoch-Schönlein purpura?

A

Clinical diagnosis with tetrad following URI

-IgA and C3 deposits on biopsy can confirm (biopsy too invasive for lack of severity?)

41
Q

What condition often present in the history of those with Henoch-Schönlein purpura?

A

-URI a few weeks prior to onset of symptoms

(URI stimulates production of IgA)

42
Q

What is the treatment and prognosis for Henoch-Schönlein purpura?

A

Supportive:

  • NSAIDs for pain
  • typically mild and self-limited; can recur

-corticosteroids in severe cases with nephropathy

43
Q

What is Goodpasture syndrome?

What vessels does it typically effect and what changes occur in the vessel?

A

antibody-mediated small vessel vasculitis

Vessels:

-small vessels (glomerular and pulmonary capillaries)

Changes:

  • necrosis
  • linear Ab deposition along basement membrane
44
Q

What is the clinical presentation of Goodpasture syndrome?

A
  • rapidly progressive glomerulonephritis
  • pulmonary hemorrhage -> cough, dyspnea, and hemoptysis
45
Q

What is the epidemiology of Goodpasture syndrome?

A
  • mostly men
  • teens to young adults
46
Q

What are diagnostic features of Goodpasture syndrome?

A

-anti-basement membrane Ab (targets portion of type IV collagen) on biopsy (typically renal)

47
Q

What is the treatment and prognosis for Goodpasture syndrome?

A
  • plasmapheresis (remove antibodies)
  • corticosteroids
  • cyclophosphamide
48
Q

What is eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?

What vessels does it typically effect and what changes occur in the vessel?

A

necrotizing, granulomatous small vessel vasculitis w/ eosinophilia

Vessels:

-small vessels throughout the body

Changes:

  • necrosis and granuloma of vessels
  • extravascular infiltration by eosinophils
49
Q

What is the clinical presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?

A

Primarily affecs lungs and skin

  • asthma
  • palpable purpora
  • GI bleeding
  • glomerulonephritis
  • pericarditis
50
Q

What are diagnostic features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?

A
  • eosinophila
  • necrotizing granulomas
  • pANCA (<50%)
51
Q

What is Behcet syndrome?

What vessels does it typically effect and what changes occur in the vessel?

A

neutrophilic vasculitis

52
Q

What is the clinical presentation of Behcet syndrome?

A

Tirad:

  • oral ulcers
  • genital ulcers
  • uveitis (inflammation of the eye)
  • GI ulcers
  • neuro s/x (similar to MS)
  • arthralgia
53
Q

What is the epidemiology of Behcet syndrome?

A

Turkey, Asia, and the Middle East