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Week 11- Musculoskeletal > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A
  • Inflammation of the blood vessels

* umbrella term for a group of autoimmune conditions called vasculitides

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2
Q

Name the different categories of vasculitis

A
  • Large vessel vasculitis
  • Medium vessel vasculitis
  • ANCA-associated small vessel vasculitis
  • Immune complex small vessel vasculitis
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3
Q

What are the large vessel vasculitis types?

A
  • Takayasu arteritis

* Giant cell arteritis

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4
Q

What are the medium vessel vasculitis types?

A
  • Polyarteritis nodosa

* Kawasaki disease

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5
Q

What are the ANCA-associated small vessel vasculitis types?

A
  • Microscopic polyangiitis
  • Wegener’s: Granulomatosis with polyangiitis
  • Churg-Strauss: Eosinophilic granulomatosis with polyangiitis
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6
Q

What are the immune complex small vessel vasculitis types?

A
  • Cryoglobulinemic vasculitis
  • IgA vasculitis (Henoch schonlein purpura)
  • Hypocomplementemic urticarial vasculitis
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7
Q

What is giant cell arteritis?

A
  • Large vessel vasculitis

* Systemic vasculitis that affects the aorta and its major branches

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8
Q

What is the clinical presentation of giant cell arteritis?

A
  • Headache: subacute onset, constant, temporal with tenderness, little relief with analgesics
  • Visual symptoms
  • Jaw claudication (sore to eat)
  • Polymyalgia rheumatica symptoms (stiffness in the shoulder and pelvic girdle)
  • Constitutional upset: weight loss, malaise, night sweats
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9
Q

What are the complications of giant cell arteritis?

A
  • Visual loss: acute ischaemic neuropathy: sudden painless loss of vision, occasionally preceded by amaurosis fungax
  • Large vessel vasculitis: vascular stenosis and aneurysm
  • CVA: obstruction or occlusion of internal carotid artery or vertebral arteries
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10
Q

How do you diagnose giant cell arteritis?

A
  • Clinical presentation: symptoms, age, no alternate diagnosis, associated features
  • Clinical examination findings: temporal artery asymmetry, thickening and loss of pulsatility (tenderness)
  • acute phase response: ESR/CRP
  • Further investigation: biopsy
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11
Q

Describe the correlation between age and temporal arteritis

A
  • Rare below 50

* Peak incidence 70-79

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12
Q

Explain what would confirm giant cell arteritis on a biopsy of the temporal artery

A
  • Interruption of the internal elastic laminae
  • Mononuclear inflammatory cell infiltrate within vessel wall
  • Multinucleated giant cells in 40-60% of cases
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13
Q

If biopsy of the temporal artery are not available, what other investigations can be used to investigate giant cell arteritis?

A
  • Temporal artery USS
  • MRI
  • PET CT
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14
Q

What is the treatment of giant cell arteritis?

A

•Prednisolone 60mg for 1 month, taper to 15mg by 12 weeks
•Aim to discontinue corticosteroids by 12-18 months •Corticosteroid sparing therapy in patients with disease relapse:
- mycophenolate mofetil
- methotrexate
- tocilizumab

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15
Q

What are the causes of a cutaneous small vessel vasculitis?

A
  • Can be idiopathic
  • drugs
  • infection: HCV, HBV, gonococcus, meningococcus, HIV
  • Secondary RA/CTD/PBC/UC
  • Malignancy: haematological > solid organ
  • Manifestation of small/medium vessel ANCA associated vasculitis
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16
Q

Describe the common patient characteristics of those with henoch-schonlein purpura

A
  • children 2-11 years or adults mean age 43
  • Male>females
  • Often after an infection
17
Q

What are the clinical signs and symptoms of henoch schonlein purpura?

A
  • Classic purpuric rash: buttocks and thighs>lower legs
  • Urticarial rash, confluent petechiae, eccymoses, ulcers
  • Arthralgia/arthritis in the lower limb in 75%
18
Q

What are the complications of henoch schonlein purpura?

A
  • GI: pain, bleeding, diarrhoea, rarely intussusception
  • Renal: IgA nephropathy
  • Urological: orchitis
19
Q

Describe an overview of the diagnosis of henoch schonlein purpura

A
  • Exclude secondary causes: history, examination, immunology, virology
  • Assess extent if involvement: urinalysis/urine PCR
20
Q

Describe the management and prognosis of Henoch schonlein purpura

A
  • Often self limiting and no treatment required (up to 16 weeks)
  • Corticosteroids for certain complications: testicualr torsion, GI disease, arthritis
  • Relapses in 5-10%, this tends to occur within 12 months
21
Q

What is it important to exclude in a suspected henoch schonlein prupura?

A
  • Virus
  • Malignancy
  • other autoimmune conditions
  • drugs
22
Q

Describe the pathology of granulomatosis with polyangiitis

A
  • Granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract
  • Often pauci-immune glomerulonephritis
23
Q

explain the triad of disease in glomerulomatosis polyangiitis

A
  • upper airway/ENT: rhinitis, chronic sinusitis, chronic otitis media, saddle nose deformity, nasal septum perforation
  • Renal: pouch-immune glomerulonephritis
  • lower respiratory: parenchymal nodules and cavitation, alveolar haemorrhage
24
Q

What are the constitutional symptoms in granulomatosis with polyangiitis?

A
  • fatigue
  • Weight loss
  • fever/sweats
  • Myalgia/arthralgia
  • Failure to thrive in elderly
25
Q

What are the organ specific features of granulomatosis with polyangiitis?

A
  • Oral cavity: ulcerations
  • Lungs: cavities, bleeds, lung infiltrates
  • skin: nodules on elbow, pupura
  • Eyes: pseudotumours, conjunctivitis
  • Nose: stuffiness, nosebleeds, saddle nose
  • Heart: pericarditis
  • Kidneys: glomerulonephritis
26
Q

What is ANCA?

A
  • Autoantibodies directed against the cytoplasmic constituents of neutrophils and monocytes
  • Two types: cANCA (cytyoplasmic) and pANCA (periphery of neutrophil)
27
Q

What are the means of testing for ANCA?

A
  • Indirect immunofluorescence

* ELISA for PR3/MPO

28
Q

What is PR3 ANCA an indicator of?

A

Granulomatosis with polyangiitis

29
Q

What is MPO ANCA indicative of?

A

Microscopic polyangiitis

30
Q

What does a high ANCA mean?

A

That relapse is more likely

31
Q

What can ANCA testing be used for?

A
  • Diagnosis
  • Prognostic info
  • Assessing a response to treatment
  • Monitoring for early signs of relapse
32
Q

Describe the treatment of ANCA associated small vessel vasculitis

A

1) remission induction for 3-6 months: switch of vasculitis activity: prednisolone and cyclophosphamide or rituximab or methotrexate (milder) or mycophenolate (milder)
2) Remission maintenance 2+ years: prevent relapse: azathioprine or methotrexate or rituximab

Week 11- Musculoskeletal (11 decks)

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