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Y2 Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Pathogenesis of Vasculitis

A

Inflammation of BVs leading to:

  • vessel wall thickening
  • stenosis
  • occlusion –>
  • ischaemia
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2
Q

Large vessel vasculitis (2)

A

Takayasu arteritis

Giant cell arteritis

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3
Q

Medium vessel vasculitis (2)

A

Polyarteritis nodosa

Kawasaki disease

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4
Q

Small-vessel vasculitis

A

Microscopic polyangitis
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Cryoglobulinemic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypocomplementemic urticarial vascilitis (Anti-C1a vasculitis)
Anti-GBM disease

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5
Q

ANCA-associated small vessel vasculitis

A

Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss

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6
Q

Immune complex small vessel vasculitis

A

Cryoglobulinemic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypocomplementemic urticarial vasculitis (Anti-C1q vasculitis)
Anti-GBM disease

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7
Q

General presentation of Vaculitis

A

Fever
Malaise
Weight loss
Fatigue

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8
Q

Large vessel vasculitis:

  • pathology?
  • main differences?
  • general symptoms
  • investigations
A

Pathology: primary vasculitis causing chronic granulomatous inflammation predominantly of the aorta & its major branches
Difference:
-age of onset (TA before 50, GCA after 50),
-GCA mainly in temporal arteries
Signs & Symptoms:
-early on they are non-specific (Fever, malaise, night sweats, weight loss, arthralgia, fatigue),
-claudicant symptoms in upper & lower limbs
Investigation:
-CPR, PV/ESR elevated;
-MR angiography (thickened vessel walls & stenosis)

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9
Q

Results of untreated large vessel vasculitis

A

Vascular stenosis & Aneurysm

  • reduced pulses
  • bruits
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10
Q

What age is Takayasu Arteritis diagnosed at?

A

before 50

young women in 20s-30s

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11
Q

Where is Takayasu Arteritis most common?

A

East Asian countries

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12
Q

Signs & Symptoms of Takayasu Arteritis

A

early on they are non-specific:
low-grade fever, malaise, night sweats, weight loss, arthralgia, fatigue
claudicant symptoms in upper & lower limbs
if untreated - vascular stenosis & aneurysm (reduced pulses, bruits)

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13
Q

Investigations of Takayasu Arteritis

A

CPR, PV/ESR elevated

MR angiography: detect thickened vessel walls and stenosis

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14
Q

Treatment of Takayasu Arteritis

A
  • prednisolone starting at 40-6mg
  • gradually reduced
  • methotrexate & azathioprine may be added
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15
Q

Pathology of GCA

A

Transmural inflammation of intima, media, adventitia of affected arteries
Patchy infiltration: lymphocytes, macrophages, multinucleated giant cells
mononuclear infiltration / granulomatous inflammation

Vessel wall thickening - arterial luminal narrowing - distal ischaemia

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16
Q

What age is GCA diagnosed at?

A

after 50

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17
Q

What is the most common form of systemic vasculitis in adults?

A

GCA

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18
Q

Signs & Symptoms of GCA?

A 
Visual disturbances (50%)
Headache: 
-continous
-temporal/occipital areas, 
-focal tenderness on direct palpation
Jaw claudication:
-fatigue/discomfort of muscles during chewing/speaking
-ischaemia of maxilalry artery
Scalp tenderness

Temporal artery may be thickened, prominent, tender to touch

Constitutional: fatigue, malaise, fever

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19
Q

When to worry about GCA

A

new onset headache in >50
elevated CPR, PV/ESR
always always consider GCA

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20
Q

Diagnosis of GCA

A

raised inflammatory markers

temporal artery biopsy - ASAP

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21
Q

specificity of positive temporal artery biopsy

A

100%

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22
Q

sensitivity of temporal artery biopsy

A

15-40% (patchy involvement)

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23
Q

treatment of GCA if no visual impairment

A

40mg prednisolone

  • start as soon as diagnosis is suspected (don’t wait for results of biopsy)
  • gradually tapered over 2 years (majority resolve)
24
Q

treatment of GCA if there is visual impairment

A

60mg prednisolone

  • start as soon as diagnosis is suspected (don’t wait for results of biopsy)
  • gradually tapered over 2 years (majority resolve)
25
Q

% of GCA patients with permanent visual impairment

A

20%

26
Q

General signs & symptoms of small-medium vasculitis

A 
fever
weight loss
raised, non blanching purpuric rash
arthralgia/arthritis
mononeuritis multiplex
glomerulonephritis
lung opacities on XRay
27
Q

ANCA associated vasculitis

+ NO granuloma

A

Microscopic polyangiitis (MPA)

28
Q

ANCA associated vasculitis
+ WITH granuloma
+ NO asthma
+ NO Eosinophilia

A

Granulomatosis with polyangiitis (GPA) (= Wegener’s)

29
Q

ANCA associated vasculitis
+WITH granuloma
+WITH asthma
+WITH eosinophilia

A

Eosinophilic granulomatosis with polyangiitis (EGPA) (=Churg-Strauss)

30
Q

Non-ANCA associated vasculitis

+ WITH IgA dominant immune deposit

A

Henoch-Scnlein Purpura

31
Q

Non-ANCA associated vasculitis
+NO IgA dominant immune deposit
+NO serum cyroglobulin

A

other non ANCA vasculitis (e.g. IBD)

32
Q

Non-ANCA associated vasculitis
+NO IgA dominant immune deposit
+serum cyroglobulin

A

Cyroglobulinemia

33
Q

Pathology behind GPA

A

Granulomatous inflammation of respiratory tract, small and medium vessels
Necrotising glomerulonephritis common

34
Q

Pathology behind EGPA

A

Eosinophilic granulomatous inflammation of resporatory tract, small and medium vessels
Associated with Asthma

35
Q

Pathology behind MPA

A

Necrotising vasculitis with few immune deposits.

Necrotising glomerulonephritis very common

36
Q

Which ANCA is GPA associated with?

A

cANCA

37
Q

Which ANCA is EGPA associated with?

A

pANCA

38
Q

Which ANCA is MPA associated with?

A

pANCA

39
Q

Which vasculitis is cANCA associated with?

A

GPA

40
Q

Which vasculitis is pANCA associated with?

A

EGPA, MPA

41
Q

Which vasculitis is PR3 ab mainly associated with?

A

GPA

42
Q

Which vasculitis is MPO associated with?

A

EGPA

MPA

43
Q

M:F ratio of GPA

age of GPA diagnosis

A

M:F 1.5:1

35-55y

44
Q

ACR criteria for GPA classification

A 
2 or more criteria 
nasal/oral inflammation: 
-painful/painless ulcers 
-or purulent/bloody nasal discharge
abnormal chest radiograph:
-nodules, fixed infiltrates/cavities
urinary sediment
-microhematuria (>5 RBC/high power field)
-or red cell casts in urine
granulomatous inflammation on biopsy:
-histologic changes showing granulomatous inflammation within the wall of an artery or  in the perivascular/ extravasscular area (artery/arteriole)
45
Q

GPA antibodies

A

cANCA PR3

46
Q

Investigation of GPA

A 
Raised inflammatory markers: CRP, PV/ESR
Anaemia of chronic disease
U&E for renal involvement 
Urinalysis
CXR
Biopsy of affected area: skin, kidney
Immunology: cANCA, PR3
47
Q

Management of ANCA Associated Vaculitis (GPA, EGPA, MPA)

A

localized/early systemic: methotrexate & steroids (?azathioprine & steroids)
Generalised/systemic:
-cyclophosphamide + steroids (1st line)
-rituximab + steroids (alternative)
-plasma exchange if creatinine > 500
-followed by azatioprine with alternatives being methotrexate
Refractory: IV immunoglobulins, rituximab

48
Q

ACR criteria for EGPA

A

4 or more of:
asthma: wheezing
eosinophilia of more than 10% in peripheral blood
paranasal sinusitis
pulmonary infiltrates (may be transient)
histological proof of vasculitis with extravasular eosinophils
mononeuritis multiplex/ polyneuropathy

49
Q

EGPA Investigation

A 
Inflammatory markers: CRP, PV/ESR raised
if chronic disease: check anaemia
U&E for renal involvement
urinalysis for renal involvement
CXR
biopsy of affected area - skin, kidney
50
Q

EGPA antibodies

A

pANCA, MPO

51
Q

% of MPA patints with necrotising glomerulonephritis

A

90%

52
Q

MPA Investigation

A 
Inflammatory markers: CRP, PV/ESR raised
if chronic disease: check anaemia
U&E for renal involvement
urinalysis for renal involvement
CXR
biopsy of affected area - skin, kidney
53
Q

MPA antibodies

A

pANCA, MPO

54
Q

Pathology behind HSP (Henoch-Schonlein Purpure)

A

Acute IgA mediatd disorder causing generalized vasculitis, involving the small vessels of the: skin, GIT, kidneys, joints, rarely the lungs or CNS

55
Q

Age of HSP

A

children common

commonly history of URTI a few weeks ago

56
Q

Signs & Symptoms of HSP

A

Purpuric rash over the buttocks & lower limbs
abdominal pain
vomiting
joint pain

57
Q

treatment of HSP

A

self-limiting condition
no specific treatment
settles over weeks-months

Y2 Rheumatology (7 decks)

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