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Y2 Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

(57 cards)

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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

Study These Flashcards
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

Study These Flashcards
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
% of GCA patients with permanent visual impairment
20%
26
General signs & symptoms of small-medium vasculitis
``` fever weight loss raised, non blanching purpuric rash arthralgia/arthritis mononeuritis multiplex glomerulonephritis lung opacities on XRay ```
27
ANCA associated vasculitis | + NO granuloma
Microscopic polyangiitis (MPA)
28
ANCA associated vasculitis + WITH granuloma + NO asthma + NO Eosinophilia
Granulomatosis with polyangiitis (GPA) (= Wegener's)
29
ANCA associated vasculitis +WITH granuloma +WITH asthma +WITH eosinophilia
Eosinophilic granulomatosis with polyangiitis (EGPA) (=Churg-Strauss)
30
Non-ANCA associated vasculitis | + WITH IgA dominant immune deposit
Henoch-Scnlein Purpura
31
Non-ANCA associated vasculitis +NO IgA dominant immune deposit +NO serum cyroglobulin
other non ANCA vasculitis (e.g. IBD)
32
Non-ANCA associated vasculitis +NO IgA dominant immune deposit +serum cyroglobulin
Cyroglobulinemia
33
Pathology behind GPA
Granulomatous inflammation of respiratory tract, small and medium vessels Necrotising glomerulonephritis common
34
Pathology behind EGPA
Eosinophilic granulomatous inflammation of resporatory tract, small and medium vessels Associated with Asthma
35
Pathology behind MPA
Necrotising vasculitis with few immune deposits. | Necrotising glomerulonephritis very common
36
Which ANCA is GPA associated with?
cANCA
37
Which ANCA is EGPA associated with?
pANCA
38
Which ANCA is MPA associated with?
pANCA
39
Which vasculitis is cANCA associated with?
GPA
40
Which vasculitis is pANCA associated with?
EGPA, MPA
41
Which vasculitis is PR3 ab mainly associated with?
GPA
42
Which vasculitis is MPO associated with?
EGPA | MPA
43
M:F ratio of GPA | age of GPA diagnosis
M:F 1.5:1 | 35-55y
44
ACR criteria for GPA classification
``` 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
GPA antibodies
cANCA PR3
46
Investigation of GPA
``` 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
Management of ANCA Associated Vaculitis (GPA, EGPA, MPA)
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
ACR criteria for EGPA
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
EGPA Investigation
``` 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
EGPA antibodies
pANCA, MPO
51
% of MPA patints with necrotising glomerulonephritis
90%
52
MPA Investigation
``` 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
MPA antibodies
pANCA, MPO
54
Pathology behind HSP (Henoch-Schonlein Purpure)
Acute IgA mediatd disorder causing generalized vasculitis, involving the small vessels of the: skin, GIT, kidneys, joints, rarely the lungs or CNS
55
Age of HSP
children common | commonly history of URTI a few weeks ago
56
Signs & Symptoms of HSP
Purpuric rash over the buttocks & lower limbs abdominal pain vomiting joint pain
57
treatment of HSP
self-limiting condition no specific treatment settles over weeks-months

Y2 Rheumatology (7 decks)

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