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MHD 1 > L47 Vasculitis > Flashcards

L47 Vasculitis Flashcards

1
Q

What is vaculitis?

A

Inflammation involving blood vessels, typically arteries

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2
Q

When should vasculitis be on the differential?

A
  1. Presence of constitutional symptoms

2. Symptoms suggestive of specific organ involvement

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3
Q

Generally, what happens in vasculitis?

A

Inflammation in the vessels leads to thickening, infiltration of immune cells, giant cell formation, thrombosis formation, and narrowing of the lumen. This leads to occlusion and ischemia.

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4
Q

What types of vasculitis affect large vessels?

A
  1. Giant cell arteritis

2. Takayasu arteritis

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5
Q

What types of vasculitis affect medium vessels?

A
  1. Polyarteritis nodosa

2. Kawasaki disease

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6
Q

What types of vasculitis affect small vessels - ANCA associated?

A
  1. Granulomatosis with polyangitis
  2. Eosinophilic granulomatosis with polyangitis
  3. Microscopic polyangitis
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7
Q

What types of vasculitis affect small vessels - immune complex associated?

A
  1. IgA vasculitis

2. Cryoglobulinemic vasculitis

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8
Q

What is another name for giant cell arteritis?

A

Temporal arteritis

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9
Q

Discuss the epidemiology of giant cell arteritis.

A
  1. Typically affects people >50 (mean: 70-75 years)
  2. 2/3 female
  3. Most common primary vasculitis
  4. Highest incidence in Scandinavian populations
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10
Q

Of the four general presentations, what is the common presentation of giant cell arteritis?

A

Polymyalgia Rheumatica (PMR)

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11
Q

What are the symptoms of PMR?

A
  1. Significant stiffness in the proximal joints (shoulders, hips)
  2. Pain in the proximal joints and muscles
  3. Prolonged morning stiffness
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12
Q

What are the 4 presentations of giant cell arteritis?

A
  1. Cranial arteritis
  2. Polymyalgia rheumatica
  3. Nonspecific inflammatory disease (constitutional symptoms)
  4. Large vessel vasculitis
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13
Q

What are the symptoms seen in cranial arteritis?

A

Headache, visual loss (up to 20%), scalp/temporal tenderness, claudication of jaw and tongue muscles

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14
Q

What are the nonspecific inflammatory disease symptoms?

A

Fever, night sweats, malaise, weight loss, anemia, leukocytosis, thrombocytosis (ESR and CRP usually high)

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15
Q

What are the large vessel vasculitis symptoms?

A

Asymptomatic, signs of ischemia may be absent

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16
Q

Where does giant cell arteritis occur?

A

Aorta, femoral, iliac, subclavian, temporal

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17
Q

What causes giant cell arteritis?

A

Cause is unknown, but it involves chronic, usually granulomatous inflammation of large and medium arteries. It involves the innate and adapative immune systems, and TNF-alpha, IL-6, and IFN-gamma are involved.

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18
Q

Describe the morphology of giant cell arteritis.

A
  1. Patchy/skip lesions
  2. Lumen occlusion, sometimes thromboses
  3. Granulomatous inflammation
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19
Q

Describe the granulomatous inflammation seen in giant cell arteritis.

A
  1. Found within the inner media centered on the internal elastic membrane
  2. Infiltrate of lymphocytes, macrophages, and giant cells
  3. Fragmentation and destruction of internal elastic lamina
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20
Q

What is a granuloma?

A

Collection of localized inflammatory cells

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21
Q

What is a giant cell?

A

Union of multiple macrophages

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22
Q

How is giant cell arteritis diagnosed?

A
  1. History and physical
  2. Biopsy temporal arteries
  3. No specific markers or serolies
  4. Elevated ESR and CRP indicate inflammation
  5. Angiogram if large vessels involved
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23
Q

Temporal artery biopsy carries risk of ___.

A

False negatives

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24
Q

Giant cell arteritis can cause ___.

A

Aneurysms

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25
Q

What is another name for Takayasu arteritis?

A

Pulseless disease

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26
Q

What vessels are affected in Takayasu arteritis?

A

Large and medium vessels

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27
Q

Discuss the epidemiology of Takayasu arteritis.

A
  1. Patients are younger than 50 years of age

2. Very rare

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28
Q

What vessels are affected in Takayasu arteritis?

A

Classically affects the aortic arch and arch vessels, including the subclavian artery; 1/3 also involve remainder of aorta and its branches, pulmonary, renal, and coronary arteries

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29
Q

What are the symptoms of Takayasu arteritis?

A

Pulselessness and ischemia

30
Q

How is Takayasu arteritis diagnosed?

A
  1. Clinical
  2. Elevated ESR/CRP (not always elevated)
  3. CT or MR angiogram
31
Q

Discuss the pathogenesis of Takayasu arteritis.

A

It is poorly understood; it involves the major cytokines (IL-6, IL-8, IL-18)

32
Q

Describe the histology and morphology of Takayasu arteritis.

A
  1. Granuloma formation
  2. Transmural scarring and vessel thickening
  3. Adventitial and transmural mononuclear infiltrates
  4. Giant cells
  5. Patchy medial necrosis
  6. Irregular thickening of the vessel wall, intimal hyperplasia, adventitial fibrosis
33
Q

___ is more common in Takayasu arteritis than Giant Cell arteritis.

A

Collateral vessel formation

34
Q

Discuss the epidemiology of polyarteritis nodosa.

A
  1. Very rare

2. High incidence in Hepatitis B

35
Q

Discuss the pathogenesis of polyarteritis nodosa.

A

Little known

36
Q

What arteries are affected in polyarteritis nodosa?

A

Medium and sometimes small arteris

37
Q

What are the symptoms of polyarteritis nodosa?

A
  1. Thrombosis, aneurysms, ischemia
  2. Constitutional symptoms
  3. Hypertension (kidney involvement)
  4. Bloody stools from mesenteric ischemia (GI involvement)
  5. Muscle pain
  6. Mononeuritis multiplex
  7. Orchitis
  8. Ulcers/infarcts/livedo reticularis (skin)
38
Q

Describe the histology and morphology of polyarteritis nodosa.

A
  1. Focal, segmental skip lesions
  2. Transmural necrotizing inflammation
  3. Neutrophils and mononuclear cells
  4. Fibrinoid necrosis
  5. Luminal thrombosis
39
Q

How is polyarteritis nodosa diagnosed?

A
  1. ESR and CRP
  2. Muscle/nerve biopsy
  3. Abdominal angiography if GI symptoms
  4. Test for Hep B
40
Q

Discuss the epidemiology of Kawasaki disease.

A
  1. Seen in infants and young children (<5 years)

2. Rare, but the most common vasculitis in children

41
Q

What vessels are affected in Kawasaki disease?

A

Medium vessels, especially coronary arteries

42
Q

If not treated appropriately, what happens in Kawasaki disease?

A

1/5 children develop coronary artery aneurysms

43
Q

How is Kawasaki disease diagnosed?

A
  1. Diagnostic criteria
  2. Lab testing for inflammation
  3. Echocardiogram to evaluate the heart/coronary arteries
44
Q

What are the diagnostic criteria for Kawasaki disease?

A
  1. Persistant fever (>5 days) +
  2. Nonpurulent bilateral conjunctivitis
  3. Oral mucosal involvement (erythematous pharynx, red/fissured lips, strawberry tongue)
  4. Soft tissue abnormalities of hands and feaet
  5. Polymorphous nonvesicular rash
  6. Cervical adenopathy
45
Q

Discuss the pathogenesis of Kawasaki disease.

A
  1. Microbial agent (mostly viral) triggers

2. Possible delayed type hypersensitivity response directed against cross-reactive or newly uncovered vascular Ag

46
Q

Discuss the histology/morphology of Kawasaki disease.

A
  1. Transmural inflammatory infiltrate
  2. Destruction of elastin and collagen fibers
  3. Fibrinoid necrosis (less severe than PAN)
47
Q

What is ANCA?

A

Anti-neutrophil cytoplasmic antibody - directed against components of neutrophil granules

48
Q

What are the two types of ANCA?

A
  1. Anti-myeloperoxidase (anti-MPO, p-ANCA)

2. Anti-proteinase 3 (anti-PR3, c-ANCA)

49
Q

What is myeloperoxidase?

A

Lysosomal granule involved in oxygen free radical generation

50
Q

What is proteinase 3?

A

Serine protease in the azurophilic granule that shares homology with several microbial peptides

51
Q

What diseases involve p-ANCA?

A
  1. MPA

2. EGPA

52
Q

What diseases involve c-ANCA?

A
  1. Granulomatosis with polyangitis (aka Wegener granulomatosis)
53
Q

How does ANCA work?

A
  1. ANCA formation induced by drug or cross reactive microbial antigen
  2. During subsequent infection or stimulus, inflammatory cytokines like TNF increase neutrophil surface expression or release of PR3 and MPO.
  3. ANCAs bind these cells, further activating neutrophils
  4. ANCA activated neutrophils release granule contents and reactive oxygen species leading to vascular damage.
54
Q

Describe the difference between c-ANCA and p-ANCA on immunofluorescence.

A

c-ANCA: diffuse granular centrally accentuated staining

p-ANCA: ribbon-like perinuclear staining

55
Q

Discuss the presentation of ANCA vasculitis in general.

A
  1. Affects small vessels
  2. Classic triad of inflammatory manifestation: lungs, kidneys, skin
  3. ANCA usually positive
56
Q

Discuss the pathogenesis of Granulomatosis with Polyangitis (GPA).

A
  1. Cell-mediated hypersensitivity response to an environmental trigger
  2. PR3 antibodies present in 95%
  3. T cell activation
57
Q

What upper airway disease is seen in GPA?

A

Recurrent sinusitis, recurrent nasopharyngeal ulcers, septal perforations, saddle nose deformities

58
Q

What lower respiratory disease is seen in GPA?

59
Q

What kidney disease is seen in GPA?

A

Glomerulonephritis, may lead to end stage renal disease, proteinuria, hematuria

60
Q

What other presentations are seen in GPA?

A

Arthritis, mononeuritis multiplex, conjunctivitis, uveitis, scleritis, palpable purpura, ulcers

61
Q

What is mononeuritis multiplex?

A

Loss of sensory and motor function due to inflammation of involved nerves

62
Q

What is palpable purpura?

A

Non-itchy, palpable rash on the dorsal aspects of the feet and ankles. It is non-blanching, dark, and erythematous.

63
Q

Discuss the respiratory morphology of GPA.

A
  1. Granulomatous sinusitis
  2. Necrotizing granulomatous vasculitis in the lungs
  3. Vascular destruction leads to hemorrhage and hemoptysis
64
Q

Discuss the skin morphology of GPA.

A
  1. Leukocytoclastic vasculitis - neutrophilic infiltrates surrounding and disrupting small vessels, fibrin desposits and nuclear debris, extravasated red blood cells in the adjecent dermis
65
Q

Discuss the renal morphology of GPA.

A
  1. Glomerulonephritis - glomerular necrosis, thrombosis of isolated glomerular capillary loops
  2. Crescentic glomerulonephritis in advanced disease - diffuse necrosis, parietal cell proliferation forming epithelial crescents
  3. Pauci-immune on immunofluorescence - lacking deposits
66
Q

Eosinophilic granulomatosis with polyangitis (EGPA) was formerly known as ___.

A

Churg Strauss Syndrome

67
Q

What are the three phases of EGPA?

A
  1. Atopic phase, allergic rhinitis, and asthma
  2. Eosinophilia
  3. Systemic vasculitis
68
Q

Discuss the presentation of EGPA.

A
  1. Pulmonary infiltrates
  2. Mononeuritis multiplex
  3. Ocular inflammation
  4. Glomerulonephritis
  5. Palpable purpura
  6. Asthma
  7. Cardiomyopathy
  8. Cronary arteritis
  9. Eosinophilic gastroenteritis
69
Q

MPA and GPA are similar - how are they different?

A

MPA is less likely to involve upper respiratory tract

70
Q

What are the symptoms of MPA?

A

Glomerulonephritis, alveolar hemorrhage, polyneuropathy, palpable purpura

71
Q

Describe the morphology of MPA.

A
  1. Segmental fibrinoid necrosis of media
  2. Focal transmural necrotizing lesions
  3. Granulomatous inflammation absent
  4. Similar to PAN, but spares medium/large vessels
  5. Leukocytoclastic vasculitis

MHD 1 (58 decks)

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