Gastrointestinal Manifestations of Primary Immunodeficiency Disorders / 胃肠病学

ABSTRACT

Primary immunodeficiency disorders (PIDDs) contain more than 300 inborn errors of immunity, among which common variable immunodeficiency (CVID) and chronic granulomatous disease (CGD) are relatively common in clinical practice. CVID is characterized by loss of B cell function and impaired antibody production, while CGD is mainly a congenital disorder with defective neutrophil function. In both diseases, the clinical features include an increased susceptibility to infections and may have symptoms resembling non-infectious inflammatory disorders such as inflammatory bowel disease (IBD). The endoscopic and pathologic findings are non-specific and often mimic IBD, gene analysis is important for differential diagnosis. Treatment is not well defined and is mainly empirical. Infections should be treated with broad-spectrum agents covering both bacterial and fungal pathogens. Corticosteroids or immunosuppressors can be used for PIDDs patients with IBD manifestations.