{"id":22960,"date":"2026-05-19T10:01:19","date_gmt":"2026-05-19T00:01:19","guid":{"rendered":"https:\/\/mvec.mcri.edu.au\/?post_type=references&p=22960"},"modified":"2026-05-19T10:56:06","modified_gmt":"2026-05-19T00:56:06","slug":"inborn-errors-of-immunity","status":"publish","type":"references","link":"https:\/\/mvec.mcri.edu.au\/zh\/references\/inborn-errors-of-immunity\/","title":{"rendered":"Inborn errors of immunity"},"content":{"rendered":"
<\/p>\n

\u80cc\u666f<\/span><\/b>\u00a0<\/span><\/h2>\n

Inborn errors of immunity\u00a0(IEI,\u00a0previously referred to as primary immunodeficiencies)\u00a0comprise\u00a0a broad group of inherited disorders affecting one or more components of the immune system. These conditions result in increased susceptibility to infections,\u00a0including vaccine<\/span>\u3002<\/span>preventable diseases<\/span>, as well as a higher risk of autoimmunity and autoinflammatory disease. Those with predominantly humoral (B\u2011cell) defects, cellular (T\u2011cell) defects, or combined immune deficiencies typically have absent or suboptimal responses to vaccination.<\/span><\/p>\n

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.<\/span><\/p>\n

\u5efa\u8bae<\/span><\/b>\u00a0<\/span><\/h2>\n

People <\/span>with IEI\u00a0<\/span>receiving\u00a0<\/span>IRT<\/span>, either via regular intravenous or subcutaneous infusions,\u00a0<\/span><\/span>\u4e0d\u8981<\/span><\/span> require routine vaccinations. This is because they are unlikely to mount a meaningful immune response, and they are considered protected against most vaccine\u2011preventable infections through their ongoing immunoglobulin therapy. Although inactivated vaccines are safe in this population, they are\u00a0<\/span>generally not<\/span>\u00a0administered in routine clinical practice due to their limited effectiveness.<\/span><\/span><\/p>\n

This<\/span><\/span><\/span>\u00a0approach<\/span><\/span>,<\/span><\/span><\/span>\u00a0differ<\/span><\/span>\u8033\u9f3b\u5589\u79d1<\/span><\/span><\/span> from the recommendations in the Australian Immunisation Handbook, <\/span><\/span>reflects\u00a0<\/span>current\u00a0<\/span>standard practice among paediatric immunologists in Australia and internationally.<\/span><\/span><\/p>\n

\u4e00\u4e2a\u00a0<\/span>exception to this\u00a0<\/span>broad\u00a0<\/span>recommendation is the inactivated\u00a0<\/span>injected\u00a0<\/span>

<\/div><\/a>\u00a0<\/span>vaccine, which is routinely recommended on an annual basis for people receiving <\/span>IRT<\/span>. 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\u201312 months) . For similar reasons, people receiving IRT are also recommended to receive a primary series of <\/span>\u65b0\u51a0\u80ba\u708e<\/a> vaccination\u00a0<\/span>
<\/div>\u00a0<\/span>
<\/div><\/span>\u00a0\u52a0\u5f3a\u5242\u91cf<\/span><\/span>, and adults 18 years and older are recommended to receive\u00a0<\/span><\/span><\/span>2 doses of\u00a0<\/span><\/span><\/span>inactiv<\/span><\/span><\/span>at<\/span><\/span><\/span>\u7f16\u8f91 herpes zoste<\/a><\/span><\/span><\/span>r<\/a>\u00a0<\/span><\/span><\/span>vaccine at the recommend<\/span><\/span><\/span>\u7f16\u8f91<\/span><\/span><\/span>\u00a0time interval<\/span><\/span><\/span>.<\/span><\/span><\/span><\/p>\n

\u7981\u5fcc\u75c7<\/h2>\n

Live\u2011attenuated vaccines are contraindicated in individuals with IEI and should not be given (even when receiving IRT) because of concerns regarding both safety and efficacy.<\/p>\n

Other considerations<\/h2>\n

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)<\/a>, 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.<\/span><\/p>\n

For family members\/carers of people with IEI receving IRT, ensure all routine immunisations are up to date. <\/span>Inactivated vaccines are recommended for close contacts to prevent natural infection transmission. Live-attenuated vaccines in contacts are <\/span>generally saf<\/span>e<\/span>.\u00a0<\/span>Household infants should still receive the\u202f<\/span>oral rotavirus vaccine<\/span>, but because the vaccine virus can be\u202f<\/span>shed in stool for up to about 1 to 2 weeks<\/span>, close contacts of patients with\u00a0<\/span>an IEI<\/span>\u00a0should practise\u202f<\/span>strict hand hygiene after nappy changes<\/span>. The risk of transmission is\u202f<\/span>low<\/span>, but these precautions help minimise potential exposure<\/span>.<\/span><\/span><\/p>\n

\u5e38\u89c1\u95ee\u9898<\/span><\/b><\/h2>\n

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