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Medicine II > Rheuma: Management of Vasculitis > Flashcards

Rheuma: Management of Vasculitis Flashcards

1
Q

characteristics of vasculitic disorders

A
  • vascular inflammation
  • vascular necrosis
  • varying degrees of target-organ ischaemia
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2
Q

types of vasculitic disorders

A
  • primary systemic necrostising vasculitides
  • vasculitis associated with rheumatoid disorders
  • vasculitis-like syndromes
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3
Q

types of primary systemic necrotising vasculitides

A
  • small vessel
  • medium vessel
  • large vessel
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4
Q

how can SNV be diagnosed?

A
  • presence of ANCA
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5
Q

management of SNV

A
  • tailored depending on the stage of the disease and the specific diagnosis of the patient
  • imp is given to the history of symptoms
  • assess the patient to identify the extent and activity of the disease
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6
Q

examples of small-vessel disease

A
  • Henoch-Schonlein purpura
  • Hypersensitivity vasculitis
  • Cryoglobulinaemic vasculitis
  • Kawasaki’s disease
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7
Q

examples of medium-vessel disease

A
  • microscopic polyangiitis
  • granulomatosis with polyangiitis
  • eosinophilic granulomatosis with polyangiitis
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8
Q

examples of large vessel disease

A
  • giant cell arteritis

- Takayasu’s arteritis

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9
Q

when would you suspect vasculitis

A
  • persistently high ESR and CRP
  • arterial ulcers with good arterial pulses
  • skin ulcers which are non-responsive to conventional treatment
  • systemically ill patient
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10
Q

what is the commonest ocular manifestation of systemic vasculitis

A

episcleritis

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11
Q

causes of episcleritis

A
  • polyarteritis nodosa
  • granulomatosis with polyangiitis
  • eosinophilic granulomatosis with polyangiitis
  • microscopic polyangiitis
  • rheumatoid vasculitis
  • Takayasu’s arteritis
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12
Q

causes of nodules in a context of vasculitis

A
  • rheumatoid nodules
  • cutaneous extravascular necrotising granulomas
  • SLE
  • lymphoma
  • takayasu’s arteritis
  • granulomatosis with polyangiitis
  • eosinophilic granulomatosis with polyangiitis
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13
Q

principles of treatment

A
  • accurate diagnosis
  • check when disease activity has been controlled
  • recognise resistant disease
  • need for urgency in diagnosis
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14
Q

why is accurate diagnostics problematic?

A
  • difficult to differentiate between types of vasculitis
  • lack of highly sensitive and specific non-invasive diagnostic tests
  • low sensitivity and test efficiency of invasive tests
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15
Q

histology in polyarteritis nodosa

A
  • aneurysms within small vessels
  • fibrinoid necrosis in its wall
  • inflammatory infiltration surrounding and infiltrating artery and aneurysm
  • late stage: obliteration of the lumen
  • complete disruption of internal elastic lamina
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16
Q

types of symptoms in polyangiitis nodosa

A
  • systemic
  • renal
  • arthritis/myalgia
  • cutaneous
  • neurological
  • abdominal
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17
Q

systemic symptoms in polyangiitis nodosa

A
  • fever
  • myalgia
  • weight loss
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18
Q

renal symptoms in polyangiitis nodosa

A
  • haematuria
  • loin pain
  • acute/chronic renal failure
  • hypertension
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19
Q

cutaneous symptoms in polyangiitis nodosa

A
  • necrotic patches
  • gangrene
  • livedo reticularis
20
Q

neurological symptoms in polyangiitis nodosa

A
  • mononeuritis multiplex

- symmetrical sensori-motor neuropathy

21
Q

abdominal symptoms in polyangiitis nodosa

A
  • pain

- organ infarction

22
Q

define granulomatous polyangiitis

A

systemic disease characterised by necrotising granulomatous inflammation of upper and lower respiratory tract

23
Q

which systems are involved in granulomatous polyangiitis

A
  • pulmonary
  • sinus/nasopharynx
  • renal
  • rheumatic
  • cutaneous (vasculitic purpura)
  • ophthalmic
  • neurological
24
Q

typical feature of GP

A

saddle nose deformity due to destruction of nasal cartilage

25
Q

aim of treatment

A
  • induce remission
  • maintain remission
  • monitor disease activity and drug toxicity
  • monitor for disease recurrence
26
Q

why would you maintain remission?

A
  • maintain control of disease activity
  • prevent disease recurrence following reduction or discontinuation of medications
  • minimise the risks of drug toxicity
27
Q

how to induce remission?

A

methylprednisolone and cyclophosphamide

- switch to methotrexate or azathioprine if in remission

28
Q

what are the symptoms of regiment toxicity?

A
  • nausea
  • alopecia
  • neutropenia
  • infertility
  • haemorrhagic cystitis
29
Q

indications for cyclophosphamide

A
  • mononeuritis multiplex
  • CNS disease
  • RPGN
  • clinically-evident mesenteric vasculitis
  • cardiac involvement
  • alveolar haemorrhage
  • life-threatening features
  • cutaneous ulcers that will not heal
  • excessive steroid toxicity
30
Q

indications for methylprednisolone

A
  • cardiac arrhythmias
  • fluid overload
  • severe hyperglycaemia
  • avascular necrosis of the hip
  • severe osteoporosis
  • hypertension
31
Q

what is positive ANCA associated with?

A
  • granulomatosis with polyangiitis
  • microscopic polyangiitis
  • eosinophilic granulomatosis withh polyangiitis
  • renal limited vasculitis
  • certain drug-induced vasculitis syndromes
32
Q

is a positive ANCA result reliable?

A

no, it cannot predict subsequent flare-ups of disease

33
Q

which systems are associated with PR3-ANCA

A
  • ENT

- respiratory

34
Q

which systems are associated with MPO-ANCA?

A
  • renal
  • cutaneous
  • pulmonary
35
Q

does a negative ANCA exclude GPA

A

no, have to check with further investigations to be completely sure

36
Q

what does the accuracy of a positive ANCA result depend on?

A

depends on the severity of the clinical presentation (if severe, it has a higher predictive value)

37
Q

do ANCA values predict disease flareups

A

no

38
Q

risks of therapies for relapsed ANCA-associated vasculitis

A
  • severe infections
  • cystitis
  • bladder cancer
  • lung fibrosis
  • death
39
Q

differential diagnosis for vasculitis

A
  • systemic infections
  • malignancy
  • inflammatory/infiltrative diseases
  • non-inflammatory vascular diseases
40
Q

what is the recommended time period to stop cyclophosphamides

A

after 3-6 months

41
Q

if the diagnosis is correct and treatment has lead to initial improvement followed by deterioration

A
  • has a secondary infection supervened
  • has therapy been tapered too rapidly
  • is deterioration due to drug toxicity
42
Q

what is the best predictor for severity of renal vasculitis

A

number of normal glomeruli on renal biopsy

43
Q

if cyclophosphamide does not work, what should you consider before getting a new drug?

A

you are not dealing with a healthy bone marrow here

44
Q

what should you check before giving azathioprine?

A

thiopurine methyltransferase

45
Q

what is mepex

A

methylprednisolone and plasma exchange

46
Q

inclusion criteria for mepex?

A
  • new diagnosis
  • serum creatinine >500
  • ANCA positive
  • biopsy showing necrotising or cresentic GN

Medicine II (16 decks)

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