Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels

Resulting in

  • lumen narrowing
  • impaired blood flow
  • organ damage
  • wall thinning and aneurysm
  • perforation and haemorrhage
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2
Q

How are vasculitides classified?

List the causes

A

Large

  • GCA
  • Takayasu’s

Medium

  • Polyarteritis nodosa
  • Kawasaki

Small is split into two:

ANCA +ve:

  • Wegner’s (GPA)
  • Microscopic polyangiitis
  • Churg-Strauss

ANCA -ve

  • HSP
  • Goodpasture’s
  • Autoimmune disease associated
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3
Q

What is ANCA?

Types, associations?

A

Anti-neutrophil cytopasmic antibodies

Two types

cytoplasmic ANCA

  • Assoc with MPO antigen
  • Wegner’s (GPA)

peri-nuclear ANCA

  • Assoc with PR3 antigen
  • Churg Strauss
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4
Q

Two types of GCA?

Describe

A

Temporal: as in other flashcards

Systemic GCA: can affect any large vessels in the body

  • mesenteric
  • subclavian
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5
Q

How does systemic GCA present?

A

Often just fever, malaise, myalgia

If mesenteric - abdo pain

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6
Q

What is Takayasu’s?

Who is affected?

A

Vasculitis of aorta and major branches

Granulomatous inflammation resutling in

  • stenosis
  • thrombosis
  • aneurysms

20s-40s
Asian origin
Women

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7
Q

Features of Takayasu’s?

A

Cerebral: dizzy

Ophthalmological: visual changes

Upper limb: weak arm pulses

Plus systemic: fever, malaise, myalgia

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8
Q

Management of Takayasu’s?

A

High dose steroids
Methotrexate
Cyclophosphamide

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9
Q

What is polyarteritis nododa?

Presentation?

A

Medium vessel vasculitis

Necrotising
Aneurysms
Thrombosis

Which can lead to organ damage

Severe systemic symptoms (fever, maliase, myalgia)

Skin: livedo reticularis, ulcers, petechial rash

Renal damage (due to hypertension not glomerulonephritis)

Epidymitis

Cardiac, GI, GU

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10
Q

Investigations of polyarteritis nodosa?

A

Raised white cells
Mild eosinophilia
Raised ESR, CRP
ANCA -ve

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11
Q

Management of polyarteritis nodosa?

A

Control BP to protect kidneys

Corticosteroids
Cyclophosphamide

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12
Q

What disease is polyarteritis nodosa associated with?

A

Hepatitis B

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13
Q

Subtypes of small vessel vasculitides?

A

ANCA +ve

  • GPA granulomatosis with polyangiitis (Wegener’s)
  • EPGA eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

ANCA -ve
- HSP

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14
Q

What’s the new name for Wegener’s ?

A

Granulomatosis with polyangiitis (GPA)

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15
Q

Pathophysiology of GPA?

Who gets it?

A

Necrotising vasculitis of arterioles, capillaries, post-capillary venules

Propensity for upper resp tract, lungs, kidneys

Men 25-60

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16
Q

Presentation of GPA?

A

Upper resp tract: sinusitis, otitis, nasal crusting and bleeding, saddle nose deformity

Lungs: pulmonary nodules +/- cavitation

Renal: nephritic syndrome

Skin: ulcers, purpura

Neuropathies

Eyes: proptosis, uveitis

17
Q

Investigations of GPA?

A

cANCA against PR3 antibody

Raised ESR, CRP

CXR: cavitating lesions

Biopsy

18
Q

Pathophysiology of EGPA?

Who gets it?

A

Similar to GPA but eosinophilic involvement

30-45yrs
Men

19
Q

Presentation of EGPA?

A

Triad of

Adult onset asthma

Eosinophilia

Vasculitis

Affects nerves, skin, lungs

Presents like septic shock

20
Q

Management of the ANCA +ve small vessel vasculitides?

A

Induce remission:

  • high dose steroids + cylophosphamide or rituximab
  • plasma exchange
  • or if no organ involvement steroids + methotrexate

Maintain remission:

  • methotrextae
  • azathrioprine
21
Q

What is HSP?

Who gets it?
Causes?

A

Henoch Schonlein purpura

IgA mediated vasculitis of small vessels

Following an URTI

Children usually

22
Q

Presentation of HSP?

A

Pupuric rash over buttocks and legs and extensor surfaces

Glomerulonephritis
Arthritis
Abdo pain

23
Q

What GI disorder are HSP patients at higher risk of?

A

Intesussception

24
Q

Management and monitoring of HSP?

A

None usually needed

Steroids

Monitor renal function closely as can result in CKD