title: [Late onset of primary immune deficiencies].
abstract: Primary immune deficiencies (PID) are characterized by a failure of the immune system that is not explained by any infectious, neoplastic, or iatrogenic cause. The diagnosis of PID should be considered in cases of severe or recurrent infections but also in cases with granulomatosis, autoimmune diseases, hemophagocytic syndrome, lymphoproliferative disorders, or even some solid tumors. The onset of PID may be late, most often in adulthood. Nonetheless, late onset may also mean in the first years rather than months of life or in adolescence rather than early childhood. In adults, the diagnosis of PID cannot be considered before acquired immunodeficiencies--far more frequent--are ruled out. Factors affecting the late onset of PID are not known.
authors.affiliation: Université Paris Descartes, Faculté de médecine, Pôle de médecine interne, Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, F-75679 Paris Cedex 14, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Bussone
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency; D000328:Adult; D000361:Agammaglobulinemia / Q000235:genetics; D017668:Age of Onset; D056735:Autoimmune Lymphoproliferative Syndrome / Q000235:genetics; D015704:CD4 Antigens / Q000235:genetics; D003937:Diagnosis, Differential; D016654:Genes, RAG-1 / Q000235:genetics; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D007239:Infections / Q000209:etiology; D008231:Lymphopenia / Q000235:genetics; D009154:Mutation / Q000235:genetics; D009503:Neutropenia / Q000209:etiology; D016884:Polyendocrinopathies, Autoimmune / Q000235:genetics; D012008:Recurrence; D012307:Risk Factors; D016511:Severe Combined Immunodeficiency / Q000235:genetics
chemical_list: D015704:CD4 Antigens; D000243:Adenosine Deaminase