Introduction Immunodeficiencies (IDs) can be classified as inborn errors of immunity (IEIs) or secondary immunodeficiencies (SIDs) to other diseases or therapies. Their prevalence and diversity have increased in recent years due to earlier recognition and diagnosis, better medical education and greater access to molecular diagnosis. SIDs are also increasing due to the use of molecular targeted therapies.Aim: To show the changes in prevalence and adult immunodeficiencies profile over 40 years of follow-up.
Material and methods: Retrospective observational study of adult ID patients assisted in the Immunology Unit of Hospital C.G.Durand from 1982 to 2023 divided in two periods: from 1982 to 2020 period I (PI),and from 2020 to 2023 period II (PII). We collected epidemiological data, diagnosis according to 2022 IUIS classification and IDS causes. Results Two hundred and eighty four patients were recorded ,144 (50.7%) were women, mean age 41,4 years , r8-83. Two hundred and thirty four (82.4%) patients had IEI and 50 (17.6%) had SID. The most frequent IEIs were common variable immunodeficiency, IgA deficiency and X linked agammaglobulinemia. Non Classified IDs were observed in 9%. Molecular diagnosis was achieved in 14% versus 27 % of patients comparing periods.IEIs profile changed between periods with new molecular diseases. Rituximab was the main cause of SID in both periods since 2010, undergoing exponential growth in the last three years comprising 14% and 26% in each period.
There was an increase in patients assisted per year from 5 in PI to 26 in PII.
Conclusion: Prevalence of adult IDs is increasing with new diagnosis emerging. Molecular diagnosis and immunologic tests are essential tools for better recognizing and understanding of these disorders. SIDs in the era of targeted therapies has become a new and serious adult ID.