Manifestações orais e dentárias em pacientes com deficiências de fagócitos: uma revisão sistemática da literatura científica

Phagocytic cells are essential elements in the host reponse to a wide variety of pathogens. In Primary Immune Deficiency (PID) diseases, quantitative or qualitative defects in the development of the immune system, affecting phagocytes, account for an increase in the number of severe infections in infancy and childhood, which may involve, among other sites, the oral cavity. Despite their great clinical relevance, in view of the diverse manifestations and chronicity of the disease, PID present important practical difficulties for the nonspecialist practicioner, due to their relative scarcity in the general population, great heterogeneity in pathogenetic mechanisms, and diversity of presentation. Therefore, the frequency and nature of oral manifestations depend on the nature of the defect in immunity, varying considerably among specific PID. To our knowledge, there is no systematic study of the existing scientific literature with respect to oral manifestations in different subtypes of PID. In this study, we attempted to fill this gap, by carrying out a systematic review of case reports of PID affecting phagocytes, and establishing the frequency of the different oral and dental manifestations in this group of PID, with the goal of providing dental health professionals with more accurate information concerning these patients. Results: Case reports describing 1721 patients enabled us to detect reports of oral or dental manifestations in the clinical description of 653 pacients (37,9%). Periodontal disease was the most frequent oral manifestation (54,5%), followed by the early loss of decidual teeth, which was found in 142 pacients (21,7%) and gingivitis, found in 72 pacients (11,0%). Ulcerations (8,1%) and oral candidiasis (7,5%) were also reported. This analysis also provided evidence that recent advances in biomedical research, with an increasing focus on molecular analyses, significantly influenced the content of case reports, which are nowadays more often focused on the identification of mutated genes, rather than on the detailed description of clinical findings. As a result, online and computer-assisted information retrieval strategies do not necessarily recover the same references, when articles are searched on the basis of clinical descriptions, or on the basis of well-characterized molecular defects.