Severe Pneumocystis jirovecii Pneumonia as the Sentinel Presentation of BENTA Disease: Case Report of a 3-Month-old Infant.
Andrés Felipe Rubio-Duarte, María Paula Garcés-Rodríguez, Alexandra Hurtado-Ortiz et al.
✦ AI-curated · Sources linked
Case in brief
A 3-month-old infant with a history of acute respiratory distress syndrome and recurrent infections that did not respond to antibiotics had persistent interstitial lung opacities. Immunologic evaluation showed T-cell lymphopenia and a CARD11 mutation, while respiratory polymerase chain reaction confirmed Pneumocystis jirovecii pneumonia. The infection was treated with trimethoprim-sulfamethoxazole and intravenous immunoglobulin, and the infant recovered.
What made this case unusual
P. jirovecii pneumonia was the sentinel presentation of BENTA disease in this infant, whose recurrent infections and persistent lung opacities prompted recognition of the underlying immunodeficiency.
Diagnostic / clinical pearl
In an HIV-negative infant with persistent interstitial opacities and recurrent infections unresponsive to antibiotics, consider an underlying immunodeficiency and investigate for P. jirovecii.