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SALMAN HARRISON'S > 356 - The Vasculitis Syndrome > Flashcards

356 - The Vasculitis Syndrome Flashcards

1
Q

ANCA is a family of autoantibodies, auto immunogenic that act against proteins in the ___ of ___ and ___. They tend to be high in ___ or ___, and low in ___.

A 
granules
neutrophils
monocytes
Wegner's
microscopic polyangiitis
Churg Strauss
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2
Q

c-ANCA=___, common in ___

A

cytoplasmic Anti-neutrophil cytoplasmic antibody

Wegner’s

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3
Q

p-ANCA=___, is more common in ___. Nevertheless only Ab for ___ can be found in ___, ___, ___.

A 
perinuclear Anti-neutrophil cytoplasmic antibody
IBD
MPO
microscopic polyangiitis
Churg Strauss
Wegner's
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4
Q

Clinical symptoms that suggest vasculitis include: (5). Remember we must first overrule other disease with similar presentation (mostly infectious)

A 
palpable urticaria
pulmonary infiltration/microscopic hematuria
chronic sinusitis
mononeuritis multiplex
GN with systemic disease
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5
Q

What is the classic triad of Wegner’s (granulomatosis with polyangiitis- GPA)? What gets damaged?

A

granulomatosis vasculitis of the URT/LRT + GN

necrotizing of small veins together with granuloma formation inside/outside the blood vessel

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6
Q

In GPA we can find multifocal _-lateral infiltrations in the lungs. URT gets damages especially in the ___ and ___.

A

bi
nasopharynx
sinuses

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7
Q

Renal involvement in GPA in its early stages is defined by ___ and ___inflammation of the __ which can evolve into ___.

A

focal
segmental
glomeruli
RPGN (rapidly progressive glomerulonephritis)

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8
Q

In GPA patients we can find an increase in ___ blood cells which secret an increased amount of ___ and ___ with an increase of ___. Additionally, there will be high levels of ___ secreted from monocytes.

A 
mononuclear 
IFN- gamma
TNF- alpha
T CD4
IL 12
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9
Q

Which systems can be involved in GPA? (6)

A 
respiratory (90%)
renal (72%)  
ocular (52%)
cutaneous (46%)
nervous (23%)
cardiac (8%)
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10
Q

What are the clinical symptoms of the respiratory system in GPA (4)?

A

asymptomatic infiltration
cough
hemoptysis
dyspnea+ chest pain

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11
Q

What are the clinical symptoms of the ocular system in GPA (3)?

A

conjunctivitis
episcleritis
sclerites

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12
Q

What are the clinical symptoms of the cutaneous system in GPA (5)?

A 
papule
vesicles
palpable rash
ulcers
nodules
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13
Q

What are the clinical symptoms of the cardiac system in GPA (3)?

A

pericarditis
coronary arteries vasculitis
cardiomyopathy

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14
Q

What are the clinical symptoms of the nervous system in GPA (3)?

A

cranial neuritis
mononeuritis multiplex
brain vasculitis/granuloma

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15
Q

Non specific GPA symptoms such as (4) may also appear when the disease is active.

A

fever
arthralgia
lethargy
weight loss/anorexia

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16
Q

In GPA patients there is an increase in ___ ___ events. Therefor we must pay attention to __ and ___

A

venous
thromboembolic
PE
DVT

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17
Q

90% of patients with active GPA have anti-PR3 ___, and in a non active disease- __%. A small percentage will also have __ instead of anti PR3, and 20% will not have __ at all.

A

ANCA
60%
MPO
ANCA

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18
Q

Lab results of GPA patients may include an increase in __, ___, ___and ___, ____ (especially IgA)

A 
ESR
anemia
leukocytosis
thrombocytosis
hypergammaglobulinemia
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19
Q

Diagnosing GPA is through ___ showing ___ in a patient with the suspected clinical presentation. The best location to sample is the ___.

A

biopsy
necrotizing granulomatous vasculitis
lung

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20
Q

Name 5 different disease in the DDx of GPA:

A

anti GBM (Goodpasture’s syndrome)
URT/lung malignancies
lymphoma
infectious disease (histoplasmosis/leishmania)

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21
Q

GPA treatment includes two phases:

A

1- Induction to remission

2- Maintenance

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22
Q

In severe cases of GPA, __+__ is given to achieve remission. In fulminant disease we will also add ___. Other alternative could be ___ or ___

A 
cyclophosphamide
glucocorticoids
plasmapheresis
rituximab
methotrexate
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23
Q

If cyclophosphamide was used for induction use one of the following drugs for maintenance: (4). This treatment should be given for at least ___ years from remission

A 
methotrexate
azathioprine 
mycophenolate mofetil
rituximab 
2
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24
Q

Microscopic polyangiitis is a __ and ___vessel disease of ___ vasculitis type. ___ Ab are common.

A

small
medium
necrotizing vasculitis
ANCA

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25
Q

What is the main difference between GPA and microscopic polyangiitis?

A

the lack of granulomas in the latter

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26
Q

When the vasculitis is more focused in the ___ or ___ it is more likely to GPA

A

URT

lung nodules

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27
Q

The treatment of ___ and ___ is similar and includes: (3)

A 
GPA
microscopic polyangiitis
cyclophosphamide
prednisone
rituximab
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28
Q

Churg Strauss involves the __ and __ blood vessels.

A

small

medium

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29
Q

In Churg Strauss it is common to see: (5)

A

asthma
eosinophilia
extravascular granuloma vasculitis

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30
Q

Churg Strauss patients usually present with non specific clinical symptoms of: (4). In the early stages ___ and ___ are common.

A 
fever
anorexia
abdominal pain
loss of weight
rhinitis
allergic sinusitis
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31
Q

The second most common clinical presentation of Churg Strauss is:

A

mononeuritis multiplex

32
Q

The typical lab results of patients with Churg Strauss include:

A

> 1000 eosinophils
increased ESR
fibrinogen
anti MPO ANCA

33
Q

In order to diagnose a Churg Strauss patient, we must see ___, ____ in peripheral blood, and typical ____ clinical manifestation.

A

asthma
eosinophils
vasculitis

34
Q

If untreated Churg Strauss patients have a poor prognosis with a __ year survival of __%. The main cause for mortality is ___ involvement. For this reason, ___ should be performed when diagnosed.

A

5
25
cardiac
echocardiography

35
Q

In most Churg Strauss patients ___ will be sufficient treatment.

A

glucocorticoid

36
Q

polyarteritis nodosa (PAN) is a ___ and ___ necrotizing vasculitis with systemic and ___ involvement

A

small
medium
renal

37
Q

IN PAN the vascular lesions tend to be ___ and involve the ___ and ___ of the arteries and nearby veins.

A

segmental
bifurcations
branching

38
Q

If we see involvement of __ in polyarteritis nodosa than it is most likely infect ___

A

venules

microscopic polyangiitis

39
Q

The renal pathology in PAN is of ___ without ___. In patients with substantial HTN the kidney may have ___ features

A

arteritis
glomerulonephritis
glomerulosclerosis

40
Q

In patients with viral infections (___ or ___) similar vasculitis to PAN can appear. Another disease that may be associated to PAN is ___

A

HCV
HBV
hairy cell leukemia

41
Q

PAN will have non specific clinical presentation (5), and complains regarding the affected organs.

A 
fever
weight loss
abdominal pain
lethargy/myalgia
headache
42
Q

In order to diagnose PAN we must perform ___ from a relevant site (__, __, __). If it is not possible, we can use ___ to show aneurisms in small/medium blood vessels.

A 
biopsy 
testis
skin patches
muscles
arteriography
43
Q

There is no diagnostic ___ test in PAN. usually we will see ____with ___ dominance. Other results may include ___, ___, ___.

A 
serologic
leukocytosis
neutrophils 
anemia (of chronic disease)
ESR
hypergammaglobulinemia
44
Q

If left untreated, PAN has a __ prognosis. Death may occur mainly due to ___ or ___complication. Treatment should include ___ and ___ to improve survival.

A 
bad
GI
cardiovascular 
prednisone
cyclophosphamide
45
Q

In less sever cases of PAN we can treat patients with __. In HBV patients with PAN treat with ___ + __ + __. Remember that treating HTN will also improve survival

A

GC
antiviral
GC
plasmapheresis

46
Q

Temporal arteritis= ___

A

Giant cell arteritis & polymyalgia rheumatica

47
Q

Temporal arteritis is an inflammation of the __ and __ arteries, usually involving the branches of the ___, especially the __ but also the __ in its branches.

A 
medium
large
carotid
temporal
aorta
48
Q

Temporal arteritis is more common in __ __ __ under the age of __. A correlation was found to HLA DR4 and HLA-DRB1

A

young
white
women
50

49
Q

In __% of patients with temporal arteritis we will also find ___ syndrome which characterized in pain and stiffness of the muscles (4)

A 
50
polymyalgia rheumatica
neck
back
shoulders 
knees
50
Q

The pathogenesis of temporal arteritis involves the attachment of __ cells to the walls of the arteries encouraging __ formation. In the blood vessel we will see ___ cells leading to damage of the internal elastic ___ layer

A

T
macrophages
giant
lamina

51
Q

Temporal arteritis is more common in patients > __ with general symptoms. When cranial arteries are involved the main complain is ___ together with a sensitive and thicken ___.

A

50

artery

52
Q

In temporal arteritis, ischemic cranial complications may include ___ and ___ claudication. The ___ nerve may also be ischemic- leading to a sudden ___. Before those complain appear the patient will suffer from other head/eyes complains.

A

scalp
tongue
optic
loss of sight

53
Q

The lab results we will typically see in temporal arteritis are: (5)

A 
ESR
normochromic anemia
liver functions damage (ALP)
IgG
hypercomplementemia
54
Q

In temporal arteritis there is an increased risk for thoracic aorta aneurysm (X__). The main goal of treatment is to reduce events of loss of ___.

A

18

sight

55
Q 
Temporal arteritis treatment includes \_\_\_. If there are ocular symptoms we should consider giving \_\_\_. We should also add \_\_\_ to reduce cranial events.
Adding \_\_\_ (IL6 receptor antagonist) can be complementary to GC treatment.
A

prednisone
methyl prednisone
aspirin
tocilizumab

56
Q

Takayasu arteritis is an inflammation of the __ and __ arteries with a tendency to involve the ___ and the ___ artery. More common in female___ and in ___

A 
medium
large
aorta
pulmonary
teenagers
Asians
57
Q

In Takayasu there is an ___ of inflammatory cells to the blood vessels- ___ in the intima, ___ of the media layer. As a result the lumen will ___ and sometimes even by ___.

A 
invasion
fibrosis
scarring
narrow
thrombi
58
Q

Takayasu clinical manifestation include ___ symptoms , followed by the loss of ___ in the involved blood vessels (especially in the ___ artery

A

general
pulse
subclavian

59
Q

Takayasu should be suspected in ___ women with a decreased peripheral ___ and with abnormal ___ and arterial ___.

A

young
pulse
HTN
murmurs

60
Q

Using ___ in Takayasu helps to confirm the diagnosis. The least likely artery to be affected is the ___ artery

A

arteriography

coronary

61
Q

Histological examination of arteries of Takayasu patients will show: (3) in the media.

A

lymphocytes
granulomas
giant cells

62
Q

IgA vasculitis= ____

A

Henoch Schonlein

63
Q

IgA vasculitis is a ___ vessel inflammation, characterized with palpable ___ in the ___ and lower extremities areas together with arthralgia, GI symptoms and ___.

A

small
rash
buttocks
GN

64
Q

Henoch Schonlein is more common in children between the ages of ___ but also in infants and adults.. It is more common during the ___ season

A

4-7

spring

65
Q

The common abdominal pain in IgA vasculitis is ___, together with other GI symptoms (3)

A

colic
diarrhea/constipation
vomiting
blood/mucus in PR

66
Q

The renal involvement in IgA vasculitis (10-50% of patients) includes light GN (3) which will resolve spontaneously

A

proteinuria
microscopic hematuria
RBC casts

67
Q

The lab results of Henoch Schonlein includes: (3)

A

leukocytosis
eosinophilia
Increased IgA

68
Q

Diagnosing IgA vasculitis is mostly based on ___ and symptoms. Skin biopsy may show vasculitis with __ and __ deposition

A

clinical presentation
IgA
C3

69
Q

Henoch Schonlein usually resolves ____. In order to reduce the edema we can use __ but it does not improve cutaneous symptoms or joint pain.

A

spontaneously

prednisone

70
Q

Cryoglobulins are Ab that become sediments in __ temperature

A

cold

71
Q

Cryoglobulins can be found in the following disorders: (5)

A 
lymphoproliferative disorders
MM
connective tissue disorders
infections
hepatic disorders
72
Q

The most common causes for essential mixed cryoglobulinemia (__/__ mix sediments)is ___

A

IgM
IgG
chronic hepatitis infection

73
Q

The most common clinical presentation of cryoglobulinemia vasculitis is ___. Other manifestations include: (3)

A

cutaneous vasculitis
arthritis
peripheral neuropathy
GN

74
Q

Less common clinical manifestations of cryoglobulinemia vasculitis include: (2)

A

RPGN

CNC/cardiac vasculitis

75
Q

When examining the lab results of patients with cryoglobulinemia vasculitis, we can see: (5)

A 
cryoprecipitates
RF
ESR
anemia
hypocomplementemia
76
Q

In every cryoglobulinemia vasculitis patient we should look for ___ infection

A

HCV

77
Q

Treatment for cryoglobulinemia include ___ treatment and ___ which will improve symptoms but will not cure. Another drug is ___

A

anti viral
GC
rituximab (anti CD20)

SALMAN HARRISON'S (163 decks)

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