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Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

what is vasculitis?

A

inflammation of blood vessels
lots of different types of vasculitis

small vessels
medium sized vessels
large vessels

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2
Q

types of vasculitis affecting the small vessels

A

Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)

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3
Q

types of vasculitis affecting the medium sized vessels

A

Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease

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4
Q

types of vasculitis affecting the large vessels

A

Giant cell arteritis

Takayasu’s arteritis

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5
Q

how might vasculitis present?

A

Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.

Joint and muscle pain

Peripheral neuropathy

Renal impairment

Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)

Anterior uveitis and scleritis

Hypertension

*fatigue, fever, weight loss, anorexia, anaemia

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6
Q

what are the tests for vasculitis?

A

bloods:
CRP, ESR

special:
ANCA (anti neutrophil cytoplasmic antibodies)

pANCA (anti MPO) (microscopic polyangitis, churg-strauss syndrome)

cANCA (anti PR3) (weighers granulomatosis)

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7
Q

management of vasculitis

A

depends of the type

referral to rheumatology

medical:
- steroids (oral: predniosolone, IV: hydrocortisone, nasal, inhaled)

- immunosupressants
cyclophosphamide
methotrexate
azathioprine
rituximab
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8
Q

HSP

A

IgA vasculitis
purpuric rash which affects lower limb and buttocks in children

deposition of IgA in blood vessels

affects skin, kidneys, GI tract and often triggered by an URTI (tonsillitis) gastroenteritis

the rash is due to leaking of blood from small vessels under the skin (purport)

  1. purpura
  2. joint pain
  3. abdo pain
  4. renal involvement (IgA nephritis)

management:
supportive, analgesia, rest and hydration, steroids

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9
Q

eosinophilic granulomatoiss with polyangitis (churg strauss syndrome)

A

small and medium vessel vasculitis

lung and skin
can affect other organs
severe asthma

! raised eosinophils on FBC

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10
Q

microscopic polyangitis

A

small vessel vasculitis
renal failure
can affect lung causing SOB and haemoptysis

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11
Q

Wegner’s granulomatosis

A

granulomatosis with polyangitis

small vessel vasculitis
affects resp tract and kidneys

nose (epistaxis) and crusty nasal secretions
ears - hearing loss
sinusitis

saddle shaped nose due to perforated nasal septum

lung: wheeze, cough, haemoptysis (X-ray shows condoliation)

kidney - glomeulonephrits

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12
Q

polyarteritis nodosa

A

medium vessel vasculitis
hep B, hep C, HIV
effects skin, GI tract, kidney and heart.
renal impairment, strokes, MI

livedo reticularis
mottled, purplish, lace like rash

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13
Q

Kawasaki disease

A

medium vessel vasculitis
young children <5 y/o

high fever >5 days
erythematous rash
bilateral conjunctivitis
erythema
desquamation of palms and soles (skin peeling)
strawberry tongue

complication is coronary artery aneurysm. treat with aspirin and IV immunoglobulins

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14
Q

takaysau’s arteritis

A

large vessel vasculitis
affects aorta and branches
pulmonary arteries

40 y/o
non specific: fever, malaise, muscle aches, arm claudication, syncope

investigate: CT, MRI, doppler US of carotid arteries.

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15
Q

GCA- giant cell arteritis

A

systemic vasculitis of medium and large arteries

typically affects temporal arteries (temporal arteritis)

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16
Q

symptoms of GCA

A
  • Severe unilateral headache typically around temple and forehead
  • Scalp tenderness my be noticed when brushing hair
  • Jaw claudication
  • Blurred or double vision
  • Irreversible painless complete sight loss can occur rapidly
  • Fever
  • Muscle aches
  • Fatigue
  • Weight loss
  • Loss of appetite
  • Peripheral oedema

complication- vision loss
link with polymyalgia rheumatic

17
Q

how is GCA diagnosed?

A

clinical presentation

bloods:
raised ESR >50mm/hour
FBC: normocytic anaemia, thrombocytosis
LFT raised ALP
CRP

imaging:
duplex ultrasound of temporal artery (hypo echoic halo sign)

special:
temporal artery biopsy
shows multinucleate giant cells

18
Q

management of GCA

A

conservative

medical:
steroids: 40-60mg prednisone (60mg if jaw claudication or visual symptoms) review in 48 hours

aspirin 75mg
PPI (while on steroids)

referral:
vascular surgeon
rheumatology
opthalmology

19
Q

steroids ongoing DONT STOP

A

DON’T – Don’t stop taking steroids abruptly. There is a risk of adrenal crisis.
S – Sick Day Rules.
T – Treatment Card.
O – Osteoporosis prevention with bisphosphonates and supplemental calcium and vitamin D.
P – Proton pump inhibitor for gastric protection.

20
Q

complications of GCA

A

Early neuro-ophthalmic complications:
Vision loss
Cerebrovascular accident (stroke)

Late:
Relapses of the condition are common
Steroid related side effects and complications
Cerebrovascular accident (stroke)
Aortitis leading to aortic aneurysm and aortic dissection

21
Q

antiphospholipid syndrome

A

disorder associated with antiphospholipid antibodies. blood becomes prone to clotting (hyper-coagulable state)

  • thormobisis
  • pregnancy- recurrent miscarriage

can exist alone or secondary to SLE

22
Q

antiphospholipid antibodies

A

Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies

they interfere with coagulation

23
Q

associated symptoms of antiphospholipid syndrome

A

venous thromboembolism
DVT, PE

arterial thrombosis:
stroke, MI, renal thrombosis

pregnancy:
recurrent miscarriage
still birth
pre eclampsia

livedo reticularis
libmann-scks endocarditis (mitral valve)
thrombocytopenia

24
Q

how is antiphospholipid syndrome diagnosed?

A

history of thrombosis / pregnancy complications

Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies

25
Q

management of antiphospholipid syndroem

A

rheumatology
haematology
obstetrics (if pregnant)

medical:
warfarin INR 2-3
(3-4 if recurrent thrombosis)

LMWH (enoxaparin)
aspirin

26
Q

Behcet’s disease

A

a complex inflammatory condition. oral and genital ulcers. inflammation of skin, GI, lung, blood vessels, MSK and CNS

link with HLA-B51 gene

27
Q

differential diagnosis of Behcet’s disease

A

(mouth ulcers)

Simple aphthous ulcers are very common
Inflammatory bowel disease (particularly Crohn’s disease)
Coeliac disease
Vitamin deficiency (B12, folate or iron)
Herpes simplex ulcers
Hand, foot and mouth disease (coxsackie A virus)
Squamous cell carcinoma

28
Q

features of Behcets disease

A

mouth ulcers- 3 episodes per year. painful, sharply circumscribed erosions, red halo. heals 2-4 weeks

genial ulcers- kissing ulcers (two opposing surfaces facing each other)

skin- erythema nodosum, papule and pustules, vasculitis type rashes

eyes- anterior/posterior uveitis, retinal vasculitis, retina haemorrhage

MSK - morning stiffness, arthralgia, oligoarthritis

GI- ileum, cecum ascending colon inflammation and ulceration

CNS- memory impairment, headaches, migraine, aseptic menigntisi, meningoencephalitis

veins- buddd chiari, DVT, thrombus in pulmonary veins, cerebral venous sinus thrombosis

lungs- pulmonary artery aneurysm

29
Q

investigation for Behcet’s disease

A

pathergy test: sterile needle to create a subcutaneous abrasion on the forearm. reviewed 24-48 hours later to look for a weal of 5mm or more. +ve

30
Q

management of Behcet’s disease

A

rheumatology
opthalmology
dermatology
neurology

conservative

medical:
steroids- soluble bethamethasone for mouth ulcers
systemic steroids- prednsiolone
colchine - anti inflamamotry
topical anaesthetic- lidocaine
immunosupressants- azathoprine
biological therapy infliximab
31
Q

prognosis for Behcet’s disease

A

relapsing remitting

increased mortality with haemoptysis, neurological involvement

Rheumatology (20 decks)

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