Inborn errors of immunity and related microbiome

Area of Science:

• Immunology
• Microbiology
• Genetics

Background:

• Inborn errors of immunity (IEI) present with varied symptoms, including infections, autoimmunity, and malignancy, significantly impacting patient health.
• Emerging evidence suggests a strong correlation between IEI clinical features and alterations in the gut microbiota, known as dysbiosis.
• Dysbiosis in IEI involves an imbalance of gut bacteria, with an increase in pro-inflammatory species and a decrease in beneficial ones.

Purpose of the Study:

• To explore the relationship between clinical manifestations of Inborn Errors of Immunity and gut microbiota composition.
• To highlight the prevalence and impact of microbial dysbiosis in various specific IEI conditions.
• To underscore the potential of microbiota modulation as a therapeutic and preventive strategy for IEI.

Main Methods:

• Review of existing literature correlating IEI phenotypes with gut microbiome alterations.
• Identification of specific IEI where dysbiosis is a well-established feature.
• Analysis of the role of gut microbiota in host immunity and infectious disease susceptibility in IEI patients.

Main Results:

• Dysbiosis is frequently observed in numerous IEI, including common variable immunodeficiency, severe combined immunodeficiency, and autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy (APECED).
• Gastrointestinal, respiratory, and cutaneous manifestations in certain IEI are often associated with dysbiosis, prompting interest in microbiome analysis.
• The understanding of the gut microbiota's role in immunity supports its potential as a therapeutic target.

Conclusions:

• Microbiota modulation, including prebiotics, probiotics, postbiotics, and fecal microbial transplantation, presents a promising avenue for rebalancing the gut microbiota.
• These interventions aim to reduce disease activity and prevent infections in patients with Inborn Errors of Immunity.
• Targeting the gut microbiome offers a novel and relevant strategy for managing the complex challenges posed by IEI.