had their immunological status re-evaluated. Patients with a confirmed IgG deficiency have become eligible under another indication (e.g. primary immunodeficiency with antibody deficiency). Patients without a confirmed IgG deficiency have ceased IVIg therapy. With clinically active bronchiectasis or suppurative lung disease over the previous 12 months: To be eligible to receive IVIg for a further 12 months, the following is required: Written confirmation from the treating clinical immunologist that : an annual review has been undertaken; the patient has demonstrated clinical benefit; and a trial period of cessation of IVIg for the purpose of immunological evaluation is medically contraindicated on safety grounds. AND Written confirmation by a second physician that cessation of IVIg for the purpose of immunological evaluation is medically contraindicated on safety grounds. diagnosis of specific antibody deficiency should be confirmed following cessation of immunoglobulin therapy. Existing patient on IgG therapy funded under the National Blood Arrangements in Australia AND Clinically active bronchiectasis and/or suppurative lung disease proven by radiology (see note) AND The patient has had demonstrated clinical benefit over the last 12 months and has had at least 2 episodes of infection requiring antibiotics AND A cessation of therapy will be undertaken next September or October to undertake an immunological evaluation, unless a trial period of cessation of immunoglobulin therapy for the purpose of immunological evaluation is medically contraindicated on safety grounds (e.g. neutropenia, or other cytopenia, immunosuppressant medications or clinically
disease or bronchiectasis. An immunoglobulin washout period of four to six months is necessary to enable an accurate assessment. Prophylactic antibiotics may be considered to cover the period of IVIg cessation. Patients may qualify for further IVIg therapy: other immunodeficiency criteria (e.g. common variable immunodeficiency [CVID]) depending on the results of subsequent immune evaluation; or rarely under specific antibody deficiency following re-emergence of severe significant infection requiring hospitalisation. In principle, IVIg should only be continued or renewed if there is a demonstrated clinical benefit. Note that re-vaccination with pneumococcal polysaccharide vaccine is not recommended because of safety concerns, and the potential immunological re-evaluation, unless a trial period of cessation of intravenous immunoglobulin (IVIg) for the purpose of immunological evaluation is medically contraindicated on safety grounds (such as a patient with neutropenia, on immunosuppressant medication or with clinically active bronchiectasis and/or suppurative lung disease*). Please note*: a diagnosis of bronchiectasis and/or suppurative lung disease must be consistent with the position statement of the Thoracic Society of Australia and New Zealand and the Australian Lung Foundation (Chang et al 2010). Life-threatening infection or a series of significant infections following trial-off Ig therapy in patients with proven specific antibody deficiency. Natural history of specific antibody deficiency remains poorly defined, although antibody production will improve for many patients over time, particularly children.