Rheumatology: Vasculitis Flashcards Preview

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

Flashcards in Rheumatology: Vasculitis Deck (52)
1

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

inflammation of blood vessels

2

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

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

3

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.

primary; secondary

4

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

the size of the vessels

5

Large-cell vasculitis can either be ____ or ____.

giant cell arteritis, Takayasu's arteritis

6

What BVs are involved in giant cell arteritis?

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

7

What are the s/s of giant cell arteritis?

temporal headache
jaw claudication
scalp tenderness
visual loss

8

What BVs are involved in Takayasu's arteritis?

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

9

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

claudication of upper>lower extremities
CNS events
granulomatous panarteritis

10

Medium-vessel vasculitis can be ____ or ____.

polyarteritis nodosa; Kawasaki's disease

11

What BVs are involved in polyarteritis nodosa?

small and medium sized arteries, especially at vessel bifurcations

12

What are the s/s of polyarteritis nodosa?

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

13

What BVs are involved in Kawasaki disease?

small and medium sized arteries

14

What are the s/s of Kawasaki disease?

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

15

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

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

16

ANCA positive vasculitides can be?

Granulomatosis with polyangiitis (Wegener’s) aka GPA

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Microscopic polyangiitis (MPA)

17

What arteries does GPA affect?

small and medium-sized arteries

18

Where does GPA manifest?

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

19

GPA is usually assoc. with serum _____.

cytoplasmic-ANCA (c-ANCA)

20

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

small arteries and venules

21

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

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

22

Microscopic polyangiitis (MPA) affects which BVs?

arterioles, capillaries, and venules

23

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

pulmonary hemorrhage
glomerulonephritis
palpable purpura
peripheral neuropathy
joint and abdominal pain

24

Serum _____ is common in microscopic polyangiitis (MPA).

perinuclear- ANCA (p-ANCA)

25

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

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

26

Henoch-Schönlein Purpura (HSP) affects which BVs?

arterioles and venules

27

Essential cryoglobulinemic vasculitis affects which BVs?

small vessels, including glomerulocapillaries

28

Henoch-Schönlein Purpura (HSP) presents as?

palpable purpuric skin lesions in lower extremities
arthritis
abdominal pain
hematuria

29

Essential cryoglobulinemic vasculitis presents as?

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

30

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

rheumatoid factor; hepatitis C antibody

31

Cutaneous leukocytoclastic angiitis manifests in which BVs?

arterioles and venules

32

What are the manifestations in cutaneous leukocytoclastic angiitis?

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

33

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

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

34

What are the common lab features for all the vasculides?

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

How are the vasculides diagnosed?

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

36

At what age do the vasculides typically present?

mean = 5th decade

37

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

Takayasu’s and giant cell arteritis

38

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.

Takayasu’s arteritis; giant cell arteritis

39

What are the treatments for the vasculides?

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

40

What is the cause of giant cell arteritis?

disruption of the internal elastic lamina

41

What is the cause of Takayasu’s arteritis?

granulomatous panarteritis

42

What is the cause of polyarteritis nodosa?

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

43

What is the cause of Kawasaki’s disease?

probable infectious vector resulting in cytokine-mediated endothelial damage

44

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

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

45

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

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

46

What is the cause of Microscopic polyangiitis (MPA)?

pauci-immune, necrotizing vasculitis, serum perinuclear-
ANCA (p-ANCA) common

47

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

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

48

What is the cause of essential cryoglobulinemic vasculitis?

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

49

What is the cause of cutaneous leukocytoclastic angiitis?

leukocytoclastic vasculitis

50

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

- 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

What is the general pathology for the small vessels?

- 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

What are the 5 suspected mechanisms of vascular damage?

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