Prognosis
Prognosis is often dictated by the underlying condition. Despite this, early detection and treatment of hypogammaglobulinaemia does reduce morbidity rates and the chance of long-term pulmonary complications.[13] Evidence suggests an association between achieving higher IgG levels and reduced infection frequency.[63]
Primary immunodeficiency: primary antibody deficiency with severe reduction in IgG
Unfortunately, this diagnosis is often significantly delayed, and hypogammaglobulinaemia remains untreated.[64] This can lead to increased pulmonary complications due to recurrent pulmonary infections.[65] Treatment with immunoglobulin is likely to result in better survival, particularly if the hypogammaglobulinaemia is detected early.
Evidence regarding mortality is limited. Distinct clinical phenotypes of combined variable immunodeficiency (CVID), each with different survival rates, have been identified.[66] In one cohort study of 473 US patients diagnosed with CVID, the risk of death was 11 times higher among those with non-infectious complications than those with infectious complications alone.[67] The overall mortality of the cohort over a four-decade period was 19.6%.
Primary immunodeficiency: IgA deficiency/IgG subclass deficiency/impaired specific antibody production
Isolated IgA deficiency or IgG subclass deficiency is largely asymptomatic. If it is associated with symptoms, it tends to run a relatively mild course.
Impaired specific antibody production with normal total immunoglobulin levels also tends to be relatively benign, but a subgroup of these patients develop significant respiratory complications.
Primary immunodeficiency: combined immunodeficiency
Combined immunodeficiencies generally have a poorer outcome than primary antibody deficiency alone. There is susceptibility to a broader range of infections than with hypogammaglobulinaemia alone.
Severe combined immunodeficiency is usually fatal without definitive treatment in the form of haematopoietic stem cell transplantation and/or gene therapy. Adenosine deaminase deficiency is usually fatal without enzyme replacement therapy or gene therapy.
Secondary hypogammaglobulinaemia
Prognosis depends on the course of the underlying disease.
Hypogammaglobulinaemia can sometimes be resolved by addressing the underlying condition (e.g., by withdrawing causative medication).
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