Autoimmune manifestations of primary minor immunodeficiencies (PMDs)—highly prevalent conditions that may represent a population-level model of autoimmunity—have not been systematically analyzed. The study aims to evaluate the current evidence for associations between PMDs and autoimmune syndromes. A systematic search of PubMed (MEDLINE) for 1980–2025 was conducted using keywords related to PMDs and autoimmune syndromes. The search was performed in two stages: an initial broad screening and a subsequent refined search with specific disease-related terms. High-quality peer-reviewed studies most relevant to the study objectives were selected. Available evidence indicates that PMDs commonly manifest through autoimmune syndromes according to a principle of universality, with important geographic, ethnic, age-related, sex-related, and ontogenetic variation. The strength of PMD–autoimmunity associations varies widely, reflecting major heterogeneity in both disease mechanisms and study quality. Key factors influencing clinical and epidemiological findings include terminological inconsistencies, multiple autoimmune pathways, heterogeneity of PMD origin and evolution, overlap with other immunodeficiencies and comorbidities, variability of clinical phenotypes, difficulties distinguishing PMDs from secondary immunosuppression, and the absence of standardized diagnostic criteria for many PMDs. Elementary models of autoimmune induction in immunocompromised individuals are proposed, together with a framework for personalized assessment of autoimmune disease in the context of PMDs, and implications for rational immunotherapy are discussed. PMDs are associated with diverse autoimmune syndromes and likely represent a major etiological factor of human autoimmunity at the population level. However, this data should be validated in further studies.
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