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Unit 4: Blood and Lymph > Rheumatology: Vasculitis > Flashcards

Rheumatology: Vasculitis Flashcards

1
Q

The vasculitides are a heterogeneous group of clinical disorders characterized by ______.

A

inflammation of blood vessels

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2
Q

Vasculitis classification can be based on ____, ____, and ____.

A

the size of the vessel involved
type of pathologic change in the vessel wall
the clinical presentation

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3
Q

Vasculitides can be _____, occurring separately from any known underlying disease, or _____ to infectious disorders such as hepatitis B or C and endocarditis, drug hypersensitivity, connective tissue diseases (RA, SLE, Sjögren’s), cryoglobulins, and malignancies.

A

primary; secondary

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4
Q

The Chapel Hill Classification of vasculitis is based largely on _____ involved.

A

the size of the vessels

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5
Q

Large-cell vasculitis can either be ____ or ____.

A

giant cell arteritis, Takayasu’s arteritis

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6
Q

What BVs are involved in giant cell arteritis?

A

temporal arteries, vessels originating from the aortic arch, other arteries (less common)

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7
Q

What are the s/s of giant cell arteritis?

A

temporal headache
jaw claudication
scalp tenderness
visual loss

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8
Q

What BVs are involved in Takayasu’s arteritis?

A

aortic arch and its branches (any part of the aorta)

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9
Q

What are the s/s of Takayasu’s arteritis?

A

claudication of upper>lower extremities
CNS events
granulomatous panarteritis

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10
Q

Medium-vessel vasculitis can be ____ or ____.

A

polyarteritis nodosa; Kawasaki’s disease

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11
Q

What BVs are involved in polyarteritis nodosa?

A

small and medium sized arteries, especially at vessel bifurcations

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12
Q

What are the s/s of polyarteritis nodosa?

A

any organ can be involved, esp skin, joints, peripheral nerves, gut, and kidney

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13
Q

What BVs are involved in Kawasaki disease?

A

small and medium sized arteries

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14
Q

What are the s/s of Kawasaki disease?

A 
fever
prominent mucocutaneous changes
cervical lymphadenopathy
polymorphous rash
erythema and edema of hands and feet
desquamation
myocarditis
coronary vasculitis
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15
Q

The 2 broad categories of small vessel vasculitis are ____ and ____.

A

antineutrophil cytoplasmic antibody (ANCA) positive vasculides and antineutrophil cytoplasmic antibody (ANCA) negative vasculitides

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16
Q

ANCA positive vasculitides can be?

A

Granulomatosis with polyangiitis (Wegener’s) aka GPA

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Microscopic polyangiitis (MPA)

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17
Q

What arteries does GPA affect?

A

small and medium-sized arteries

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18
Q

Where does GPA manifest?

A

upper respiratory tract (sinuses)
lungs
kidneys
may affect other organs

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19
Q

GPA is usually assoc. with serum _____.

A

cytoplasmic-ANCA (c-ANCA)

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20
Q

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) affects ____.

A

small arteries and venules

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21
Q

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) manifests where?

A 
multiorgan involvement:
lungs
skin
peripheral nerves 
gut
heart
renal (rare)
22
Q

Microscopic polyangiitis (MPA) affects which BVs?

A

arterioles, capillaries, and venules

23
Q

What are the s/s of microscopic polyangiitis (MPA)?

A 
pulmonary hemorrhage
glomerulonephritis
palpable purpura
peripheral neuropathy
joint and abdominal pain
24
Q

Serum _____ is common in microscopic polyangiitis (MPA).

A

perinuclear- ANCA (p-ANCA)

25
Q

Antineutrophil cytoplasmic antibody (ANCA) negative vasculitides can be either ____, ____, or ____.

A

Henoch-Schönlein Purpura (HSP), Essential cryoglobulinemic vasculitis, or Cutaneous leukocytoclastic angiitis

26
Q

Henoch-Schönlein Purpura (HSP) affects which BVs?

A

arterioles and venules

27
Q

Essential cryoglobulinemic vasculitis affects which BVs?

A

small vessels, including glomerulocapillaries

28
Q

Henoch-Schönlein Purpura (HSP) presents as?

A

palpable purpuric skin lesions in lower extremities
arthritis
abdominal pain
hematuria

29
Q

Essential cryoglobulinemic vasculitis presents as?

A 
purpura
arthralgias
weakness
peripheral neuropathy
Raynaud’s phenomenon
glomerulonephritis
pulmonary hemorrhage
30
Q

Often pts are ____ and _____ positive in essential cryoglobulinemic vasculitis.

A

rheumatoid factor; hepatitis C antibody

31
Q

Cutaneous leukocytoclastic angiitis manifests in which BVs?

A

arterioles and venules

32
Q

What are the manifestations in cutaneous leukocytoclastic angiitis?

A

palpable purpuric skin lesions
arthralgias
systemic symptoms may be present, usually secondary
to an immune response

33
Q

What are the common clinical s/s for all the vasculides?

A 
skin lesions
constitutional symptoms (fever, anorexia, weight loss, weakness, fatigue) 
musculoskeletal symptoms (arthralgias, arthritis, myalgias, peripheral neuropathy)
34
Q

What are the common lab features for all the vasculides?

A 
anemia of inflammatory disease
thrombocytosis
low albumin
elevated sedimentation rate and C-reactive protein
polyclonal gammopathy
possibly elevated liver enzyme tests
low complement levels
low cryoglobulin
35
Q

How are the vasculides diagnosed?

A

hx and phys exam
detection of serum Abs (RF, ANA, ANCA)
biopsy
angiogram

36
Q

At what age do the vasculides typically present?

A

mean = 5th decade

37
Q

The vasculides are as common in men as they are in women, except for in ____ and ____, in which women have a higher incidence.

A

Takayasu’s and giant cell arteritis

38
Q

Although these disorders can occur in any population, there is a higher incidence of _____ in Japanese and Asian populations, and _____ in populations of Northern European background.

A

Takayasu’s arteritis; giant cell arteritis

39
Q

What are the treatments for the vasculides?

A

treat or remove the inciting agent or antigen; glucocorticoids, cytotoxic drugs, plasmapheresis, Rituximab

40
Q

What is the cause of giant cell arteritis?

A

disruption of the internal elastic lamina

41
Q

What is the cause of Takayasu’s arteritis?

A

granulomatous panarteritis

42
Q

What is the cause of polyarteritis nodosa?

A

focal but panmural necrotizing arteritis with a predilection for involvement at the vessel bifurcation

43
Q

What is the cause of Kawasaki’s disease?

A

probable infectious vector resulting in cytokine-mediated endothelial damage

44
Q

What is the cause of Granulomatosis with polyangiitis (Wegener’s) (GPA)?

A

pauci-immune, necrotizing, granulomatous arteritis usually associated with serum cytoplasmic-ANCA (c-ANCA)

45
Q

What is the cause of Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)?

A

necrotizing extravascular granulomas and vasculitis of small arteries and venules; eosinophils present in early stage

46
Q

What is the cause of Microscopic polyangiitis (MPA)?

A

pauci-immune, necrotizing vasculitis, serum perinuclear-

ANCA (p-ANCA) common

47
Q

What is the cause of Henoch-Schönlein Purpura (HSP)?

A

leukocytoclastic (neutrophilic perivascular/transmural infiltrate) or necrotizing vasculitis often with IgA deposition

48
Q

What is the cause of essential cryoglobulinemic vasculitis?

A

cryoglobulins deposited on the vascular wall stimulate complement activation and a cellular inflammatory response

49
Q

What is the cause of cutaneous leukocytoclastic angiitis?

A

leukocytoclastic vasculitis

50
Q

What is the general pathology for the large and medium vessels?

A
  • panarteritis with infiltration of lymphocytes, monocytes, histiocytes, eosinophils, and PMNs through the vessel wall
  • granuloma or giant cell formation in some disorders
  • disruption of the elastic lamina
  • thickening results in narrowing or obliteration of the vessel lumen
51
Q

What is the general pathology for the small vessels?

A
  • fibrinoid necrosis of the vessel wall with a transmural
    inflammatory reaction
  • “leukocytoclastic” vasculitis – a pathologic
    term used to describe necrotizing vasculitis with PMN-derived nuclear debris that often accompanies the neutrophilic perivascular infiltrate of the vessel wall
  • immunoglobulin (IgM, IgA in HSP) and complement (C3) vascular deposition
52
Q

What are the 5 suspected mechanisms of vascular damage?

A
  1. immune complexes
  2. Antineutrophil Cytoplasmic Antibodies (ANCA)
  3. Antiendothelial antibodies
  4. T Cell Dependent Mediated Endothelial Cell Injury
  5. Infection of Endothelial Cells

Unit 4: Blood and Lymph (25 decks)

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