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MSK-Derm by Minnie AH Version > 230: Vasculitis > Flashcards

230: Vasculitis Flashcards

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1
Q

What is the treatment for anti-GBM disease?

A

Plasmapheresis, glucocorticoids, cyclophosphamide

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2
Q

List the immune-complex small vessel vasculitides

A
  • Anti-GBM
  • IgA Vasculitis
  • Cryoglobulinemia
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3
Q

Describe the pathologic findings associated with GPA

A

Granulomatosis with Polyangiitis (GPA)

  • Necrotizign granulomas
  • Few or no immune deposits
  • Associated wtih c-ANCA/PR3+
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4
Q

Describe Polyarteritis Nodosa

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Polyarteritis Nodosa

  • “Typical patient”: Male 40-60 years old
    • But can affect people of any age
  • Arteries affected: Medium-sized muscular arteries, often at branch points
  • Presentation:
    • Cutaneous
      • ​Nodules, purpura, livedo, retucularis, ulcers
    • Renal disease
    • Neuropathy/Myalgia/Weakness​
    • Mesenteric Ischemia
      • ​“Stomach pain after large meals”
    • Orchitis
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5
Q

Describe Cryoglobulinemia

  • “Typical Patient”:
  • Arteries affected:
  • Presentation:
A

Cryoglobulinemia

  • “Typical Patient”: Person with MGUS, B-cell malignancy, chronic Hep C, Hep B, HIV, or autoimmune disease
  • Arteries affected: Small vessels (immune complex mediated)
  • Presentation:
    • Digital ischemia
    • Livedo reticularis and skin necrosis
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6
Q

What pathologic findings are associated with microscopic polyangiitis?

A

Necrotizing arteries

Few or no immune deposits

No granulomatous inflammation (will be present in GPA)

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7
Q

Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?

Age of onset <40 years old

A

Takayasu Arteritis

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8
Q

Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?

Affects large vessels

A

Both

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9
Q

Which vasculitis does this statement apply to?

Frequent URI

A

Granulomatosis with polyangiitis

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10
Q

How is GCA treated?

A
  • High dose corticosteroids
  • Tocilizumab (an anti IL-6 antibody)

Treat immediately (while waiting for biopsy) to prevent vision loss!

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11
Q

Which vasculitis does this statement apply to?

Most patients present with oral and genital ulcers

A

Behcets

Occurs most commonly in patients of Mediterranean, Middle Eastern, and Far Eastern descent

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12
Q

Describe the treatment for granulomatosis with polyangiitis and microscopic polyangiitis

A

Steroids

+ Rituximab or cyclophosphamide if severe

+ Methotrexate if not severe

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13
Q

How is GCA diagnosed?

A
  • Labs
    • Elevated ESR, CRP
    • Thrombocytosis maybe
  • Imaging
    • Termporal artery ultrasound
    • PET
  • Biopsy
    • Temporal artery
    • Should be done urgently in all patients with suspected GCA
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14
Q

Which vasculitis is associated with chronic HepC infection?

A

Cryoglobulinemia type 2

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15
Q

Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?

Constitutional symptoms

A

Both

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16
Q

Describe granulomatosis with polyangiitis

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Granulomatosis with Polyangiitis (GPA)

  • “Typical patient”: Caucasian 40-50 y/o or >70 y/o
  • Arteries affected: Small to medium vessel vasculitis
  • Presentation:
    • Associated wtih c-ANCA/PR3+
    • Upper airway disease
      • ​Saddle nose deformity, sinus nodules
    • Pulmonary disease
      • ​Cavitary lung nodules
    • Glomerulonephritis
      • ​Crescents
    • Arthritis/arthralgia
    • Neuropathy
    • Ocular disease
    • Vascular
      • ​Gangrene, venous thrombosis
    • Skin
      • ​Oral ulcers, purpura, nodules
    • Cardiac
    • GI
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17
Q

What is the treatment for Kawasaki disease?

Why is it important to identify and treat early?

A

Aspirin + IVIG

Prevent heart disease/aneurysm later in life

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18
Q

Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?

Bruits

A

Takayasu Arteritis

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19
Q

Describe Bechets Syndrome

  • “Typical Patient”:
  • Arteries affected:
  • Presentation:
A

Bechets Syndrome

  • “Typical Patient”: 20-30 year old person of Mediterranean, Middle Eastern, or Far Eastern descent
  • Arteries affected: Any! Variable vasculitis
  • Presentation:
    • Oral ulcers
    • Genital ulcers
    • Other cutaneous lesions
    • Ocular involvemnt
    • Arthralgia
    • Neurologic
    • GI
    • Inner ear
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20
Q

Describe the clinical presentation of Eosinophilic Granulomatosis with Polyangiitis (EGPA)

A

3 phases

  • Prodrome
    • Asthma, allegic rhinitis, nasal polyp
  • Eosinophilic phase
    • Tissue and periphery
  • Vasculitic phase
    • Cutaneous
    • Cardiac
    • Neurologic
    • Renal
    • Gastroenteritis
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21
Q

What are the three ANCA-associated vasculitides?

A
  • Microscopic polyangitiis
  • Granulomatosis with polyangiitis
  • Eosinophilic granulomatosis with polyangiitis
22
Q

Which vasculidities affect medium vessels?

A

Polyarteritis Nodosa

Kawasaki Disease

23
Q

Describe giant cell arteritis

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Giant Cell Arteritis

  • “Typical patient”: Elderly Scandinavian woman
    • ​Can also occur in men, but more common in women
  • Arteries affected: Large vessels; cranial or extracranial involvement
  • Presentation:
    • New headache
    • Constitutional symptoms
    • Jaw claudication
    • Partial or complete loss of vision
24
Q

Which vasculitis does this statement apply to?

Presents most commonly in children <5

A

Kawasaki disease

25
Q

What are the pathologic hallmarks of Kawasaki disease?

A
  • Affects coronary arteries
  • Destruction of luminal endothelial cells, elastic lamina, smooth muscle cells
    • -> Arterial walll dilation +/- aneurysm formation
26
Q

What complications are associated with Takayasu arteritis?

A

Aortic regurgitation and acute MI

27
Q

Which arteritis is associated with jaw claudication, new headache, and partial or complete loss of vision?

A

Giant cell arteritis

28
Q

Describe the treatment for Takayasu arteritis

A
  • High-dose corticosteroids
  • Methotrexate or Azathioprine
  • Surgical bypass
    • Angioplasty +/- stenting has poor outcomes
29
Q

Which vasculitis does this statement apply to?

Associated with p-ANCA/MPO+

A

Microscopic polyangiitis

EGPA has p-ANCA/MPO in 40% of cases, but otherwise ANCA(-)

30
Q

Which vasculitis does this statement apply to?

Treat with aspirin and IVIG

A

Kawasaki

31
Q

Describe the pathologic findings associated with polyarteritis nodosa

A
  • Transmural inflammation
  • No giant cells or granulomas
  • Common for different phases of vasculitis to occur within different vessels or one vessel at the same time
32
Q

70 yo WM with chronic cough for past year. Exam shows nasal ulcers and lungs with diffuse crackles.

  • Creat 4.5
  • UA with 20 RBCs +casts
  • c-ANCA +
  • CXR with small scattered pulmonary nodules
  • Lung bx shows vasculitis of small peripheral arteries and arterioles. +granulomatous inflammation adjacent to small arterioles

What is the most likely diagnosis?

A

Granulomatosis with polyangiitis

2nd peak in elderly patients, c-ANCA is the key

33
Q

What is the most common form of systemic vasculitis in adults?

A

Giant cell arteritis

34
Q

What is the appropriate management of a patient for whom you suspect giant cell arteritis?

A

Ugent temporal artery biopsy

Begin treatment with corticosteroids immediately

  • Biopsy to confirm diagnosis, treat immediately to prevent complications
    • Vision loss
35
Q

Describe Takayasu Arteritis

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Takayasu Arteritis

  • “Typical patient”: Asian woman <40 years old
  • Arteries affected: Aorta, subclavian, carotid most common
    • Can affect any artery
  • Presentation:
    • ​Constitutional symptoms
    • Unexplained lightheadedness
    • Extremity pain/claudication
    • Bruits
    • Absent/diminshed pulses or BP difference >10 mmHg
36
Q

What pathologic findings are associated with Eosinophilic Granulomatosis with Polyangiitis (EGPA)?

A

ANCA negative most of the tiem

37
Q

Which vasculitis does this statement apply to?

Strawberry tongue

A

Kawasaki

38
Q

Which vasculitis does this statement apply to?

p-ANCA +

A

Microcsopic polyangiitis

39
Q

Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?

Affects females > males

A

Both

GCA - F>M = 2:1

Takayasu - F>M = 9:1

40
Q

Describe the pathological findings associated with Takayasu arthritis

A
  • Granulomatous panarteritis
  • Intimal hyperplasia
  • Fixed stenosis and occlusions
  • Occasional aneurysm
41
Q

Which vasculitis typically presents in children, and is associated with acute onset fever, strawberry tongue, and rash?

A

Kawasaki Disease

42
Q

Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?

Age of onset >50 years old

A

Giant Cell Arteritis

43
Q

Describe Anti-GBM disease

  • “Typical Patient”:
  • Arteries affected:
  • Presentation:
A

Anti-GBM

  • “Typical Patient”: Older children and adults
  • Arteries affected: Small vessel (Immune-complex mediated)
  • Presentation:
    • Acute renal failure
    • Alveolar hemorrhage
    • No constitutional symptoms
44
Q

Describe Kawasaki Disease

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Kawasaki Disease

  • “Typical patient”: Asian male child <5 years old
    • ​M>F 2:1
  • Arteries affected: Medium-sized muscular arteries, esp. coronary
  • Presentation:
    • Acute onset
    • Fever > 5 days
    • Rash (Basically anything except vesicular)
    • Extremity edema
    • Involvement of lips and mouth
45
Q

Which vasculidities affect large vessels?

A

Takayasu Ateritis

Giant Cell Arteritis

46
Q

What vasculitis is associated with MGUS or B cell malignancy?

A

Cryoglobulinemia Type 1

47
Q

Describe the treatment for polyarteritis nodosa

A
  • Mild
    • Glucocorticoid monotherapy
    • MTX or AZA if resistant
  • Moderate
    • Glucocorticoid + cyclophosphamide
  • Viral-associated secondary PAN
    • Check Hepatitis B
    • Initiate appropriate antiviral therapy
48
Q

Which vasculitis does this statement apply to?

c-ANCA +

A

Granulomatosis with Polyangiitis

49
Q

Describe microscopic polyangiitis

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Microscopic polyangiitis

  • “Typical patient”: Middle age
  • Arteries affected: Small vessels
  • Presentation:
    • Glomerulonephritis
    • Pulmonary capillartis
    • Peripheral neruopathy
    • Skin lesions
    • Weight loss
    • Fever
    • p-ANCA/MPO+
50
Q

Which cytokine is associated with GCA?

A

IL-6

Treat with tocilizumab

51
Q

Describe Eosinophilic Granulomatosis with Polyangiitis

  • “Typical patient”:
  • Arteries affected:
  • Presentation:
A

Eosinophilic Granulomatosis with Polyangiitis

  • “Typical patient”: Male 35-45 with new asthma
    • ​Only slight male predominance
  • Arteries affected: Small vessels
  • Presentation:
    • Often ANCA negative
      • p-ANCA/MPO+ in 40% of cases
    • Eosinophilia/elevated IgE
    • Adult onset asthma
    • 3 phases
      • ​Prodrome, Eosiniphilic, Vasculitic
52
Q

Which antigens are associated with p-ANCA and c-ANCA?

A

p-ANCA = Myeloperoxidase (MPO)

c-ANCA = Proteinase 3 (PR3)

MSK-Derm by Minnie AH Version (47 decks)

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