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rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

define vasculitis

A

a heterogenous group of rare inflammatory conditions that can occur independently or as a complication of an established disease (eg. RA or SLE)

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2
Q

basic pathophysiology

A

sterile inflammation directed against a blood vessel wall, leading to damage and destruction to the vessel wall (histologically seen as fibrinoid necrosis)

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3
Q

distribution of vasculitis

A

may localise to a single organ/vascular bed (relatively benign)
more commonly generalised and causing organ failure - combination of ischaemia (vessel obstruction) and bleeding (aneurysm formation)

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4
Q

clinical distinction of different types of vasculitis is mainly based on

A

assumed vessel size affected based on pattern of organ injury

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5
Q

types of large vessel vasculitis

A

takayasu arteritis
giant cell arteritis

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6
Q

medium vessel vasculitis

A

polyarteritis nodosa
Kawasaki disease

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7
Q

ANCA-associated small vessel vasculitis

A

microscopic polyangitis
granulomatosis with polyangitis (Wegener)
eosinophilic granulomatosis with polyangitis (churg-strauss)

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8
Q

immune complex small-vessel vasculitis

A

cryoglobulinaemic vasculitis
IgA vasculitis (hence-schonlein)
hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

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9
Q

where might you find a continuous vascular tissue bed

A

has tight junctions

found in CNS, lymph nodes, muscle

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10
Q

what’s continuous vascular tissue beds used for

A

has tight junctions

used for BBB, lymphocyte homing, metabolic exchange

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11
Q

where might you find a fenestrated vascular tissue bed

A

has fenestra

found in exocrine glands, GI tract, choroid plexus, kidney glomeruli

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12
Q

what’s fenestrated vascular tissue used for

A

has fenestra

secretion
absorption
secretion
filtration

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13
Q

where might you find discontinuous vascular tissue

A

has gaps

liver, bone marrow, spleen

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14
Q

what’s the purpose of discontinuous vascular tissue

A

has gaps

particle exchange, haematopoiesis, blood cell transfer

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15
Q

what are pericytes

A

cells interacting with blood vessels in brain parenchyma
help to
- contribute to BBB
- immune and phagocyte functions
- role in haemostasis
- contracitile functions
- participate in vascular development

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16
Q

what increases the vulnerability of vessel walls to inflammation

A

exogenous (infections/toxic)
endogenous (ageing)

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17
Q

typical symptoms of vasculitis

A

regardless of size of vessels involved, patients often present with:
fever, night sweats, fatigue, anorexia, weight loss, arthralgiaa

18
Q

some life or organ threatening manifestations of vasculitis

A

alveolar damage
rapidly progressive glomerulonephritis
mesenteric ischaaemia
orbital pseudotumour threatening optic nerve (in GPA)
vision loss in patients with giant cell arteritis

19
Q

lab diagnostics for vasculitis

A

hb, ESR, CRP, serum albumin, creatinine, urine MCS/ACR
antineutrophil cytoplasmic antibodies (ANCA) tests

20
Q

inducing remission

A

high dose corticosteroids (often combined with cytotoxic immunosuppressants for severe disease), usually for 3-6 months

21
Q

gold standaard for ANCA associated vasculitis

A

rituximab (anti-CD20, B cell depleting agent)

22
Q

aims during period post remission

A

during this period, goal is to eliminate corticosteroids or reduce their dose and to use less potent immunosuppressants eg. methotrexate, azathioprine

23
Q

maintaining remission

A

corticosteroids tapered to zero or lowest dose that maintains remission
usually methotrexate with folate or azathioprine is prescribed to replace cyclophosphamide

24
Q

if patients have frequent relapses

A

they may need to take immunosuppressants indefinitely

25
Q

giant cell arteritis affects

A

most common for of systemic vasculitis in the elderly
involvement of large vessels, predominantly the extra cranial branches of the aorta

26
Q

symptoms of giant cell arteritis

A

fever, fatigue, anorexia, weight loss and depression
headache - over one or both temporal lobes
jaw claudication - pain in the tongue or jaw during mastication, which resolves with rest

27
Q

giant cell arteritis is closely associated with

A

polymyalgia rheumatica PMR
overlap in 25-50% of patients

28
Q

opthalmic manifestations of GCA

A

acute visual loss in 20% of patients, usually sudden and painless

29
Q

causes of ophthalmic manifestations of GCA

A

anterior ischaemic optic neuropathy
central retinal artery occlusion
posterior ischaemic optic neuropathy
cortical blindness

30
Q

warning symptoms of imminent blindness in GCA

A

blurry vision, amaurosis fugax, visual hallucinations, and diplopia
this is medical emergency - prompt treatment may prevent development of irreversible blindness

31
Q

GCA on examination

A

temporal arteries tender/nonpulsitile/thickened
bruits may be audible over affected arteries (carotid and brachial)

32
Q

biopsy for GCA

A

biopsy specimen with an artery showing vasculitis characterised by a predominance of mononuclear infiltration or granulomatous inflammation

33
Q

age of inset of GCA

A

> 50 years

34
Q

4 types ANCA associated vasculitis

A

MPA: microscopic polyangitis
GPA: granulomatosis with polyangitis
EGPA: eosinophilic grnaulomatosis with polyangitis
ANCA glomerulonephritis (or renal limited vasculitis)

35
Q

MPA

A

microscopic polyangitis
vasculitis in kidneys, skin, nerves, and lungs

36
Q

GPA

A

granulomatosis with polyangitis
vasculitis+granulomatosis inflammation: lung, sinuses, nose, eyes or ears

37
Q

EGPA

A

eosinophilic granulomatosis with polyaangitis
vasculitis+granulomatous inflammation + asthma + eosinophilia

38
Q

ANCA stands for

A

anti neutrophil cytoplasmic antibodies

39
Q

do the levels of ANCA correlate with disease activity

A

only modest relationship with disease activity

40
Q

what us HCV associated cryglobulinaemia with vasculitis

A

immune complex mediated small vessel disease
predilection for skin, peripheral nerve and renal glomerulus injury
when cryoglobulins precipitate and deposit on vascular endothelium, resulting in vasculitis

41
Q

what are cryoglobulins

A

cold-insoluble complexes

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