Rheuma: Management of Vasculitis Flashcards
1
Q
characteristics of vasculitic disorders
A
- vascular inflammation
- vascular necrosis
- varying degrees of target-organ ischaemia
2
Q
types of vasculitic disorders
A
- primary systemic necrostising vasculitides
- vasculitis associated with rheumatoid disorders
- vasculitis-like syndromes
3
Q
types of primary systemic necrotising vasculitides
A
- small vessel
- medium vessel
- large vessel
4
Q
how can SNV be diagnosed?
A
- presence of ANCA
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
6
Q
examples of small-vessel disease
A
- Henoch-Schonlein purpura
- Hypersensitivity vasculitis
- Cryoglobulinaemic vasculitis
- Kawasaki’s disease
7
Q
examples of medium-vessel disease
A
- microscopic polyangiitis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
8
Q
examples of large vessel disease
A
- giant cell arteritis
- Takayasu’s arteritis
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
10
Q
what is the commonest ocular manifestation of systemic vasculitis
A
episcleritis
11
Q
causes of episcleritis
A
- polyarteritis nodosa
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
- microscopic polyangiitis
- rheumatoid vasculitis
- Takayasu’s arteritis
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
13
Q
principles of treatment
A
- accurate diagnosis
- check when disease activity has been controlled
- recognise resistant disease
- need for urgency in diagnosis
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
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
16
Q
types of symptoms in polyangiitis nodosa
A
- systemic
- renal
- arthritis/myalgia
- cutaneous
- neurological
- abdominal
17
Q
systemic symptoms in polyangiitis nodosa
A
- fever
- myalgia
- weight loss
18
Q
renal symptoms in polyangiitis nodosa
A
- haematuria
- loin pain
- acute/chronic renal failure
- hypertension
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
aim of treatment
- induce remission
- maintain remission
- monitor disease activity and drug toxicity
- monitor for disease recurrence
26
why would you maintain remission?
- maintain control of disease activity
- prevent disease recurrence following reduction or discontinuation of medications
- minimise the risks of drug toxicity
27
how to induce remission?
methylprednisolone and cyclophosphamide
| - switch to methotrexate or azathioprine if in remission
28
what are the symptoms of regiment toxicity?
- nausea
- alopecia
- neutropenia
- infertility
- haemorrhagic cystitis
29
indications for cyclophosphamide
- 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
indications for methylprednisolone
- cardiac arrhythmias
- fluid overload
- severe hyperglycaemia
- avascular necrosis of the hip
- severe osteoporosis
- hypertension
31
what is positive ANCA associated with?
- granulomatosis with polyangiitis
- microscopic polyangiitis
- eosinophilic granulomatosis withh polyangiitis
- renal limited vasculitis
- certain drug-induced vasculitis syndromes
32
is a positive ANCA result reliable?
no, it cannot predict subsequent flare-ups of disease
33
which systems are associated with PR3-ANCA
- ENT
| - respiratory
34
which systems are associated with MPO-ANCA?
- renal
- cutaneous
- pulmonary
35
does a negative ANCA exclude GPA
no, have to check with further investigations to be completely sure
36
what does the accuracy of a positive ANCA result depend on?
depends on the severity of the clinical presentation (if severe, it has a higher predictive value)
37
do ANCA values predict disease flareups
no
38
risks of therapies for relapsed ANCA-associated vasculitis
- severe infections
- cystitis
- bladder cancer
- lung fibrosis
- death
39
differential diagnosis for vasculitis
- systemic infections
- malignancy
- inflammatory/infiltrative diseases
- non-inflammatory vascular diseases
40
what is the recommended time period to stop cyclophosphamides
after 3-6 months
41
if the diagnosis is correct and treatment has lead to initial improvement followed by deterioration
- has a secondary infection supervened
- has therapy been tapered too rapidly
- is deterioration due to drug toxicity
42
what is the best predictor for severity of renal vasculitis
number of normal glomeruli on renal biopsy
43
if cyclophosphamide does not work, what should you consider before getting a new drug?
you are not dealing with a healthy bone marrow here
44
what should you check before giving azathioprine?
thiopurine methyltransferase
45
what is mepex
methylprednisolone and plasma exchange
46
inclusion criteria for mepex?
- new diagnosis
- serum creatinine >500
- ANCA positive
- biopsy showing necrotising or cresentic GN
