Dental anomalies in congenital error of immunity

Developmental disorders of the teeth and maxillofacial region are part of a symptoms complex that often occur with inborn errors of the immune system. The purpose of the study is to identify the frequency of various dentofacial anomalies occurrence in patients with diagnosed immunodeficiencies. The study involved 64 patients of the Sverdlovsk Regional Clinical Hospital No. 1 and the Regional Children’s Clinical Hospital (Ekaterinburg) with various diagnoses: combined immunodeficiencies, antibody defects, autoinflammatory disorders, defects in the number and functions of phagocytes, defects in immune dysregulation, complement defects, and unverified diagnosis with signs of immunodeficiency. All patients underwent examination of the oral cavity, hard dental tissues, periodontal tissue and jaw bone tissue. It was found that an anomaly in jaw size is characteristic of all studied patient groups, except for the group with complement defects. Along with this, such pathology as quantitative and qualitative changes in teeth was identified in four classification groups: “combined immunodeficiencies” (25% of cases from the subgroup), “antibody defects” (31% of cases from the subgroup), “phagocyte defects” (10 % of cases from the subgroup), and “immune dysregulation defects” (50% of cases from the subgroup). The same frequency of occurrence of tissue repair defects: in “combined immunodeficiencies” (16.6% of cases from the subgroup), “autoinflammatory disorders” (26.7% of cases from the subgroup), “phagocyte defects” (10% of cases from the subgroup), in “unverified diagnoses” (20% of cases from the subgroup). The highest percentage of quantitative manifestations of such anomalies as jaw size (26% of cases from the entire study group) and facial dysmorphism (27% of cases from the entire study group). In patients with “combined immunodeficiencies”, all the dental anomalies described in the work were identified. However, the highest percentage of people in this group had delayed teething (33.3%) and facial dysmorphism (33%). The only patient diagnosed with “complement defects” had no dental anomalies. The minimum number of manifestations of the studied anomalies was observed in groups with “antibody defects” and “immune dysregulation defects”. The accumulated experience in describing various dental diagnoses will contribute to the creation of dental criteria for the diagnosis of primary immunodeficiencies.