vasculitis Flashcards

1
Q

Describe Giant Cell Arteritis

A

Most common form of vasculitis among older individuals in the US and Europe

T cell-mediated response against vessel wall antigens that drive proinflammatory cytokine production

Typically occurring in the temporal, vertebral or ophthalmic arteries

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2
Q

Discern Giant cell arteritis from Takayasu arteritis

A

Giant cell arteritis occurs in people > 50 y/o

Pulseless Disease typically occurs in people < 50y/o

  • can cause syncope and angina due to the disparity in blood pressure
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3
Q

Describe the morphology seen on biopsy of Giant cell arteritis and Pulseless Disease

A

medial granulomatous inflammation centered on the internal elastic lamina that produces elastic lamina fragmentation

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4
Q

Another name for Kawasaki Disease

A

Mucocutaneous Lymph Node Syndrome

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5
Q

Clinical Presentation of Kawasaki disease

A

Conjunctivitis

Rash

Adenopathy of cervical lymph nodes

Strawberry tongue

Hand/ feet edema

5+ days of fever

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6
Q

what is polyarteritis nodosa

A

systemic vasculitis of small-medium sized arteries, typically involving the renal and visceral vessels and sparing the pulmonary circulation

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7
Q

Risk of Kawasaki Disease

A

predilection for coronary aneurysms

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8
Q

clinical presentation of PAN

A

rapidly accelerating HTN in adults 45-65 y/o

fever/ malaise

infarction causing: renal, abd or neurologic impairment

rash/ ulcerations/ nodules

PAN RAN a fever

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9
Q

Diagnostics of PAN

A

30% of patients have chronic HepB with antigen complex deposition

negative ANCA

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10
Q

morphology of PAN

A

vessels at various stages of acute and chronic segmental transmural necrotizing inflammation of small-medium sized arteries

acute stage fibrinoid necrosis

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11
Q

how is PAN treated

A

cyclophosphamide

glucocorticoids

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12
Q

Another name for Thromboangitis Obliterans

A

Buerger Disease

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13
Q

Describe Buerger Disease

A

segmental thrombosing acute and chronic inflammation of the small-medium arteries with occasional secondary extension into the veins and nerves of the extremities

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14
Q

Clinical presentation of Thromboangitis obliterans

A

Men >35y/o

smoker

intermittent claudication

necrosis of digits/ amputation

Raynaud phenomenon

chronic extremity ulcerations/ gangrene

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15
Q

morphology of Buerger Disease

A

nonatherosclerotic segmental occlusion of arteries

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16
Q

Another name or Granulomatosis with polyangiitis

A

Wegner Disease

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17
Q

what is Wegner Disease

A

T cell-mediated hypersensitivity in response to innocuous microbial or other environmental agents

18
Q

Clinical Presentation of Granulomatosis with polyangiitis

A

Looks like PAN wit respiratory involvement

fever/ malaise

rash/ nodules

adenopathy of cervical lymph nodes

neuro/renal/abd and respiratory involvement

Necrotizing granulomas within the respiratory tract

chronic otitis media and mastoiditis

rapidly progressive glomerulonephritis

19
Q

DIagnostics of Wegner Disease

A

PRE-ANCA/cANCA

multiple bilateral cavitating nodular lesions on CXR

20
Q

Morphology of Granulomatosis with polyangiitis

A

granulomas with geographic patterns of central necrosis and accompanying vasculitis

-surrounded by a zone of proliferating fibroblasts associated wit giant cels ad leukocyte infiltrate

During late stages, there is extensive necrotizing granulomatous involvement of the parenchyma, leading to alveolar hemorrhage

21
Q

Another name or Churg-Strauss

A

Eosinophilic granulomatosis with polyangiitis

22
Q

what is Eosinophilic granulomatosis with polyangiitis

A

small vessel necrotizing vasculitis with hyperresponsiveness to allergic stimuli and leukotriene receptor antagonists in asthmatics

23
Q

clinical presentation of Eosinophilic granulomatosis with polyangiitis

A

severe allergic asthma/ sinusitis

palpable purpura

peripheral neuropathy

cardiomyopathy with eosinophilic infiltrates

24
Q

diagnostics of Churg-Strauss

A

MPO-ANCA/ p-ANCA

eosinophilia

increased IgE

25
Q

Morphology of Churg Straus

A

vessels with various stages of inflammation, eosinophils, necrotizing granulomas, and fibrinoid necrosis

similar to PAN but with eosinophils and granulomas

26
Q

what is microscopic polyangiitis

A

necrotizing vasculitis that generally affects capillaries, small arterioles, and venules

27
Q

other names for microscopic polyangiitis

A

hypersensitivity vasulitis

leukocytoclastic vasculitis

28
Q

clinical presentation of microscopic polyangiitis

A

HTN

necrotizing glomerulonephritis

palpable purpura

pulmonary capillaritis

hemoptysis

hematuria

proteinuria

ad pain

29
Q

Diagnostics of microscopic polyangiitis

A

MPO-ANCA/p-ANCA

30
Q

Morphology of microscopic polyangiitis

A

lesions appear to be the same age, but without granulomatous inflammation.

segmental fibrinoid necrosis o the media and focal transmural necrotizing lesions

similar to PAN, but spares medium and large arteries

31
Q

Most common vasculitis in children

A

IgA Vasculitis

32
Q

another name for IgA Vasculitis

A

Henoch-Schonlein Purpura

33
Q

Clinical presentation of IgA vasculitis

A

child with palpable purpura in lower limbs

arthralgias

colicky abd pain, vomiting, melena

hematuria

spontaneous recovery in 4 months

Associated with Buerger disease

34
Q

how do you treat IgA Vasculitis

A

NSAIDS

Glucocorticoids

IV hydration

Supportive care

35
Q

what is cryoglobulinemic vasculitis

A

vasculitis due to mixed IgG and IgA deposition

36
Q

Clinical symptoms of cryoglobulinemic vasculitis

A

fatigue

arthralgias

palpable purpura

glomerulonephritis

peripheral neuropathy

37
Q

associations of cryoglobulinemic vasculitis

A

viral infection

Hep C

38
Q

treatment for cryoglobulinemic vasculitis

A

glucocorticoids

cyclophosphamide

Hep C treatment (IFN-alpha/ ribavirin)

39
Q

clinical presentation of Bechet Disease

A

Middle eastern/ Japanese

mucosal ulcers

uveitis

erythema nodosum

40
Q

Diagnostics of Bechet disease

A

associated with HLA-B51

cross-reacts wit TH17 microbes

41
Q

treatment for Bechet disease

A

glucocorticoids

42
Q

Compare/ Contrast Primary and Secondary Raynaud phenomenon

A

Primary:

  • presents as exaggerated central and local vasomotor responses to cold or emotion
  • typically occurring in young women
  • s_ymmetrically affects the extremities_ with no structural changes to the vessel wall
  • Chronically appears as atrophy, ulcers and ischemic gangrene

Secondary

  • presents as vascular insufficiency due to arterial disease
  • typically occurs with SLE, scleroderma, Buerger Disease, and atherosclerosis with asymmetric involvement of the extremities
  • worsens over time