Adult-onset primary immunodeficiencies

Der Internist
S GadolaB Grimbacher

Abstract

Different individuals with the same kind of primary immunodeficiency may start having symptoms from early childhood on, or alternatively much later in adult life, or never. The differences in phenotype can only partly be deduced from genotype-analysis or--in case of female patients with X-linked diseases--from age-related skewing of lyonisation. The role of compensatory immune mechanisms is less clear. The microbial spectrum of infections is usually the same for both adult and infantile forms of a special primary immunodeficiency syndrome. Yet, many of the adult forms are associated with non-infectious complications, such as granuloma formation, autoimmunity or tumors. Besides standard antibiotic treatment and IgG replacement therapy, there are now different cytokine- or enzyme-replacement regimens available for some of the primary immunodeficiencies. However, exact diagnostic classification of the immunodeficiency should be obtained before such treatment modalities are used. Adult primary immunodeficiency syndromes therefore represent a challenge to both clinicians and molecular biologists.

Citations

Sep 1, 2005·Journal of Clinical Immunology·Davood MansouriStuart E Turvey
Jan 6, 2015·Expert Review of Clinical Immunology·Hassan AbolhassaniAsghar Aghamohammadi
Dec 6, 2019·Frontiers in Immunology·Claudia SchröderFaranaz Atschekzei

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