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Primary immunodeficiency diseases (PID) with antibody deficiency Conditions for which IVIg has an established therapeutic role. Specific Conditions Severe combined immunodeficiency (SCID) Combined immunodeficiency generally less profound than SCID (e.g. thymoma) Combined immunodeficiency with associated or syndromal features (e.g. Wiskott Aldrich syndrome; ataxia telangiectasia) Severe reduction in all Ig isotypes with decreased or absent B‐cells (e.g. XLA def) Severe reduction in at least two Ig isotypes with low/normal B‐cells (e.g. CVID) Severe reduction in serum IgG and IgA with normal/elevated IgM (e.g. CD40L def) Transient hypogammaglobulinaemia of infancy Lymphoproliferative syndromes (e.g. XLP1, XLP2, CD27 def) Possible Common variable immune deficiency (CVID) ‐ below normal serum IgG but normal serum IgA level Indication for IVIg Use Replacement therapy in common variable immune deficiency (CVID) – ESID diagnostic criteria met Replacement therapy in possible common variable immune deficiency (CVID) – (below normal serum IgG but normal serum IgA level) Replacement therapy in transient hypogammaglobulinaemia of infancy (children aged less than 4 years) Replacement therapy in recognised primary immunodeficiencies for which immunoglobulin replacement is universally indicated (e.g. SCID, Wiskott‐ Aldrich syndrome, etc.) Level of Evidence Evidence of probable benefit – more research needed (Category 2a) Description and Diagnostic Criteria More than 280 primary immundeficiency diseases (PIDs) have been identified. Many of these cause antibody deficiency. In some cases, antibody deficiency is associated with B‐cell deficiency (e.g. X‐linked agammaglobulinaemia), while in others, B‐cells are present. Antibody deficiency can be the only manifestation of PID, or there can be other defects as well (e.g. T‐cell deficiency, autoimmunity). Not all PIDs cause antibody defects and, therefore, immunoglobulin replacement is not always indicated. Recognised primary immunodeficiencies for which immunoglobulin replacement is universally indicated are: X‐linked agamma/hypogammaglobulinaemia, severe combined immunodeficiency, Wiskott‐Aldrich syndrome, X‐linked lymphoproliferative syndrome, hyper IgM syndrome and severe T‐cell immunodeficiency. The revised European Society for Immunodeficiency Diseases (ESID) (2014) diagnostic criteria for common variable immune deficiency (CVID) require the diagnosis to be established after the fourth year of life (but symptoms may be present before) and at least one of the following: increased susceptibility to infection autoimmune manifestations granulomatous disease unexplained polyclonal lymphoproliferation affected family member with antibody deficiency.
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Primary immunodeficiency diseases (PID) with antibody deficiencyConditions for which IVIg has an established therapeutic role.
Specific ConditionsSevere combined immunodeficiency (SCID)
Combined immunodeficiency generally less profound than SCID (e.g.thymoma)
Combined immunodeficiency with associated or syndromal features (e.g.Wiskott Aldrich syndrome; ataxia telangiectasia)
Severe reduction in all Ig isotypes with decreased or absent B‐cells (e.g.XLA def)
Severe reduction in at least two Ig isotypes with low/normal B‐cells (e.g.CVID)
Severe reduction in serum IgG and IgA with normal/elevated IgM (e.g.CD40L def)
Transient hypogammaglobulinaemia of infancy
Lymphoproliferative syndromes (e.g. XLP1, XLP2, CD27 def)
Possible Common variable immune deficiency (CVID) ‐ below normalserum IgG but normal serum IgA level
Indication for IVIg UseReplacement therapy in common variable immune deficiency (CVID) – ESIDdiagnostic criteria met
Replacement therapy in possible common variable immune deficiency(CVID) – (below normal serum IgG but normal serum IgA level)
Replacement therapy in transient hypogammaglobulinaemia of infancy(children aged less than 4 years)
Replacement therapy in recognised primary immunodeficiencies for whichimmunoglobulin replacement is universally indicated (e.g. SCID, Wiskott‐Aldrich syndrome, etc.)
Level of Evidence Evidence of probable benefit – more research needed (Category 2a)
Description and DiagnosticCriteria
More than 280 primary immundeficiency diseases (PIDs) have been identified.Many of these cause antibody deficiency. In some cases, antibody deficiency isassociated with B‐cell deficiency (e.g. X‐linked agammaglobulinaemia), while inothers, B‐cells are present. Antibody deficiency can be the only manifestation ofPID, or there can be other defects as well (e.g. T‐cell deficiency, autoimmunity).Not all PIDs cause antibody defects and, therefore, immunoglobulin replacement isnot always indicated.
Recognised primary immunodeficiencies for which immunoglobulin replacement isuniversally indicated are: X‐linked agamma/hypogammaglobulinaemia, severecombined immunodeficiency, Wiskott‐Aldrich syndrome, X‐linkedlymphoproliferative syndrome, hyper IgM syndrome and severe T‐cellimmunodeficiency.
The revised European Society for Immunodeficiency Diseases (ESID) (2014)diagnostic criteria for common variable immune deficiency (CVID) require thediagnosis to be established after the fourth year of life (but symptoms may bepresent before) and at least one of the following:
increased susceptibility to infectionautoimmune manifestationsgranulomatous diseaseunexplained polyclonal lymphoproliferationaffected family member with antibody deficiency.
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AND
A marked decrease of immunoglobulin G (IgG) and marked decrease of IgA with orwithout low IgM levels (measured at least twice; less than the normal referencerange for their age).
AND
At least one of the following:
poor antibody response to vaccines (and/or absent isohemagglutinins); i.e.absence of protective levels despite vaccination where definedlow switched memory B‐cells (less than 70 percent of age‐related normalvalue).
AND
Secondary causes of hypogammaglobulinemia have been excluded.
The ESID diagnostic criteria for PID have been used as a guide in the developmentof the qualifying criteria for Ig therapy in Australia. It is acknowledged that a lowIgG alone is not a sufficient indication for immunoglobulin replacement.
Genetic diagnoses are continually being updated as described in the classificationsystem for the International Union of Immunology Societies (IUIS). It is recognisedthat genetic diagnoses are not always possible.
Justification for EvidenceCategory
The Biotext (2004) review reported level 2a evidence for the use of intravenousimmunoglobulin (IVIg) in the treatment of common variable immunodeficiencyand primary hypogammaglobulinaemia.
Diagnosis Requirements A diagnosis must be made by an Immunologist.
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Qualifying Criteria for IVIgTherapy
Replacement therapy in common variable immune deficiency (CVID) – ESIDdiagnostic criteria met
Note: If less than four years the request must be under the indicationTransient hypogammaglobulinaemia of infancy (children aged less than fouryears)
The patient is older than four years of age
AND
Blood samples for IgG and IgA testing should be taken on two occasions, atleast one hour apart and at least one sample taken when the patient does nothave an infection
Evidence of a marked decrease of IgG and a marked decrease of IgA withor without low IgM levels and causes of secondaryhypogammaglobulinaemia have been excluded
AND
Documented failure of serum antibody response after vaccination withconjugated or unconjugated pneumococcal vaccine or following proteinvaccine challenge
OR
The patient’s serum IgG is less than 2 g/L and a delay to providing Igreplacement (e.g. following an invasive bacterial infection) would presentsignificant risk
OR
The patient has absent haemagglutinins (if not blood group AB)
OR
The patient has low switched memory B‐cells (less than 70 percent ofage‐related normal value)
AND
The patient has demonstrated an increased susceptibility to infection
OR
The patient has autoimmune manifestations, granulomatous disease,unexplained polyclonal lymphoproliferation or an affected familymember with antibody deficiency
Initial review by an immunologist is required at six months and annuallythereafter. Documentation of clinical effectiveness is necessary forcontinuation of Ig therapy.
The review criteria for primary immunodeficiency diseases are to ensureadequate replacement of antibody deficiency and to demonstrate clinicalbenefit from treatment.
Replacement therapy in possible common variable immune deficiency (CVID) –(below normal serum IgG but normal serum IgA level)
A low IgG (normal IgA with or without a low IgM) alone is not a sufficientindication for immunoglobulin replacement therapy. Many patients will be welldespite the finding of a serum IgG below the normal range for age.
The patient is older than four years of age
AND
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Blood samples for IgG testing should be taken on two occasions, at least onehour apart and at least one sample taken when the patient does not have aninfection
Evidence of a marked decrease of IgG with normal IgA (with or withoutlow IgM) levels and causes of secondary hypogammaglobulinaemia havebeen excluded
AND
Documented failure of serum antibody response after vaccination withconjugated or unconjugated pneumococcal vaccine or following proteinvaccine challenge
OR
The patient’s serum IgG less than 2 g/L and a delay to providing Igreplacement (e.g. following an invasive bacterial infection) would presentsignificant risk
OR
The patient has absent haemagglutinins (if not blood group AB)
OR
The patient has low switched memory B‐cells (less than 70 percent ofage‐related normal value)
AND
The patient has demonstrated an increased susceptibility to infection
OR
The patient has autoimmune manifestations, granulomatous disease,unexplained polyclonal lymphoproliferation or an affected familymember with antibody deficiency
Initial review is required by an Immunologist at six months and ongoingreviews at least annually to assess clinical benefit. Documentation of clinicaleffectiveness is necessary for continuation of Ig therapy. Cessation of Ig therapy should be considered at least after each 12 months oftreatment. If serum IgM and IgA levels are trending upwards and near normal,this may suggest recovery of the immune system and a trial might beconsidered if the patient is well. Once the patient has normal IgA and IgMlevels, the IgG is also likely to be normal and a trial off therapy may beundertaken. Ig therapy should be extended as required to enable cessation of therapy inSeptember/October, with repeat clinical and/or immunological evaluationbefore re‐commencement of therapy. This should particularly be considered in patients who do not have activebronchiectasis and/or suppurative lung disease. An immunoglobulin washoutperiod of four to six months is necessary to enable an accurate assessment.Prophylactic antibiotics may be considered to cover the period of cessation ofimmunoglobulin therapy.
*Please note: a diagnosis of bronchiectasis and/or suppurative lung diseasemust be consistent with the guideline of the Thoracic Society of Australiaand New Zealand and the Australian Lung Foundation (Chang et al 2014).
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Replacement therapy in transient hypogammaglobulinaemia of infancy(children aged less than 4 years)
The majority of young children with transient hypogammaglobulinaemia donot require immunoglobulin (Ig) therapy. However, if the patient has hadrecurrent suppurative infections that threaten organ function, review by animmunologist is recommended for consideration of Ig therapy. Some patientsmay require treatment during the winter months only and others will benefitfrom more prolonged treatment. Blood samples for IgG testing should be taken on two occasions, at least onehour apart and at least one sample taken when the patient does not have aninfection.
Younger than four years of age at diagnosis
AND
Evidence of a marked decrease of IgG and causes of secondaryhypogammaglobulinemia have been excluded
AND
The patient has demonstrated an increased susceptibility to infection
Initial review is required by an Immunologist, at six months, and ongoingreviews at least annually to assess clinical benefit. Documentation of clinicaleffectiveness is necessary for continuation of Ig therapy. Cessation of Ig therapy should be considered at least after 24 months oftreatment. If serum IgM and IgA levels are trending upwards and close tonormal, this may suggest recovery of the immune system and a trial might beconsidered if the patient is well. Once the patient has normal IgA and IgMlevels, the IgG is also likely to be normal and a trial off therapy should beundertaken. Ig therapy should be extended as required to enable cessation of therapy inSeptember/October, with repeat clinical and/or immunological evaluationbefore re‐commencement of therapy. When the child is four years old, a decision must be made regarding a trial offtreatment or qualification may be appropriate under a different indicationsuch as possible or confirmed CVID.
Replacement therapy in recognised primary immunodeficiencies for whichimmunoglobulin replacement is universally indicated (e.g. SCID, Wiskott‐Aldrich syndrome, etc.)
Blood samples for IgG testing should be taken on two occasions, at least onehour apart and at least one sample taken when the patient does not have aninfection.
Confirmed or suspected diagnosis of primary immunodeficiency
AND
Evidence of hypogammaglobulinaemia
Initial review by an Immunologist is required at six months, with reviewsannually thereafter. Documentation of clinical effectiveness is necessary forcontinuation of IVIg therapy.
Where a diagnosis has initially been suspected, confirmation will be requiredfor access to continuing Ig therapy.
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Exclusion Criteria Acquired hypogammaglobulinaemia secondary to haematological malignancy orpost HSCT ‐ see Acquired‐hypogammaglobulinaemia — haematologicalmalignancy or post HSCT Specific antibody deficiency ‐ see Specific antibody deficiency (SAD) IgG subclass deficiency ‐ see Specific antibody deficiency (SAD) Secondary hypogammaglobulinaemia unrelated to haematological malignancy orstem cell transplantation ‐ see Secondary hypogammaglobulinaemia (includingiatrogenic immunodeficiency)
Review Criteria for Assessingthe Effectiveness of IVIg Use
Replacement therapy in common variable immune deficiency (CVID) – ESIDdiagnostic criteria met
Initial review by an immunologist is required at six months and annuallythereafter. Documentation of clinical effectiveness is necessary forcontinuation of Ig therapy.
The review criteria for primary immunodeficiency diseases are to ensureadequate replacement of antibody deficiency and to demonstrate clinicalbenefit from treatment.
Clinical effectiveness of Ig therapy may be assessed by:
On review of the initial authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
A diagnosis of CVID has been made
On review of a continuing authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
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Replacement therapy in possible common variable immune deficiency (CVID) –(below normal serum IgG but normal serum IgA level)
Initial review is required by an Immunologist at six months and ongoingreviews at least annually to assess clinical benefit. Documentation of clinicaleffectiveness is necessary for continuation of Ig therapy. Cessation of Ig therapy should be considered at least after each 12 months oftreatment. If serum IgM and IgA levels are trending upwards and near normal,this may suggest recovery of the immune system and a trial might beconsidered if the patient is well. Once the patient has normal IgA and IgMlevels, the IgG is also likely to be normal and a trial off therapy may beundertaken. Ig therapy should be extended as required to enable cessation of therapy inSeptember/October, with repeat clinical and/or immunological evaluationbefore re‐commencement of therapy. This should particularly be considered in patients who do not have activebronchiectasis and/or suppurative lung disease. An immunoglobulin washoutperiod of four to six months is necessary to enable an accurate assessment.Prophylactic antibiotics may be considered to cover the period of cessation ofimmunoglobulin therapy.
Clinical effectiveness of Ig therapy may be assessed by:
On review of the initial authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
When IgA and IgM are trending upwards and close to normal and thepatient is well, a trial off therapy (in September or October) should beconsidered to allow immunological re‐evaluation
On review of a continuing authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
When IgA and IgM are trending upwards and close to normal and thepatient is well, a trial off therapy (in September or October) should beconsidered to allow immunological re‐evaluation
Antibiotic therapy may be indicated in addition to immunoglobulin therapy.
*Please note: a diagnosis of bronchiectasis and/or suppurative lung diseasemust be consistent with the guideline of the Thoracic Society of Australiaand New Zealand (Chang et al 2014).
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Replacement therapy in transient hypogammaglobulinaemia of infancy(children aged less than 4 years)
Initial review is required by an Immunologist, at six months, and ongoingreviews at least annually to assess clinical benefit. Documentation of clinicaleffectiveness is necessary for continuation of Ig therapy. Cessation of Ig therapy should be considered at least after 24 months oftreatment. If serum IgM and IgA levels are trending upwards and close tonormal, this may suggest recovery of the immune system and a trial might beconsidered if the patient is well. Once the patient has normal IgA and IgMlevels, the IgG is also likely to be normal and a trial off therapy should beundertaken. Ig therapy should be extended as required to enable cessation of therapy inSeptember/October, with repeat clinical and/or immunological evaluationbefore re‐commencement of therapy. When the child is four years old, a decision must be made regarding a trial offtreatment or qualification may be appropriate under a different indicationsuch as possible or confirmed CVID.
Clinical effectiveness of Ig therapy may be assessed by:
On review of the initial authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
When IgA and IgM are trending upwards and close to normal and thepatient is well, a trial off therapy (in September or October) should beconsidered to allow immunological re‐evaluation
On review of a continuing authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
When IgA and IgM are trending upwards and close to normal and thepatient is well, a trial off therapy (in September or October) should beconsidered to allow immunological re‐evaluation
Antibiotic therapy may be indicated in addition to immunoglobulin therapy.
*Please note: a diagnosis of bronchiectasis and/or suppurative lung diseasemust be consistent with the guideline of the Thoracic Society of Australiaand New Zealand (Chang et al 2014).
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Replacement therapy in recognised primary immunodeficiencies for whichimmunoglobulin replacement is universally indicated (e.g. SCID, Wiskott‐Aldrich syndrome, etc.)
Initial review by an Immunologist is required at six months, with reviewsannually thereafter. Documentation of clinical effectiveness is necessary forcontinuation of IVIg therapy.
The review criteria for primary immunodeficiency diseases are to ensureadequate replacement of antibody deficiency and to demonstrate clinicalbenefit from treatment.
Clinical effectiveness of Ig therapy may be assessed by:
On review of the initial authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
A diagnosis of primary immunodeficiency has been confirmed
On review of a continuing authorisation period
Monitoring of serum immunoglobulin levels (IgG, IgA and IgM) and anyhistory of infection
AND
A diagnosis of primary immunodeficiency has been confirmed
Dose Replacement therapy in common variable immune deficiency (CVID) – ESIDdiagnostic criteria met
Loading Dose ‐ One loading dose of 0.4 g/kg in the first month oftherapy (in addition to the maintenance dose) is permitted if theserum IgG level is <4 g/L.
Disseminated Enterovirus dose ‐ One dose of 2g/kg at any stage ispermitted (in addition to the maintenance dose) in the managementof disseminated enterovirus infection.
Maintenance Dose ‐ 0.4 g/kg every four weeks or more frequently,to achieve IgG trough level of at least the lower limit of the age‐specific serum IgG reference range. More frequent dosing toachieve IgG trough level of up to 9g/L is permitted if chronicsuppurative lung disease is not adequately controlled at an IgGtrough level at the lower limit of the age‐specific serum IgGreference range. A total dose of up to 1g/kg may be given over anyfour week period.
Subcutaneous administration of immunoglobulin can be considered as analternative to IVIg. A suggested dose is 0.1 g/kg lean body mass every week,modified to achieve an IgG trough level of at least the lower limit of the age‐specific serum IgG reference range. The aim should be to use the lowest dose possible that achieves theappropriate clinical outcome for each patient.
Refer to the current product information sheet for further information ondose, administration and contraindications.
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Replacement therapy in possible common variable immune deficiency (CVID) –(below normal serum IgG but normal serum IgA level)
Loading Dose ‐ One loading dose of 0.4 g/kg in the first month oftherapy (in addition to the maintenance dose) is permitted if theserum IgG level is <4 g/L.
Dissemination Enterovirus Dose ‐ One dose of 2 g/kg at any stageis permitted (in addition to the maintenance dose) in themanagement of disseminated enterovirus infection.
Maintenance Dose ‐ 0.4 g/kg every four weeks or more frequently,to achieve IgG trough level of at least the lower limit of the age‐specific serum IgG reference range. More frequent dosing toachieve IgG trough level of up to 9 g/L is permitted if chronicsuppurative lung disease is not adequately controlled at an IgGtrough level at the lower limit of the age‐specific serum IgGreference range. A total dose of up to 1 g/kg may be given over anyfour week period.
Subcutaneous administration of immunoglobulins is a suitable alternative toIVIg in this condition; a suggested dose is 0.1 g/kg lean body mass every week,modified to achieve a serum IgG level of at least the lower limit of the agespecific serum IgG reference range. The aim should be to use the lowest dose possible that achieves theappropriate clinical outcome for each patient.
Refer to the current product information sheet for further information ondose, administration and contraindications.
Replacement therapy in transient hypogammaglobulinaemia of infancy(children aged less than 4 years)
Loading Dose ‐ One loading dose of 0.4 g/kg in the first month oftherapy (in addition to the maintenance dose) is permitted if theserum IgG level is <4 g/L.
Maintenance Dose ‐ .4 g/kg every four weeks or more frequently,to achieve IgG trough level of at least the lower limit of the age‐specific serum IgG reference range. More frequent dosing toachieve IgG trough level of up to 9 g/L is permitted if chronicsuppurative lung disease is not adequately controlled at an IgGtrough level at the lower limit of the age‐specific serum IgGreference range. A total dose of up to 1 g/kg may be given over anyfour week period.
Subcutaneous administration of immunoglobulin can be considered as analternative to IVIg. A suggested dose is 0.1 g/kg lean body mass every week,modified to achieve an IgG trough level of at least the lower limit of the age‐specific serum IgG reference range. The aim should be to use the lowest dose possible that achieves theappropriate clinical outcome for each patient.
Refer to the current product information sheet for further information ondose, administration and contraindications.
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Replacement therapy in recognised primary immunodeficiencies for whichimmunoglobulin replacement is universally indicated (e.g. SCID, Wiskott‐Aldrich syndrome, etc.)
Loading Dose ‐ One loading dose of 0.4 g/kg in the first month oftherapy (in addition to the maintenance dose) is permitted if theserum IgG level is <4 g/L.
Disseminated Enterovirus Dose ‐ One dose of 2 g/kg at any stage ispermitted (in addition to the maintenance dose) in the managementof disseminated enterovirus infection.
Maintenance Dose ‐ 0.4 g/kg every four weeks or more frequently,to achieve IgG trough level of at least the lower limit of the age‐specific serum IgG reference range. More frequent dosing toachieve IgG trough level of up to 9 g/L is permitted if chronicsuppurative lung disease is not adequately controlled at an IgGtrough level at the lower limit of the age‐specific serum IgGreference range. A total dose of up to 1 g/kg may be given over anyfour week period.
Subcutaneous administration of immunoglobulin can be considered as analternative to IVIg. A suggested dose is 0.1 g/kg lean body mass every week,modified to achieve an IgG trough level of at least the lower limit of the age‐specific serum IgG reference range.
The aim should be to use the lowest dose possible that achieves theappropriate clinical outcome for each patient.
Refer to the current product information sheet for further information ondose, administration and contraindications.
Bibliography
Biotext 2004, ‘Summary data on conditions and papers’, in A systematic literature review and report on the efficacy ofintravenous immunoglobulin therapy and its risks, commissioned by the National Blood Authority on behalf of allAustralian Governments, pp. 218. Available from: https://www.blood.gov.au/system/files/A‐systematic‐literature‐review‐and‐report‐on‐the‐efficacy‐of‐IVIg‐therapy‐and‐its‐risks.pdf
Bonilla, FA, Bernstein, L, Khan, DA, et al 2005, ‘Practice parameter for the diagnosis and management of primaryimmunodeficiency’, Annals of Allergy, Asthma and Immunology, vol. 94, no. 5, suppl. 1, pp. S1–63.
Chang, AB, Bell, SC, Torzillo, PJ, et al 2014, ‘Thoracic Sodiety of Australia and New Zealand Chronic Suppurative LungDisease and Bronchiectasis in children and adults in Australia and New Zealand – Clinical Practice Guideline’. Availablefrom: https://www.thoracic.org.au/journal‐publishing/command/download_file/id/36/filename/TSANZ‐ChronicSuppurativeLungDisease‐Guidelines‐2016‐web.pdf.
Cooper, MD & Schroeder, Jr HW 2005, ‘Primary immune deficiency diseases’, in DL Kasper, E Braunwald, AS Fauci, et al(eds), Harrison’s Textbook of Medicine, 16th edn, McGraw‐Hill, New York, pp. 1939–47.
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