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Y4: Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A
  • Inflammation of blood vessel walls
  • Associated with the ANCA protein
  • Classified by vessel size
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2
Q

What are the causes of vasculitis?

A
  • Infection (majority)
  • Drugs
  • Cancer
  • Autoimmune (RA)
  • Cold temperature
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3
Q

Describe the categories of Vasculitis?

A
  • Large vessels
  • Medium vessels
  • Small vessels
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4
Q

Name types of large vessel vasculitis?

A
  • Giant cell/ Temporal arteritis
  • Takayasus’s arteritis
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5
Q

Name types of medium vessel vasculitis?

A
  • Kawasaki’s
  • Polyarteritis nodosa
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6
Q

Name types of small vessel vasculitis?

A

Henoch-schonlein purpura

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7
Q

Name some of the clinical manifestations of vasculitis?

A
  • Arthritis
  • Kidney failure
  • Purpura rashes
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8
Q

What is the treatment for vasculitis?

A

Corticosteroids

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9
Q

How is vasculitis diagnosed?

A
  • Raised ESR and CRP in blood
  • Definitive diagnosis made by biopsy of vessel
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10
Q

Describe Henoch-schonlein purpura?

A
  • Children
  • Begins in foot then moves up
    • Arthritis
    • Abdominal pain
    • Purpura rash in LLs
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11
Q

What are the causes of cutaneous vasculitis?

A
  • Idiopathic
  • Drugs
  • Infection
  • Malignancy
  • Manifestation of small/medium ANCA associated vasculitis
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12
Q

Name some complications of Henoch-Schonlein Purpura?

A
  • Orchitis
  • GI bleeds
  • Diarrhoea
  • IgA nephropathy
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13
Q

Describe the management of Henoch Schonlein Purpura?

A
  • Usually self-limiting
  • Corticosteroids for complications
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14
Q

What can sometimes trigger Henoch Schonlein Purpura?

A

Streptoccocal sore throat

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15
Q

Describe Granulomatosis with polyangiitis (GPA)?

A
  • Granuloma formation
    • Nasal passages, airways and kidney
  • Presentation with epistaxis, nasal crusting, sinusitis
  • Deafness and proptosis may occur
  • Pauci-immune glomerulonephritis
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16
Q

What was the previous name of Granulomatosis with polyangiitis (GPA)?

A

Wegener’s granulomatosis

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17
Q

What is ANCA?

A
  • Autoantibodies which attack neutrophils and monocytes
  • Often present in vasculitis
18
Q

How can you test for ANCA?

A
  • Indirect immunofluoresence
  • ELISA for PR3 / MPO
19
Q

What are the uses of ANCA?

A
  • Diagnosis
  • Prognosis
  • Response to treatment
  • Monitoring for relapse
20
Q

What are the pitfalls of ANCA testing?

A
  • Must be done in clinical setting
  • Not all autoimmune vasculiis is positive for ANCA
21
Q

How do you treat someone who is positive for ANCA?

A
  • Remission induction
  • Remission maintenace
22
Q

What drugs are used for remission induction?

A
  • Prednisolone AND
  • For severe disease:
    • Cyclophosphamide
    • Rituximab
  • For moderate disease:
    • Methotrexate
    • Mycophenolate
23
Q

What drugs would be used for remission maintenance?

A
  • Azathioprine
  • Methotrexate
24
Q

What are the main subtypes of ANCA vasculitis?

A
  • Microscopic polyangitis
  • Granulomatosis with polyangitis
25
Q

Describe Microscopic polyangitis?

A
  • Necrotising small-vessel vasculitis
  • Rapidly progressive glomerulonephritis
  • Often with alveolar haemorrhage
  • MPO antibody positive
26
Q

Gold standard for diagnosis of ANCA vasculitis?

A

Biopsy of kidneys or lesions in sinuses/upper airways

27
Q

Describe the management of ANCA-associated vasculitis?

A
  • High dose glucocorticoids
      • Cyclophosphamide
  • Followed by maintenace therapy:
    • Glucocorticoids + methotrexate
  • Plasmapheresis for fulminant lung disease
28
Q

Describe Takayasu arteritis?

A
  • Aorta and its major branches
  • Granulomatous inflammation of vessel wall leading to occlusion
  • Claudication, fever, arthralgia, weight loss
  • Tx:
    • High dose glucocorticoids + immunosuppressants
29
Q

Describe Kawasaki disease?

aka Mucocutaneous lymph node syndrome

A
  • Systemic vasculitis in children <5 years old
    • May eventually affect the coronary vessels
  • Fever, generliased rash, enlarged lymph nodes, strawberry tongue
  • Inflamed oral mucosa and conjunctiva
  • Tx:
    • High doses of Aspirin + IV immunoglobulin
30
Q

Describe Polyarteritis nodosa?

A
  • HBV is an important risk factor
  • Fever, myalgia, arthralgia, weight loss
  • Palpable purpura, ulceration, livedo reticularis
31
Q

What is pictured here?

A
  • Palpable purpura of systemic vasculitis
    • aka Polyarteritis nodosa
32
Q

What is pictured here?

A

Livedo reticularis

33
Q

How is Polyarteritis nodosa diagnosed?

A
  • MRI
    • Multiple aneurysms
    • Smooth narrowing of mesenteric, hepatic or renal systems
  • Muscle / Sural nerve biopsy
34
Q

Describe the treatment of polyarteritis nodosa?

A
  • High-dose glucocorticoids + cyclophosphamide
  • Maintenance therapy
    • Glucocorticoid + methotrexate
35
Q

What is another name for Churg-Strauss syndrome?

A

Eosinophilic granulomatosis with polyangitis

36
Q

Describe Eosinophilic granulomatosis with polyangitis?

A
  • Small vessel vasculitis with eosinophilia
  • Prodrome can occur for several years before onset
    • Allergic rhinitis, nasal polyposis, late-onset asthma​
37
Q

Describe the acute presentation of Eosinophilic granulomatosis with polyangitis?

A
  • Triad of acute presentation:
    • Purpura
    • Asymmetric mononeuritis multiplex
    • Eosinophilia
38
Q

Describe the diagnosis and management of Eosinophilic granulomatosis with polyangitis?

A
  • Vessel biopsy
    • Small vessel vasculitis
    • Eosinophilic infiltration of vessel wall
  • Management
    • High dose glucocorticoids + cyclophosphamide
    • Maintenance therapy
39
Q

Describe Behcet’s disease?

A
  • Vasculitis of unknown cause
  • Oral ulcers
    • Deep and multiple
  • Genital ulcers
  • Erythema nodosum, acneiform lesions
  • Neurological involvement can occur
40
Q

Describe the management of Behcet’s disease?

A
  • Topical glucocorticoid
    • Prednisolone mouthwash
  • Erythema nodosum, arthralgia
    • Colchicine
  • Resistant oral and genital ulcers
    • Thalidomide

Y4: Rheumatology (19 decks)

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