A Case of Fatal Cytomegalovirus Pneumonia Following Recurrent Organizing Pneumonia after Mild COVID-19 in a Patient with X-linked Agammaglobulinemia

Abstract

 A 46-year-old male with X-linked agammaglobulinemia（XLA）and bronchiectasis developed COVID-19. Initially, his condition improved with molnupiravir. However, he presented to our hospital with respiratory failure on day 16 of illness. Despite receiving antibiotic therapy, his respiratory condition worsened, which led to a diagnosis of organizing pneumonia. He then received steroid pulse therapy followed by 10 days of veno-venous extracorporeal membrane oxygenation （ECMO). Although he was successfully weaned off ECMO, subsequent steroid tapering led to recurrent acute respiratory distress syndrome (ARDS) secondary to worsening organizing pneumonia. The patient ultimately died of respiratory failure on day 93 of illness. Autopsy showed no chronic histological changes but rather findings of acute-phase inflammation. Immunohistochemical staining was positive for cytomegalovirus (CMV) antigen, leading to the diagnosis of CMV pneumonia as the direct cause of death. CMV antigen tests were negative on days 35, 50, and 63 of illness. However, no such tests were conducted during his final deterioration from day 80 onward. COVID-19 testing during this hospitalization, including nasopharyngeal PCR and a FilmArray respiratory panel from bronchial lavage, was consistently negative. In the absence of other identifiable causes for organizing pneumonia, it was considered a likely complication of the initial COVID-19 infection. Bronchiectasis is known to be associated with poorer responses to steroid therapy for organizing pneumonia. While COVID-19 in XLA patients is generally reported to be mild, this case highlights the potential for severe and even fatal complications, emphasizing the need for careful monitoring.