背景
Inborn errors of immunity (IEI, previously referred to as primary immunodeficiencies) comprise a broad group of inherited disorders affecting one or more components of the immune system. These conditions result in increased susceptibility to infections, including vaccine。preventable diseases, as well as a higher risk of autoimmunity and autoinflammatory disease. Those with predominantly humoral (B‑cell) defects, cellular (T‑cell) defects, or combined immune deficiencies typically have absent or suboptimal responses to vaccination.
The majority of people with IEI are managed with lifelong immunoglobulin replacement therapy (IRT), which provides passive immunity and offers effective protection against many invasive bacterial and viral infections.
建议
People with IEI receiving IRT, either via regular intravenous or subcutaneous infusions, 不要 require routine vaccinations. This is because they are unlikely to mount a meaningful immune response, and they are considered protected against most vaccine‑preventable infections through their ongoing immunoglobulin therapy. Although inactivated vaccines are safe in this population, they are generally not administered in routine clinical practice due to their limited effectiveness.
This approach, differ耳鼻喉科 from the recommendations in the Australian Immunisation Handbook, reflects current standard practice among paediatric immunologists in Australia and internationally.
一个 exception to this broad recommendation is the inactivated injected vaccine, which is routinely recommended on an annual basis for people receiving IRT. This is because people receiving IRT may still mount a T-cell response that confers partial protection or reduces disease severity, and because immunoglobulin products are unlikely to contain protective antibodies against current circulating influenza strains due to the lag between plasma donation and distribution of immunoglobulin products (7–12 months) . For similar reasons, people receiving IRT are also recommended to receive a primary series of 新冠肺炎 vaccination 加强剂量, and adults 18 years and older are recommended to receive 2 doses of inactivat编辑 herpes zoster vaccine at the recommend编辑 time interval.
禁忌症
Live‑attenuated vaccines are contraindicated in individuals with IEI and should not be given (even when receiving IRT) because of concerns regarding both safety and efficacy.
Other considerations
In some cases, IRT is administered on a temporary basis, such as prior to definitive treatment of an IEI with haematopoietic stem cell transplantation (HSCT), or in conditions where therapy is provided for a defined period before immune function is reassessed. Immunisation status should be reviewed following cessation of treatment. In most cases, routine vaccinations can be recommenced according to the standard immunisation schedule.
For family members/carers of people with IEI receving IRT, ensure all routine immunisations are up to date. Inactivated vaccines are recommended for close contacts to prevent natural infection transmission. Live-attenuated vaccines in contacts are generally safe. Household infants should still receive the oral rotavirus vaccine, but because the vaccine virus can be shed in stool for up to about 1 to 2 weeks, close contacts of patients with an IEI should practise strict hand hygiene after nappy changes. The risk of transmission is low, but these precautions help minimise potential exposure.
常见问题
What if a vaccine is inadvertently given to a person receiving IRT?
For inactivated vaccines:
The individual/family should be informed of the error using the open disclosure framework and reassured that this is not a safety concern. The inadvertently administered dose is an invalid dose and will need to be repeated (after the appropriate interval has passed) if the patient ceases IRT in the future.
It should also be noted in the patient records that this is considered an invalid dose and must be repeated if the patient ceases IRT in the future. All vaccines should be reported on the Australian Immunisation Record (AIR), whether they were intended for administration or not.
For live-attenuated vaccines:
There is the potential for safety concerns if a live-attenuated vaccine is administered to a person with an IEI (regardless of whether they are receiving IRT or not). Immediate consultation with the individual’s treating immunologist and/or infectious disease physician is required so appropriate management can be commenced (e.g. anti-viral therapy, monitoring).
The individual/family should be informed of the error using the open disclosure framework. The error must also be reported to the relevant authority to ensure appropriate follow up and support can be provided.
What if I am not sure if my patient is receiving immunoglobulin therapy for replacement or another indication?
Immunoglobulin replacement therapy (IRT) is typically administered as an ongoing treatment either every four weeks via intravenous infusion (IVIG) or weekly via subcutaneous infusion (SCIG). The doses used for replacement are generally lower than those used when normal human immunoglobulin is prescribed for its immunomodulatory effects. Typical dosing for replacement therapy is approximately 0.4 g/kg every four weeks for IVIG or 0.1 g/kg weekly for SCIG.
In Australia, the clinical use of immunoglobulin is strictly regulated by the National Blood Authority (NBA), which sets specific eligibility criteria for funded treatment. For patients with an inborn error of immunity (IEI) receiving IRT, the NBA maintains a defined list of approved conditions that meet these criteria, and the diagnosis must be confirmed by a clinical immunologist.
As a result, patients receiving immunoglobulin replacement therapy are typically under the care of an immunologist and receive regular infusions (monthly for IVIG or weekly for SCIG) as part of long-term management.
Should I complete an immunisation medical exemption form on AIR if I believe a patient is on IRT and should not be receiving immunisations?
When a patient with an IEI begins IRT, their treating immunologist will usually complete the medical exemption form 在 空气. If this has not yet been done, it is recommended that you contact the patient’s treating immunologist before submitting the form to confirm whether a medical exemption 是 .
Should I complete an immunisation medical exemption form on AIR if I believe a patient is on IRT and should not be receiving immunisations?
When a patient with an IEI begins IRT, their treating immunologist will usually complete the medical exemption form 在 空气. If this has not yet been done, it is recommended that you contact the patient’s treating immunologist before submitting the form to confirm whether a medical exemption 是 .
资源
- Immunization of immunocompromised persons: Canadian Immunization Guide
- Rubin LG, Levin MJ, Ljungman P, et al. 2013 IDSA clinical practice guideline for vaccination of the immunocompromised host. Clin Infect Dis. 2014;58(3):309-318. doi:10.1093/cid/cit816
- Prevot J, Jolles S. Global immunoglobulin supply: steaming towards the iceberg?. Curr Opin Allergy Clin Immunol. 2020;20(6):557-564. doi:10.1097/ACI.0000000000000696
作者： Gabby Mahoney (Paediatric Allergist and Immunologist, Royal Children’s Hospital), Theresa Cole (Consultant, Allergy and Immunology, Royal Children’s Hospital), Samantha Chan (Deputy Director, Clinical Immunology and Allergy, Royal Melbourne Hospital) and Nigel Crawford (MVEC Director)
日期： May 2026
本节中的材料会根据新信息的发布进行更新。墨尔本疫苗教育中心（MVEC）工作人员定期审查材料的准确性。
You should not consider the information on this site to be specific, professional medical advice for your personal health or for your family’s personal health. For medical concerns, including decisions about vaccinations, medications and other treatments, you should always consult a healthcare professional.