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CR > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Define vasculitis

A

Inflammatory disease of the blood vessels

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2
Q

How is vasculitis classified?

A
  1. Large vessel
  2. Medium vessel
  3. Small vessel
    (4. Variable)
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3
Q

Examples of large vessel vasculitis (2)

A

Giant cell arteritis

Takayasu arteritis

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4
Q

Examples of medium vessel vasculitis (2)

A

Polyarteritis nodosa

Kawasaki disease

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5
Q

Examples of small vessel vasculitis

A

ANCA positive - GPA, EGPA, MPA

Immune complex - anti-GBM, cryoglobulinaemic, IgAV, HUV

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6
Q

Examples of variable vessel vasculitis (2)

A

Behcet’s

Cogan’s

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7
Q

Giant cell arteritis - pathology

A

Systemic granulomatous arteritis (large vessel vasculitis). It affects the aorta and its major branches, particularly extracranial branches of the carotid artery.

Granulomatous lesions may be seen on biopsy (although up to 50% are normal)

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8
Q

Most common type of GCA

A

Temporal arteritis

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9
Q

Who is most typically affected by GCA?

A

Older (>50), white, female > males

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10
Q

Temporal arteritis features

A 
usually rapid onset (e.g. < 1 month)
headache
jaw claudication
visual disturbances (anterior ischemic optic neuropathy)
tender, palpable temporal artery
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11
Q

Temporal arteritis investigations

A

ESR > 50 mm/hr
CRP may also be elevated
Temporal artery biopsy- granulomatous inflammation, but normal in 50% due to skip lesions
Creatine kinase and EMG = normal

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12
Q

Temporal arteritis treatment

A

High-dose prednisolone + urgent opthal review (same day)

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13
Q

What other condition is GCA/TA associated with?

What are the symptoms?

A

PMR (polymyalgia rheumatica)
Aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats

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14
Q

Takayasu arteritis - what is it?

Typically affects…?

A

Granulomatous inflammation, affecting aorta and its major branches

Young (< 40), mostly Asians, more common in females

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15
Q

Takayasu arteritis symptoms

A 
Intermittent claudication (usually upper limb)
Systemic features e.g. malaise, headache
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16
Q

Takayasu arteritis signs

A

Decreased arterial pulse
BP difference between arms > 10 mmHg
Carotid bruit (listen for any large vessel bruit)
Aortic regurg

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17
Q

Takayasu arteritis investigations

A

ESR raised
Arteriogram - narrowing of the aorta or its proximal branches (usually focal/segmental)
CT PET with increased FDG uptake in affected vessel walls

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18
Q

Polyarteritis nodosa - what is it/pathology?

A

Vasculitis affecting medium-sized vessels with necrotizing inflammation leading to aneurysm formation.
–> major organ infarcts, ruptured aneurysms

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19
Q

PAN is associated with?

More common in?

A

Hepatitis B

Middle aged men

20
Q

PAN symptoms

A 
Malaise, arthralgia, weight loss
Mononeuritis multiplex
Sensorimotor polyneuropathy
Testicular pain
Haematuria
21
Q

PAN signs

A 
Pyrexia
Hypertension
Livedo reticularis
Renal failure (due to renal artery involvement)
Digital infarcts
22
Q

PAN investigations

A

Check Hep B serology
Perinuclear antineutrophil cytoplasmic antibodies (ANCA) positive in 20%
Mesenteric/renal angiogram -saccular aneurisms, vessel narrowing, dilatation

23
Q

Kawasaki disease - what is it, who does it usually affect

A

Uncommon medium vessel vasculitis

Children

24
Q

Kawasaki disease features

A 
Conjunctival injection
Rash
Adenopathy (cervical lymphadenopathy)
Strawberry tongue (and red cracked lips)
Hands and feet (red + later peel)
and
BURN = high grade fever which lasts for >5 days (resistant to antipyretics)
25
Q

Kawasaki disease management

A

Screen for coronary artery aneurysms (echo)
High dose aspirin
IVIG

26
Q

Granulomatosis with polyangiitis (previously known as Wegener’s)
- what is it?

A

Autoimmune small vessel necrotising granulomatous vasculitis, affecting the upper and lower resp tract and the kidneys

27
Q

Granulomatosis with polyangiitis features

A

Upper respiratory tract - epistaxis, sinusitis, nasal crusting, saddle shape nose deformity
Lower respiratory tract - dyspnoea, haemoptysis
Kidneys - rapidly progressive glomerulonephritis (‘pauci-immune’, 80% of patients)

Can also get vasculitic rash, eye involvement and CN lesions

28
Q

Granulomatosis with polyangiitis investigations

A

cytoplasmic (c)ANCA positive in > 90%
CXR - may have cavitating lesions
Renal biopsy - epithelial crescents in Bowman’s capsule

29
Q

Granulomatosis with polyangiitis management

A

steroids
cyclophosphamide
plasma exchange

30
Q

Eosinophilic granulomatosis with polyangiitis (previously known as Churg-Strauss) - what is it?

A

ANCA-associated small vessel vasculitis

31
Q

Eosinophilic granulomatosis with polyangiitis features

A

Asthma, blood eosinophilia (>10%), paranasal sinusitis, allergic rhinitis, mononeuritis multiplex

32
Q

Eosinophilic granulomatosis with polyangiitis investigations

A

perinuclear (p)ANCA positive in 60%

33
Q

Simple difference between GPA and EGPA

A

GP has kidney involvement
GP = cANCA
EGPA = pANCA

34
Q

Microscopic polyangiitis features

A

Renal impairment: raised creatinine, haematuria, proteinuria
Systemic symptoms: fever, lethargy, myalgia, weight loss
Rash: palpable purpura
Cough, dyspnoea, haemoptysis
Mononeuritis multiplex

35
Q

Microscopic polyangiitis investigations

A

pANCA (against myeloperoxidase) - positive in 50-75%
cANCA (against proteinase 3) - positive in 40%
NO GRANULOMATOSIS

36
Q

What is anti-GBM/pathology (Goodpasture’s)

A

Rare small vessel vasculitis associated with pulmonary haemorrhage followed by rapidly progressive glomerulonephritis
It is caused by anti-glomerular basement membrane antibodies against type IV collagen

37
Q

Associations with anti-GBM

A

HLA DR2
Men
Ages 20-30, and 60-70 (two peaks)

38
Q

Anti-GBM investigations

A

Renal biopsy: linear IgG deposits along the basement membrane
Raised transfer factor secondary to pulmonary haemorrhages

39
Q

Anti-GBM management

A

plasma exchange
steroids
cyclophosphamide

40
Q

What is Behcet’s?

A

Complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins.

41
Q

Behcet’s classic triad

A

Oral ulcers
Genital ulcers
Anterior uveitis

42
Q

Other features of Behcet’s

A

VTE, arthritis, neuro involvement e.g. aseptic meningitis, abdo pain, diarrhoea, colitis, erythema nodosum

43
Q

Behcet’s investigations

A

Clinical diagnosis

Positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)

44
Q

Behcet’s epidemiology

A

More common in the eastern Mediterranean (e.g. Turkey), in men, and in young adults
Associated with HLA B51

45
Q

General diagnostic approach to vasculitis

A

Lab tests - RF, ANA, ANCA, cryoglobulins, C3, C4, ESR, CRP
Tissue biopsy of affected organ
Imaging e.g. CT PET for large vessels, angiography for PAN, echo for Kawasaki’s
Rule out differentials - blood culture, viral hep and HIV screen, echo, protein electrophoresis

46
Q

Vasculitis mimics

A

Drugs
Infections e.g. HIV, sepsis, hepatitis
Malignancy e.g. myeloma

CR (19 decks)

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