Download Specialist Working Group for Immunology Proposed changes to the

Specialist Working Group for Immunology
Proposed changes to the Criteria for the clinical use of intravenous immunoglobulin in Australia, Second Edition
ITEM
CRITERIA FOR THE CLINICAL USE OF INTRAVENOUS
IMMUNOGLOBULIN IN AUSTRALIA, SECOND EDITION
(CRITERIA)
PROPOSED REVISIONS TO THE CRITERIA
(INCLUDING ADAPTATION TO THE IG SYSTEM)
Condition
Name
Toxic epidermal necrolysis (TEN; Lyell syndrome)
Stevens–Johnson syndrome (SJS)
Toxic epidermal necrolysis (TEN; Lyell
syndrome) Stevens–Johnson syndrome (SJS)
Specialty
Dermatology
Dermatology and immunology
Chapter
6
6
Specific
Conditions
SWG RATIONALE FOR PROPOSED CHANGE
(A) Administrative)
(B) Progressive
(C) Programmed
Stevens–Johnson syndrome / toxic epidermal
necrolysis overlap (SJS/TEN);
Toxic epidermal necrolysis (TEN)
Specific conditions were defined (A)
Level of
Evidence
Small case studies only; insufficient data (Category
4a).
Small case studies only; insufficient data
(Category 4a).
Unchanged
Justification
for Evidence
Category
The Biotext (2004) review identified one small cohort
Data surrounding the use of intravenous
immunoglobulin (IVIg) for SJS/TEN
are limited and conflicting. Review of the
literature provides no high-quality evidence
supporting the use of Ig in SJS/TEN. Systematic
review indicates that Ig therapy for patients
with severe disease should be given in the early
phase of the disease (i.e. within 24 to 48 hours
of symptom onset). Systemic corticosteroids
and Ig have been administered in combination
to patients with SJS/TEN, but the data are too
limited to draw any firm conclusions.
This section has been reviewed and revised.
study (20 patients) without a control group, which
found that there appeared to be no significant effect
and that death rate seems to be higher than
previously reported.
The Frommer and Madronio (2006) review found one
small randomised study (four patients) with a control
group of two patients (supportive care only). This
study found that there was some improvement in
epithelialisation and prominent difference in CD95
receptor between treated patients and controls.
However, neither IVIg nor its comparison group could
ITEM
CRITERIA FOR THE CLINICAL USE OF INTRAVENOUS
IMMUNOGLOBULIN IN AUSTRALIA, SECOND EDITION
(CRITERIA)
PROPOSED REVISIONS TO THE CRITERIA
(INCLUDING ADAPTATION TO THE IG SYSTEM)
SWG RATIONALE FOR PROPOSED CHANGE
(A) Administrative)
(B) Progressive
(C) Programmed
SJS and TEN are severe mucocutaneous adverse
reactions, most commonly triggered by
medications such as sulphonamides, antibiotics,
non-steroidal anti-inflammatory drugs (NSAIDs)
and anti-convulsants.
SJS and TEN are characterised by severe bullous
reaction with extensive destruction of the
epidermis, and morphologically by ongoing
apoptotic keratinocyte cell death that results in
the separation of the epidermis from the
dermis.
SJS and TEN are considered a disease
continuum and are distinguished chiefly by
severity, based upon the percentage of body
surface involved with skin detachment. The
term SJS describes patients with blistering and
skin detachment involving a total body surface
area of <10%, SJS/TEN overlap describes
patients with 10–30%, and TEN refers to patient
with >30% skin detachment.
Description and diagnostic criteria have been
revised.
Yes
Which
Speciality
Unchanged
No
Which
Specialty
completely stop the TEN process.
Description
and
Diagnostic
Criteria
There should
be no change
the
published
text
TEN is a rare, life-threatening hypersensitivity reaction
to certain medications, such as sulphonamides,
antibiotics, non-steroidal anti-inflammatory drugs and
anti-convulsants. Drug-induced epidermal apoptosis
has been proposed as possible pathogenesis. Stevens–
Johnson syndrome (SJS) is a less extensive
manifestation of the same phenomenon.
TEN and SJS are characterised by severe bullous
reaction with extensive destruction of the epidermis,
and morphologically by ongoing apoptotic
keratinocyte cell death that results in the separation
of the epidermis from the dermis.
The term SJS is now used to describe patients with
blistering and skin detachment involving a total body
surface area of <10%. SJS/TEN describes patients with
10–30% detachment, and TEN describes patients with
>30% skin detachment.
Diagnosis is
required
Diagnosis by a dermatologist;
Diagnosis
must be
verified
National Blood Authority
Dermatologist
pg. 2
ITEM
CRITERIA FOR THE CLINICAL USE OF INTRAVENOUS
IMMUNOGLOBULIN IN AUSTRALIA, SECOND EDITION
(CRITERIA)
PROPOSED REVISIONS TO THE CRITERIA
(INCLUDING ADAPTATION TO THE IG SYSTEM)
Exclusion
Criteria
SJS alone
SJS alone
Indication
for use
To limit progression of TEN or SJS/TEN when
administered in early stages.
TEN or SJS/TEN with rapid evolution and >10%
body surface area affected.
Qualifying
Criteria
TEN or SJS/TEN overlap with all the following:
Erythema and/or erosions affecting >10% body
surface area.
1. Diagnosis by a dermatologist;
AND
2. Body surface area (erythema and/or erosions)
of 10% or more;
AND
3. Evidence of rapid evolution.
Notes:

Urgent skin biopsy should be performed for
confirmation but should not delay IVIg
therapy if indicated.

The Adverse Drug Reactions Advisory
Committee should be notified of the inciting
National Blood Authority
Given that SJS alone is excluded and this
condition describes body surface area of <10%
involvement, the indication has been modified to
exclude SJS alone.
Eligibility criteria have been extended to support
the early treatment of significant conjunctival
lesions and labial blisters rather than waiting for
10% of skin eruptions to develop. (B)
Significant mucosal lesions, including
conjunctival erosions and labial blisters require
early treatment
AND
Onset of significant skin manifestations (painful
red skin with or without blisters and/or any
mucosal/conjunctival involvement) has
occurred within the last 48 hours.
IVIg should be initiated as early as possible,
preferably within 24 hours of diagnosis.

OR
SWG RATIONALE FOR PROPOSED CHANGE
(A) Administrative)
(B) Progressive
(C) Programmed
Unchanged
IVIg should be initiated as early as possible,
preferably within 24 hours of diagnosis.
Urgent skin biopsy should be performed for
confirmation but should not delay IVIg therapy
if indicated.
The Adverse Drug Reactions Advisory
pg. 3
ITEM
CRITERIA FOR THE CLINICAL USE OF INTRAVENOUS
IMMUNOGLOBULIN IN AUSTRALIA, SECOND EDITION
(CRITERIA)
SWG RATIONALE FOR PROPOSED CHANGE
(A) Administrative)
(B) Progressive
(C) Programmed
Committee should be notified of the inciting
medication.
medication.
Review
Criteria
PROPOSED REVISIONS TO THE CRITERIA
(INCLUDING ADAPTATION TO THE IG SYSTEM)
N/A
There is no review required as this condition
requires one-off treatment.
Given the one-off dosing - this section will only be
completed after treatment and if prescriber
enters the data.
Outcome measures
Clinical assessment one month after
immunoglobulin treatment, including:
 % body surface area still affected
 diagnostic confirmation by biopsy
 description of response to Ig
treatment.
The Adverse Drug Reactions Committee should
be notified of the inciting medication.
Dose
2 g/kg, preferably as a single dose, or divided over
three consecutive days.
Dosing above 1 g/kg per day is contraindicated for
some IVIg products.
Refer to the current product information sheet for
further information.
The aim should be to use the lowest dose possible
that achieves the appropriate clinical outcome for
each patient.
National Blood Authority
Up to 1 g/kg for 3 days within 24 to 48 hours of
symptom onset.
Up to 2 g/kg as a single dose within 24 to 48
hours of symptom
onset.
IVIg should be initiated as early as possible,
preferably within 24 hours of diagnosis.
Skin biopsy should be performed for
confirmation, but should not delay IVIg therapy
if indicated.
Two dosing options are to be offered. From
review of the literature, the dosing varies
between 2g/Kg as a single dose and 1g/kg for 3
days in the early phase of disease. Systemic
corticosteroids and IVIG have been administered
in combination to patients with SJS/TEN, but the
data are too limited to draw any firm conclusions.
No study with set Ig dose and number of days
was found. SWG recommendation is to support
both dosing of 1g/Kg per day for 3 consecutive
days or 2g/Kg as a single dose. This dosing
recommendation has been endorsed by the
College of Dermatology. (B)
pg. 4
ITEM
CRITERIA FOR THE CLINICAL USE OF INTRAVENOUS
IMMUNOGLOBULIN IN AUSTRALIA, SECOND EDITION
(CRITERIA)
PROPOSED REVISIONS TO THE CRITERIA
(INCLUDING ADAPTATION TO THE IG SYSTEM)
SWG RATIONALE FOR PROPOSED CHANGE
(A) Administrative)
(B) Progressive
(C) Programmed
Dosing above 1 g/kg per day is contraindicated
for some IVIg products.
Refer to the current product information sheet
for further information.
The aim should be to use the lowest dose
possible that achieves the appropriate clinical
outcome for each patient.
POTENTIAL OPERATIONAL IMPACT
No operational impacts are anticipated.
POTENTIAL IMPACT ON DEMAND
Patient Numbers
Total treated: 48
2013-14
No impact on demand is anticipated given the very low
number of patients treated in any year, despite one
dosing option potentially increasing usage by 1g/Kg.
POTENTIAL IMPACT ON COST
Current cost
Anticipated reduction in cost, if any
Marginal
Marginal = borderline or unchanged from current cost
Minor = decrease by $500K - $1.99M from current cost
Major = decrease $2M+ from current cost
BIBLIOGRAPHY
Bachot, N, Revuz, J & Roujeau, JC 2003, ‘Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis: a prospective noncomparative study showing no benefit on mortality or progression’ (comment), Archives of Dermatology, vol. 139, no. 1, pp. 85–6.
Biotext 2004, ‘Summary data on conditions and papers’, in A systematic literature review and report on the efficacy of intravenous immunoglobulin therapy and its risks,
National Blood Authority
pg. 5
commissioned by the National Blood Authority on behalf of all Australian Governments, pp. 242–7. Available from: http://www.nba.gov.au/pubs/pdf/report-lit-rev.pdf.
Campione, E, Marulli, GC, Carrozzo, AM, et al 2003, ‘High-dose intravenous immunoglobulin for severe drug reactions: efficacy in toxic epidermal necrolysis’, Acta Dermatovenereologica, vol. 83, no. 6, pp. 430–2.
Del Pozzo-Magana, BR, Lazo-Langner, A, Carleton, B, et al. 2011, ‘A systematic review of treatment of drug-induced Stevens–Johnson syndrome and toxic epidermal
necrolysis in children’, Journal of Population Therapeutics and Clinical Pharmacology, vol 18, no. 1, pp. e121–e133.
Downey, A, Jackson, C, Harun, ,N et al 2012, ‘Toxic epidermal necrolysis: review of pathogenesis and management’ [Review], Journal of the American Academy of
Dermatology, vol. 66, no. 6, pp. 995–1003
Frommer, M & Madronio, C 2006, The use of intravenous immunoglobulin in Australia. A report for the National Blood Authority, Part B: systematic literature review, Sydney
Health Projects Group, University of Sydney, Sydney, pp. 55–6.
Huang, YC, Li, YC, Chen, TJ, 2012, ‘The efficacy of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis: a systematic review and meta-analysis’
[Review], British Journal of Dermatology, vol. 167, no. 2, pp. 424–32.
Mockenhaupt, M 2014, ‘Stevens–Johnson syndrome and toxic epidermal necrolysis: clinical patterns, diagnostic considerations, etiology, and therapeutic management’
[Review], Seminars in Cutaneous Medicine & Surgery, vol. 33, no. 1, pp. 10–6.
Paquet, P, Jacob, E, Damas, P, et al 2005, ‘Analytical quantification of the inflammatory cell infiltrate and CD95R expression during treatment of drug-induced toxic epidermal
necrolysis’, Archives of Dermatological Research, vol. 297, no. 6, pp. 266–73.
Prins, C, Vittorio, C, Padilla, RS, et al 2003, ‘Effect of high- dose intravenous immunoglobulin therapy in Stevens-Johnson syndrome: a retrospective, multicentre study’,
Dermatology, vol. 207, no. 1, pp. 96–9.
Trent, J, Halem, M, French, LE, et al 2006, ‘Toxic epidermal necrolysis and intravenous immunoglobulin: a review’, Seminars in Cutaneous Medicine and Surgery, vol. 25, no.
2, pp. 91–3.
END OF DOCUMENT
National Blood Authority
pg. 6