Severe Pneumocystis jirovecii Pneumonia as the Sentinel Presentation of BENTA Disease: Case Report of a 3-month-old Infant

Pneumocystis jirovecii can cause severe pneumonia in HIV-negative infants with underlying immunodeficiency. We report an infant with prior acute respiratory distress syndrome and recurrent infections unresponsive to antibiotics. Persistent interstitial opacities led to immunologic testing, revealing T-cell lymphopenia and a CARD11 mutation. Respiratory polymerase chain reaction confirmed P. jirovecii . Treatment with trimethoprim–sulfamethoxazole and intravenous immunoglobulin led to recovery.