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Vasculitides Flashcards

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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?

Study These Flashcards
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?

Study These Flashcards
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?

Study These Flashcards
A
  • children (<5)
  • most common in Asians
25
What are **diagnostic** features of **Kawasaki disease**?
- **fever \>5** days + **4/5 CRASH** s/x - **echocardiogram** to assess **coronary arteries**
26
What is the **treatment** and **prognosis** for **Kawasaki disease**?
- **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
What is **thromboangiitis obliterans** (**Buerger disease**)? What vessels does it typically effect and what changes occur in the vessel?
**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
What is the clinical presentation of **thromboangiitis obliterans** (**Buerger disease**)?
Early **triad**: - **Raynaud phenomenon** - **superficial thrombophlebitis** (migratory) - claudication (cramping) Late: -**ulceration, gangrene, and autoamputation** of **fingers/toes**
29
What is the epidemiology of **thromboangiitis obliterans** (**Buerger disease**)?
-**young men** **\*\*\*only seen in smokers\*\*\***
30
What are diagnostic features of **thromboangiitis obliterans** (**Buerger disease**)?
- **segmental, smooth tapering** lesions of **distal vessels** of extremities - "**corkscrew**" collaterals
31
What is the **treatment and prognosis** for **thromboangiitis obliterans** (**Buerger disease**)?
- no effective treatment - **smoking cessation** stops sympsoms - aquired damage will persist
32
What is **granulomatosis with polyangitis** (Wegener granulomatosis)? What vessels does it typically effect and what changes occur in the vessel?
**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
What is the **clinical presentation** of **granulomatosis with polyangitis** (Wegener granulomatosis)?
**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
What is the **epidemiology** of **granulomatosis with polyangitis** (Wegener granulomatosis)?
- more common in **males** - **older adults**
35
What are **diagnostic** features of **granulomatosis with polyangitis** (Wegener granulomatosis)?
- **biopsy** shows **necrotic, granulomatous vasculitis** - positive **c-ANCA** (autoantibody) - nodular lesions on CXR
36
What is the **treatment and prognosis** for **granulomatosis with polyangitis** (Wegener granulomatosis)?
-**corticosteroids** (reduce ANCA) - **cyclophosphamide** - **methotrexate** - **rituximab** - **relapses are common** - lethal without treatment
37
What is **Henoch-Schönlein purpura**? What vessels does it typically effect and what changes occur in the vessel?
**IgA immune complex mediated small** vessel vasculitis Vessels: -small vessels Changes: -**necrosis** with I**gA deposition**
38
What is the **clinical presentation** of **Henoch-Schönlein purpura**?
Tetrad 1. **palpable purpura**; **lower extremities and buttocks** (100%) 2. arthritis (75%) 3. abdominal pain (60%) 4. renal disease; **IgA nephropathy** (50%)
39
What is the **epidemiology** of **Henoch-Schönlein purpura**?
-mostly **children** (\<10)
40
What are **diagnostic** features of **Henoch-Schönlein purpura**?
Clinical diagnosis with **tetrad following URI** -**IgA and C3 deposits** on biopsy can confirm (biopsy too invasive for lack of severity?)
41
What condition often present in the history of those with **Henoch-Schönlein purpura**?
-**URI** a few **weeks prior to onset of symptoms** (URI **stimulates production of IgA**)
42
What is the treatment and prognosis for **Henoch-Schönlein purpura**?
**Supportive:** - NSAIDs for pain - typically **mild and self-limited; can recur** **-corticosteroids** in **severe cases with nephropathy**
43
What is **Goodpasture syndrome**? What vessels does it typically effect and what changes occur in the vessel?
**antibody-mediated small** vessel vasculitis Vessels: -small vessels (**glomerular and pulmonary capillaries**) Changes: - **necrosis** - **linear Ab deposition** along **basement membrane**
44
What is the **clinical presentation** of **Goodpasture syndrome**?
- **rapidly progressive glomerulonephritis** - **pulmonary hemorrhage** -\> cough, dyspnea, and **hemoptysis**
45
What is the **epidemiology** of **Goodpasture syndrome**?
- mostly **men** - **teens to young adults**
46
What are **diagnostic** features of **Goodpasture syndrome**?
-**anti-basement membrane Ab** (targets portion of type IV collagen) on **biopsy** (typically renal)
47
What is the **treatment and prognosis** for **Goodpasture syndrome**?
- **plasmapheresis** (remove antibodies) - **corticosteroids** - **cyclophosphamide**
48
What is **eosinophilic granulomatosis with polyangiitis** (Churg-Strauss syndrome)? What vessels does it typically effect and what changes occur in the vessel?
**necrotizing, granulomatous small** vessel vasculitis w/ **eosinophilia** Vessels: -small vessels throughout the body Changes: - **necrosis and granuloma** of vessels - **extravascular infiltration by eosinophils**
49
What is the **clinical presentation** of **eosinophilic granulomatosis with polyangiitis** (Churg-Strauss syndrome)?
Primarily affecs lungs and skin - **asthma** - **palpable purpora** - GI bleeding - glomerulonephritis - pericarditis
50
What are diagnostic features of **eosinophilic granulomatosis with polyangiitis** (Churg-Strauss syndrome)?
- **eosinophila** - **necrotizing granulomas** - pANCA (\<50%)
51
What is **Behcet syndrome**? What vessels does it typically effect and what changes occur in the vessel?
neutrophilic vasculitis
52
What is the **clinical presentation** of **Behcet syndrome**?
**Tirad:** - **oral ulcers** - **genital ulcers** - **uveitis** (inflammation of the eye) - GI ulcers - neuro s/x (similar to MS) - arthralgia
53
What is the **epidemiology** of **Behcet syndrome**?
**Turkey, Asia,** and the **Middle East**

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