RT Journal Article T1 Pneumocystis jirovecii in Patients With Cystic Fibrosis: A Review. A1 Bonnet, Pierre A1 Le Gal, Solène A1 Calderon, Enrique A1 Delhaes, Laurence A1 Quinio, Dorothée A1 Robert-Gangneux, Florence A1 Ramel, Sophie A1 Nevez, Gilles K1 Pneumocystis jirovecii K1 Pneumocystis pneumonia K1 Pneumocystis primary infection K1 cystic fibrosis K1 genomic diversity K1 lung transplantation K1 microbiota K1 pulmonary colonization AB Pneumocystis pneumonia (PCP) remains the most frequent AIDS-defining illness in developed countries. This infection also occurs in non-AIDS immunosuppressed patients, e.g., those who have undergone an organ transplantation. Moreover, mild Pneumocystis jirovecii infections related to low pulmonary fungal burden, frequently designated as pulmonary colonization, occurs in patients with chronic pulmonary diseases, e.g., cystic fibrosis (CF). Indeed, this autosomal recessive disorder alters mucociliary clearance leading to bacterial and fungal colonization of the airways. This mini-review compiles and discusses available information on P. jirovecii and CF. It highlights significant differences in the prevalence of P. jirovecii pulmonary colonization in European and Brazilian CF patients. It also describes the microbiota associated with P. jirovecii in CF patients colonized by P. jirovecii. Furthermore, we have described P. jirovecii genomic diversity in colonized CF patients. In addition of pulmonary colonization, it appears that PCP can occur in CF patients specifically after lung transplantation, thus requiring preventive strategies. In other respects, Pneumocystis primary infection is a worldwide phenomenon occurring in non-immunosuppressed infants within their first months. The primary infection is mostly asymptomatic but it can also present as a benign self-limiting infection. It probably occurs in the same manner in CF infants. Nonetheless, two cases of severe Pneumocystis primary infection mimicking PCP in CF infants have been reported, the genetic disease appearing in these circumstances as a risk factor of PCP while the host-pathogen interaction in older children and adults with pulmonary colonization remains to be clarified. YR 2020 FD 2020-09-29 LK http://hdl.handle.net/10668/16499 UL http://hdl.handle.net/10668/16499 LA en DS RISalud RD Feb 14, 2025