An Unusual Case of Cystic Fibrosis Associated Pneumocystis jiroveci Pneumonia in an Infant

Abstract

Pneumocystis jiroveci pneumonia (PJP) is one of the major infections in patients with impaired immunity. The entity is common in HIV-seropositive individuals but quite very rare in HIV-seronegative individuals especially children. We report here a case of 16-week-old HIV-seronegative infant with chief complaint of chronic cough of one month of evolution. Sweat chloride test for diagnosis of cystic fibrosis was positive. Bronchoalveolar lavage (BAL) fluid was collected and Pseudomonas aeruginosa was isolated on culture. Empirical antibiotic regimen comprising ceftriaxone and azithromycin was initiated that was switched to meropenem as per antimicrobial susceptibility report, but the patient did not improve. Subsequently, an immunofluorescence staining of BAL fluid was performed and P. jiroveci cysts were detected. Following a laboratory confirmation of Pneumocystis pneumonia, cotrimoxazole was added and the clinical condition of the patient significantly improved. This is an unusual case wherein unsuspected PJP occurred and since signs and symptoms of the patient persisted even after the initiation of antimicrobial therapy for Pseudomonas infection and resolved only after treatment for PJP was started, it suggests a causative role of P. jiroveci rather than colonization/contamination.

Pneumocystis jirovecii in Patients With Cystic Fibrosis: A Review

“…No additional data on genotypes in a context of primary infection in CF infants are available. Indeed, the two case reports on severe primary infection that we mentioned above did not provide any information about genotypes (Royce and Blumberg, 2000 ; Kaur et al, 2016 ). Conversely, data exist on genotypes in non-CF infants developing primary infection (Totet et al, 2003a , b ; Nevez et al, 2020 ).…”

“…Although rare, PCP can also occur contemporaneously to Pneumocystis primary infection in CF infants (Royce and Blumberg, 2000 ; Kaur et al, 2016 ) and the medical community should be aware of this. Early impairment of pulmonary function due to the genetic disease, in P. jirovecii immune-naïve infants, in the apparent absence of immunosuppression, could be the cause of PCP occurrence.…”

“…BAL examination revealed P. jirovecii asci and P. aeruginosa . The patient recovered after cotrimoxazole treatment (Kaur et al, 2016 ). The young age of the two infants strongly suggested that they effectively developed Pneumocystis primary infection.…”

“…Further studies provided additional data in this field (Respaldiza et al, 2005 ; Le Gal et al, 2010 ; Hernandez-Hernandez et al, 2012 ; Pederiva et al, 2012 ; Green et al, 2016 ; Nevez et al, 2018 ). Moreover, PCP can also occur in CF patients (Royce and Blumberg, 2000 ; Quattrucci et al, 2005 ; Solé et al, 2006 ; Kaur et al, 2016 ; Wojarski et al, 2018 ). The objective of this review is to compile available information on P. jirovecii and CF worldwide.…”