Pneumocystis jirovecii Pneumonia in Neurologic Disorders Is Prophylaxis Necessary?

Background The incidence of Pneumocystis jirovecii pneumonia (PJP) in patients with underlying neurologic conditions is not well established, and the necessity of PJP prophylaxis for immunocompromised patients with neurologic disorders is uncertain. Methods Single-center retrospective analysis of non-HIV PJP patients at a tertiary referral center from 2007 to 2016 to determine the incidence of PJP in patients with primary neurologic disorders. Results The study included 142 patients with PJP without HIV. Twenty patients had primary neurologic diagnoses, and 122 had non-neurologic conditions. Associated neurologic diagnoses included brain malignancies (N = 14), myasthenia gravis (MG) (N = 2), myopathy (N = 2), neuromyelitis optica (NMO) (N = 1), and CNS vasculitis (N = 1). Estimated incidences of PJP were 0.7% for patients with NMO and 0.3% for patients with MG for the 10-year study period, whereas 4.6% of patients with NMO and 3.8% of patients with MG were placed on PJP prophylaxis. A survey of 24 neurologists who prescribe immunotherapy or chemotherapy confirmed an infrequent occurrence of PJP in their practice. Malignancy or parenchymal organ failure was present in 90% of neurologic patients with PJP, and coexisting infections occurred in 45%. Conclusions The overall incidence of PJP in patients with non-neoplastic neurologic disorders is exceedingly low, raising doubt about the value of routine PJP prophylaxis in neurologic patients outside neuro-oncology. PJP infection occurs frequently in patients with malignancy or parenchymal organ failure, indicating that overall health status may serve as a predisposing factor for PJP.