Vasculitis Screen and Autoimmune Screen

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Vasculitis ScreenandAutoimmune Screen

• Theo de Malmanche

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Put this in your search engine

• RCPAmanual

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Your lab

• Can put HAPS in your search engine

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Or ring 14000 and ask for a chat with someone who
knows about (whatever it is)..
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The brief
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• TERM 2 PATHOLOGY TESTS ORDERING AND
  INTERPRETATION
• Should ideally include, but not be limited to, a
  discussion of the following points
• 1. Local systems for test ordering
• 2. Rational test ordering, with consideration of
• a. Costs
• b. Clinical benefit to the patient
• 3. Common order sets - what they include and
  their interpretation
• a. Vasculitic/autoimmune screen
• b. Coagulopathic screen
• c. Haemolysis screen
• d. Hepatitis screen
• e. Atypical pneumonia serology
• 4. Basic interpretation of common tests when to
  be concerned
• a. Full blood count
• b. Electrolytes (EUC and CMP)
• c. Coagulation profile
• d. Liver function tests

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1. Local systems for test ordering

• Prof Kellerman also discussing this

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Who really cares about the forms?

• Request forms are a form of consultation
• Consequence of incomplete or inaccurate request
  forms are traceable
• These forms are required by law to be retained
• When reviewing results, I am often guided by the
  level of detail.
• If you do not care about the patients result,
  then you must have a low pre-test probability

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Who reads the forms?

• At least
• The blood collector
• Specimen reception staff
• Data entry staff
• Lab scientific staff to confirm test selection
• Lab scientific staff interpretation of result
• Pathologist when validating results

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• 2. Rational test ordering, with consideration of
• a. Costs
• b. Clinical benefit to the patient

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Example of cost to taxpayer of testing

• (values are 85 MBS, i.e done in public hospital
  lab, on inpatients)

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Competing pressures

• Cost of bedstay in NSW hospitals 500 per day
• A delayed diagnosis can be expensive
• Directly and indirectly
• Every request form is a new venepuncture
• Can it be added on?
• Can it wait until Monday or Tuesday?
• Unnecessary testing leads to more testing.
• This may delay discharge

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• 3. Common order sets - what they include and
  their interpretation
• a. Vasculitic/autoimmune screen
• b. Coagulopathic screen
• c. Haemolysis screen
• d. Hepatitis screen
• e. Atypical pneumonia serology
• Haematology talk in next term

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Vasculitis ScreenandAutoimmune Screen

• 15 min on the tests you should not order
• Theo de Malmanche

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• First - the symptoms
• Second signs
• Third the provisional diagnosis
• Then Fourth the test

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For immune disorders

• Systems review eg. head to toe
• What are the symptoms
• Are there features suggesting inflammation
  without infection?
• Is it inflammatory?
• Is it organ threatening?
• Do we need to biopsy?
• Then, which tests might support the provisional
  diagnosis?
• What can we use as a marker of inflammation?
• Number of joints, extent of rash, duration of
  stiffness, frequency or duration of episodes

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If in doubt Hold Serum

• A low level / borderline / indeterminate result
  of questionable significance will cause more
  trouble than it is worth
• eg. B27 pos now what? ?XR Lspine?
• Serological tests can be added on at a later date
  to stored serum
• Tests like B27, DQ2/DQ8, HFE testing are the same
  throughout life, no urgency

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Vasculitis what it is (clinically)

• Features include
• Features of inflammation
• Features of ischaemia
• Associated specific features

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Vasculitis what it is (clinically)

• Often some features of inflammation without
  distinct focus
• Eg. Fatigue, myalgia, sweats, weight loss
• May be reflected in inflammatory markers eg. CRP
• Tissue ischaemia
• Which may or may not lead to organ dysfunction

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Symptoms vary with organ(s) affected
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Redundancy

• Picking off blood supply to some organs will
  only have noticeable effect on function when the
  functioning mass is sufficiently reduced
• Kidney, lung
• Other organs will be symptomatic as soon as blood
  flow is reduced
• Brain

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Collaterals - chronicity

• Collaterals develop if the reduction of blood
  flow has been a gradual process
• Tissue ischaemia leads to growth factor release
• This leads to new blood vessel formation

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Symptom complexes - ischaemia

• Skin involvement
• Rash, especially pupurae
• Cerebral involvement
• CNS dysfunction, can be like a stroke
• Renal involvement
• Renal failure, an active sediment
• Renal failure -gt hypertension
• Mesenteric involvement
• Pain after eating, bleeding into bowel, gut
  infarction, scrotal pain
• Muscle involvement
• Muscle pain and tenderness, cramping
• Scalp -gt headaches
• Tongue and mastoid mm. cramping classic in GCA
• Retinal involvement
• Visual loss, usually permanent
• Heart involvement
• Acute coronary syndrome (occlusion) or angina
  (stenoses)
• Peripheral nerve involvement
• Peripheral neuropathy, can be mononeuritis or
  symmetrical

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Specific features

• These are often hard to find
• Detectable vasculitis
• Tenderness and swelling of the vessels
• Esp temporal arteries in GCA, but also carotids
  and subclavians
• Bruits but more due to something other than
  vasculitis
• Eg axillary in GCA
• Granulomatous features in granulomatous
  vasculitis
• Eg. Granulomatous sinusitis, lung granulomata
• Asthma in Churg-Strauss Syndrome
• Precedent pharyngitis in Henoch-Schonlein
  Purpurae

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Vasculitis what it is not

• Thrombosis without inflammation
• Thromboembolic, cholesterol, plaque rupture
• Vasculopathy without inflammation
• Atherosclerosis, fibromuscular dysplasia,
  congophilic angiopathy, cystic medial dysplasia,
• The above are more common than vasculitis
• Also
• Vasospasm
• Organ dysfunction without vasculitis
• Cerebritis
• These do not improve with immunosuppression

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Suggested approach

• Which organs are involved
• Systems review (history)
• General examination
• Urinanalysis
• Severity
• Are any organs threatened?
• Evidence of inflammation?
• Consider confirmation of diagnosis
• If organ threatening treat with steroids, chase
  diagnosis
• If not organ threatening chase diagnosis

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Diagnosis of vasculitis

• Gold standard
• Biopsy showing necrotising vasculitis
• Inflammation with disruption of vascular
  structures
• Surrogates
• Imaging demonstrating vascular damage
• Evidence of this being inflammatory
• Classic syndrome typical of known vasculitis
• Exclusion of other likely causes

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Levels of confidence

• Biopsy with typical findings
• Biopsy with consistent findings
• Conventional angiogram with classic findings
• CT angiogram with classic findings
• Magnetic resonance angiography
• Nuclear scans
• Clinical syndrome

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Standard of proof

• Is your diagnosis
• Beyond reasonable doubt?
• On the balance of probabilities?
• On reasonable suspicion?
• On justifiable grounds?
• When the patient has an adverse effect, how
  justified was your decision to treat?
• Consider severity (urgency) and confidence of
  diagnosis

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Treatment approach

• Halt progression / Induce remission
• Steroids
• Consolidate remission
• Steroids and ?cyclophosphamide
• Maintainence (prevent relapse)
• Steroid sparers eg. Methotrexate, azathioprine

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Natural history

• Bad (will lead to organ failure /- death)
• ANCA-associated vasculitides
• Granulomatous vasculitis (nee WG), Churg Strauss
  Syndrome, microscopic polyangiitis
• Polyarteritis Nodosa, Temporal arteritis
• Meningococcaemia
• Dengue Haemorrhagic fever
• Not so bad
• Leucocytoclastic vasculitis
• Eg due to medication, viral infection, Sjogrens
  Syndrome
• Henoch-Schonlein Purpurae
• Rarely needs therapy

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The vasculitis screen

• Look for evidence of ischaemia
• Systems review
• Look for evidence of inflammation
• Examination, including UA
• Consider biopsy
• One sample needs to be not fixed (not formalin)
• Consider supportive evidence

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Which have helpful blood tests?

• No
• ANCA
• ANCA
• No
• No
• No
• No
• No

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• If in doubt, hold serum, or ask someone
• Rubbish in, rubbish out