Rheumatology: Vasculitis

Question 1

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

Answer 1

inflammation of blood vessels

Question 2

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

Answer 2

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

Question 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.

Answer 3

primary; secondary

Question 4

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

Answer 4

the size of the vessels

Question 5

Large-cell vasculitis can either be ____ or ____.

Answer 5

giant cell arteritis, Takayasu’s arteritis

Question 6

What BVs are involved in giant cell arteritis?

Answer 6

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

Question 7

What are the s/s of giant cell arteritis?

Answer 7

temporal headache
jaw claudication
scalp tenderness
visual loss

Question 8

What BVs are involved in Takayasu’s arteritis?

Answer 8

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

Question 9

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

Answer 9

claudication of upper>lower extremities
CNS events
granulomatous panarteritis

Question 10

Medium-vessel vasculitis can be ____ or ____.

Answer 10

polyarteritis nodosa; Kawasaki’s disease

Question 11

What BVs are involved in polyarteritis nodosa?

Answer 11

small and medium sized arteries, especially at vessel bifurcations

Question 12

What are the s/s of polyarteritis nodosa?

Answer 12

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

Question 13

What BVs are involved in Kawasaki disease?

Answer 13

small and medium sized arteries

Question 14

What are the s/s of Kawasaki disease?

Answer 14

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

Question 15

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

Answer 15

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

Question 16

ANCA positive vasculitides can be?

Answer 16

Granulomatosis with polyangiitis (Wegener’s) aka GPA

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Microscopic polyangiitis (MPA)

Question 17

What arteries does GPA affect?

Answer 17

small and medium-sized arteries

Question 18

Where does GPA manifest?

Answer 18

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

Question 19

GPA is usually assoc. with serum _____.

Answer 19

cytoplasmic-ANCA (c-ANCA)

Question 20

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

Answer 20

small arteries and venules

Question 21

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

Answer 21

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

Question 22

Microscopic polyangiitis (MPA) affects which BVs?

Answer 22

arterioles, capillaries, and venules

Question 23

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

Answer 23

pulmonary hemorrhage
glomerulonephritis
palpable purpura
peripheral neuropathy
joint and abdominal pain

Question 24

Serum _____ is common in microscopic polyangiitis (MPA).

Answer 24

perinuclear- ANCA (p-ANCA)

Question 25

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

Answer 25

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

Question 26

Henoch-Schönlein Purpura (HSP) affects which BVs?

Answer 26

arterioles and venules

Question 27

Essential cryoglobulinemic vasculitis affects which BVs?

Answer 27

small vessels, including glomerulocapillaries

Question 28

Henoch-Schönlein Purpura (HSP) presents as?

Answer 28

palpable purpuric skin lesions in lower extremities arthritis abdominal pain hematuria

Question 29

Essential cryoglobulinemic vasculitis presents as?

Answer 29

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

Question 30

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

Answer 30

rheumatoid factor; hepatitis C antibody

Question 31

Cutaneous leukocytoclastic angiitis manifests in which BVs?

Answer 31

arterioles and venules

Question 32

What are the manifestations in cutaneous leukocytoclastic angiitis?

Answer 32

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

Question 33

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

Answer 33

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

Question 34

What are the common lab features for all the vasculides?

Answer 34

``` 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 ```

Question 35

How are the vasculides diagnosed?

Answer 35

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

Question 36

At what age do the vasculides typically present?

Answer 36

mean = 5th decade

Question 37

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

Answer 37

Takayasu’s and giant cell arteritis

Question 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.

Answer 38

Takayasu’s arteritis; giant cell arteritis

Question 39

What are the treatments for the vasculides?

Answer 39

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

Question 40

What is the cause of giant cell arteritis?

Answer 40

disruption of the internal elastic lamina

Question 41

What is the cause of Takayasu’s arteritis?

Answer 41

granulomatous panarteritis

Question 42

What is the cause of polyarteritis nodosa?

Answer 42

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

Question 43

What is the cause of Kawasaki’s disease?

Answer 43

probable infectious vector resulting in cytokine-mediated endothelial damage

Question 44

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

Answer 44

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

Question 45

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

Answer 45

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

Question 46

What is the cause of Microscopic polyangiitis (MPA)?

Answer 46

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

Question 47

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

Answer 47

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

Question 48

What is the cause of essential cryoglobulinemic vasculitis?

Answer 48

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

Question 49

What is the cause of cutaneous leukocytoclastic angiitis?

Answer 49

leukocytoclastic vasculitis

Question 50

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

Answer 50

- 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

Question 51

What is the general pathology for the small vessels?

Answer 51

- 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

Question 52

What are the 5 suspected mechanisms of vascular damage?

Answer 52

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