Rheuma: Management of Vasculitis Flashcards
characteristics of vasculitic disorders
- vascular inflammation
- vascular necrosis
- varying degrees of target-organ ischaemia
types of vasculitic disorders
- primary systemic necrostising vasculitides
- vasculitis associated with rheumatoid disorders
- vasculitis-like syndromes
types of primary systemic necrotising vasculitides
- small vessel
- medium vessel
- large vessel
how can SNV be diagnosed?
- presence of ANCA
management of SNV
- 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
examples of small-vessel disease
- Henoch-Schonlein purpura
- Hypersensitivity vasculitis
- Cryoglobulinaemic vasculitis
- Kawasaki’s disease
examples of medium-vessel disease
- microscopic polyangiitis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
examples of large vessel disease
- giant cell arteritis
- Takayasu’s arteritis
when would you suspect vasculitis
- persistently high ESR and CRP
- arterial ulcers with good arterial pulses
- skin ulcers which are non-responsive to conventional treatment
- systemically ill patient
what is the commonest ocular manifestation of systemic vasculitis
episcleritis
causes of episcleritis
- polyarteritis nodosa
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
- microscopic polyangiitis
- rheumatoid vasculitis
- Takayasu’s arteritis
causes of nodules in a context of vasculitis
- rheumatoid nodules
- cutaneous extravascular necrotising granulomas
- SLE
- lymphoma
- takayasu’s arteritis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
principles of treatment
- accurate diagnosis
- check when disease activity has been controlled
- recognise resistant disease
- need for urgency in diagnosis
why is accurate diagnostics problematic?
- 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
histology in polyarteritis nodosa
- 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
types of symptoms in polyangiitis nodosa
- systemic
- renal
- arthritis/myalgia
- cutaneous
- neurological
- abdominal
systemic symptoms in polyangiitis nodosa
- fever
- myalgia
- weight loss
renal symptoms in polyangiitis nodosa
- haematuria
- loin pain
- acute/chronic renal failure
- hypertension
cutaneous symptoms in polyangiitis nodosa
- necrotic patches
- gangrene
- livedo reticularis
neurological symptoms in polyangiitis nodosa
- mononeuritis multiplex
- symmetrical sensori-motor neuropathy
abdominal symptoms in polyangiitis nodosa
- pain
- organ infarction
define granulomatous polyangiitis
systemic disease characterised by necrotising granulomatous inflammation of upper and lower respiratory tract
which systems are involved in granulomatous polyangiitis
- pulmonary
- sinus/nasopharynx
- renal
- rheumatic
- cutaneous (vasculitic purpura)
- ophthalmic
- neurological
typical feature of GP
saddle nose deformity due to destruction of nasal cartilage
aim of treatment
- induce remission
- maintain remission
- monitor disease activity and drug toxicity
- monitor for disease recurrence
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
how to induce remission?
methylprednisolone and cyclophosphamide
- switch to methotrexate or azathioprine if in remission
what are the symptoms of regiment toxicity?
- nausea
- alopecia
- neutropenia
- infertility
- haemorrhagic cystitis
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
indications for methylprednisolone
- cardiac arrhythmias
- fluid overload
- severe hyperglycaemia
- avascular necrosis of the hip
- severe osteoporosis
- hypertension
what is positive ANCA associated with?
- granulomatosis with polyangiitis
- microscopic polyangiitis
- eosinophilic granulomatosis withh polyangiitis
- renal limited vasculitis
- certain drug-induced vasculitis syndromes
is a positive ANCA result reliable?
no, it cannot predict subsequent flare-ups of disease
which systems are associated with PR3-ANCA
- ENT
- respiratory
which systems are associated with MPO-ANCA?
- renal
- cutaneous
- pulmonary
does a negative ANCA exclude GPA
no, have to check with further investigations to be completely sure
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)
do ANCA values predict disease flareups
no
risks of therapies for relapsed ANCA-associated vasculitis
- severe infections
- cystitis
- bladder cancer
- lung fibrosis
- death
differential diagnosis for vasculitis
- systemic infections
- malignancy
- inflammatory/infiltrative diseases
- non-inflammatory vascular diseases
what is the recommended time period to stop cyclophosphamides
after 3-6 months
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
what is the best predictor for severity of renal vasculitis
number of normal glomeruli on renal biopsy
if cyclophosphamide does not work, what should you consider before getting a new drug?
you are not dealing with a healthy bone marrow here
what should you check before giving azathioprine?
thiopurine methyltransferase
what is mepex
methylprednisolone and plasma exchange
inclusion criteria for mepex?
- new diagnosis
- serum creatinine >500
- ANCA positive
- biopsy showing necrotising or cresentic GN
