DoH IVIG Workshop Update, neurology usage and outcomes measurement Dr Michael Lunn National Hospital for Neurology and Neurosurgery Queen Square, London.
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DoH IVIG WorkshopUpdate, neurology usage and
outcomes measurement
Dr Michael LunnNational Hospital for Neurology and Neurosurgery
Queen Square, London WC1N 3BG
Outline
• Neurology IVIG usage update• Neurology in 2011 Guidelines Update• Measuring outcomes
– Update guidelines– Measuring outcomes in clinical practice– What’s wrong with what we are using– How can we make it better?
IVIG in Neurology Usage update
Diagnosis Grams Used % Of Total
Chronic inflammatory demyelinating polyradiculoneuropathy 473649.1 48.33%
Multifocal motor neuropathy 224303.7 22.89%
Guillain–Barré syndrome 97814.5 9.98%
Myasthenia gravis 69221.5 7.06%
Other (Neurology) 45410 4.63%
Other (Other) 11697.5 1.19%
Paraprotein-associated demyelinating neuropathy (IgM) 11642.5 1.19%
Stiff person syndrome 10255 1.05%
Paraprotein-associated demyelinating neuropathy (IgG or IgA) 7969 0.81%
Paraneoplastic disorders 5196.5 0.53%
Acute disseminated encephalomyelitis 3902.5 0.40%
Polymyositis 3580 0.37%
Lambert Eaton myasthenic syndrome 3215 0.33%
Vasculitic neuropathy 2735 0.28%
Rasmussen syndrome 2215 0.23%
Neuromyotonia 1380 0.14%
Multiple sclerosis 1330 0.14%
Critical illness neuropathy 1145 0.12%
CNS vasculitis 730 0.07%
Inclusion body myositis 600 0.06%
Bickerstaff's brain stem encephalitis 510 0.05%
Chronic fatigue syndrome 442.5 0.05%
Dermatomyositis 370 0.04%
Autism 360 0.04%
Acute idiopathic dysautonomia 175 0.02%
Autoimmune diabetic proximal neuropathy 110 0.01%
Neurology Infusions by Diagnosis 01/04/2010 - 31/03/2011
94.89%
Diagnosis Patients % Of Total
Chronic inflammatory demyelinating polyradiculoneuropathy 854 34.24%Guillain–Barré syndrome 633 25.38%Multifocal motor neuropathy 353 14.15%Myasthenia gravis 296 11.87%Other (Neurology) 93 3.73%Other (Other) 62 2.49%Paraneoplastic disorders 27 1.08%Paraprotein-associated demyelinating neuropathy (IgM) 27 1.08%Stiff person syndrome 27 1.08%Acute disseminated encephalomyelitis 21 0.84%Paraprotein-associated demyelinating neuropathy (IgG or IgA) 16 0.64%Polymyositis 15 0.60%Rasmussen syndrome 10 0.40%Vasculitic neuropathy 10 0.40%Lambert Eaton myasthenic syndrome 9 0.36%Multiple sclerosis 8 0.32%Neuromyotonia 6 0.24%CNS vasculitis 6 0.24%Critical illness neuropathy 5 0.20%Bickerstaff's brain stem encephalitis 5 0.20%Chronic fatigue syndrome 3 0.12%Inclusion body myositis 3 0.12%Intractable childhood epilepsy 1 0.04%Dermatomyositis 1 0.04%Acute idiopathic dysautonomia 1 0.04%Autism 1 0.04%Autoimmune diabetic proximal neuropathy 1 0.04%
Neurology Patients by Diagnosis 01/04/2010 - 31/03/2011
How inclusive is the database data now?
• In 2009 PASA estimated only 60% data capture
• GBS cases– 1.2-1.5 per 100000– 720-900 cases– 60% require Rx
• In 2009 260 GBS pts in database• Thus ?48 – 60% capture
• In 2010 – 633 cases ?almost complete – 90%?
90.7% capture
Guidelines update
• This update did not review all of the Second Edition Guidelines content, but limited its focus to three key areas
– defining selection criteria for appropriate use;– efficacy outcomes to assess treatment success;– reassignment of existing indications /inclusion of
new indications.
Reassignment of existing indications /inclusion of new
indications.
‘Grey’ diagnosis usage 2009Diagnosis
RedBlue: long term
Blue: short term
Grey:
long term
Grey:
short
term
Exceptionalit
y
Not appro
ved (reject
ed)
Awaiting
panel decisi
on
Panel decision
not recorde
d
Acute disseminated encephalomyelitis
2 8 1
Acute idiopathic dysautonomia
1
Bickerstaff's brain stem encephalitis
1 3
Intractable childhood epilepsy 1
Paraneoplastic disorders 1 1 1 4
Polymyositis 2 2 2 10 5 1 2
Vasculitic neuropathy 1 1
Other (Neurology) 7 20 4 14 23 6 1 13 2
2009
• 24 patients with polymyositis• Some life threatening• >50% approved• Polymyositis likely to be immune mediated
• IBM (previously black)– 2 cases only approved as exceptionality– Not infrequently ‘inflammatory’
Myositis criteria
• Diagnosis of myositis by a neurologist, rheumatologist, immunologist of: – Patients with PM or DM who have significant muscle
weakness; – OR Dysphagia and have not responded to
corticosteroids and other immunosuppressive agents; – OR Patients with IBM who have dysphagia affecting
nutrition (NOT patients with rapidly progressive IBM)
• Outcomes and test dosage schedule suggested
Grey indications - changes
• Immune-mediated disorders with limited evidence of immunoglobulin efficacy
• Presumed immune-mediated disorders with little or no evidence of efficacy
Immune-mediated disorders with limited evidence of immunoglobulin efficacy
• Acute disseminated encephalomyelitis
• Autoimmune encephalitis (including NMDA and VGKC antibodies, among others)
• Cerebral infarction with antiphospholipid antibodies
• Chronic regional pain syndrome
• CNS vasculitis
• Intractable childhood epilepsy
• Neuromyotonia
• Opsoclonus Myoclonus
Immune-mediated disorders with limited evidence of immunoglobulin efficacy
• Acute disseminated encephalomyelitis
• Autoimmune encephalitis (including NMDA and VGKC antibodies, among others)
• Cerebral infarction with antiphospholipid antibodies
• Chronic regional pain syndrome
• CNS vasculitis
• Intractable childhood epilepsy
• Neuromyotonia
• Opsoclonus Myoclonus
• Autoimmune encephalitis (including NMDA and VGKC antibodies, among others) and neuromyotonia
• Granerod J et al 2009 – Lancet Infectious Disease– 203 patients with encephalitis in UK in 2006-2008– 42% infectious, 21% autoimmune, 37% unknown
• 16 case reports and small series of IVIG responsive Ab-mediated encephalitis since 2009
• Complex Regional Pain Syndrome
• Goebel A, Baranowski A, Maurer K, Ghiai A, McCabe C, Ambler G.
• Intravenous immunoglobulin treatment of the complex regional pain syndrome: a randomized trial.
• Ann Intern Med. 2010 Feb 2;152(3):152-8.
Presumed immune-mediated disorders with little or no evidence of efficacy
• Acute idiopathic dysautonomia
• Diabetic proximal neuropathy
• PANDAS
• Paraneoplastic disorders that are known not to be B- or T-cell mediated
• POEMS
There remain rare disorders….
Efficacy outcomes to assess treatment success
Measuring outcomes:Current practice, potential and future possibilities
• ‘This update provides efficacy outcomes to be measured in all indications…. Efficacy outcomes are expected to play an important role in the IAP decision-making process for patients……This change reflects the wider change of focus in the NHS to patient outcomes, as presented in The NHS Outcomes Framework.’
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