Pneumocystis jirovecii Pneumonia in Neurologic Disorders

Abstract

Background:The incidence of Pneumocystis jirovecii pneumonia (PJP) in patients with underlying neurological conditions is not well established, and the necessity of PJP prophylaxis for immunocompromised patients with neurological disorders is uncertain.Methods:Single-center retrospective analysis of non-HIV PJP patients at a tertiary referral center from 2007-2016 to determine the incidence of PJP in patients with primary neurological disordersResults:The study included 142 non-HIV PJP patients. Twenty patients had primary neurological diagnoses and 122 had non-neurological conditions. Associated neurological diagnoses included brain malignancies (N=16), myasthenia gravis (MG) (N=2), myopathy (N=2), neuromyelitis optica (NMO) (N=1) and central nervous system vasculitis (N=1). Estimated incidences of PJP were 0.7% for NMO and 0.3% for MG patients for the 10-year study period, while 4.6% of NMO and 3.8% of MG patients 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 neurological PJP patients and coexisting infections occurred in 45%.Conclusion:The overall incidence of PJP in patients with non-neoplastic neurological disorders is exceedingly low, raising doubt about the value of routine PJP prophylaxis in neurological 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.

Opinions on Pneumocystis jirovecii prophylaxis in autoimmune neuromuscular disorders

“…Available survey data, however, suggest that routine PJP prophylaxis is not a common practice among neurologists. 16 In large clinical trials evaluating the effectiveness of immunomodulating therapies in patients with MG (ie, REGAIN, ADAPT, and MGTX) and chronic inflammatory demyelinating polyneuropathy (CIDP; ie, ICE and PATH), not a single case of PJP was reported, which we have further confirmed in personal correspondences with study authors and sponsors. Thus, the infrequent occurrence of PJP in autoimmune neuromuscular disorders is likely reflective of a low baseline risk.…”

“…Others may argue that this low risk of PJP in autoimmune neuromuscular disorders reflects widespread use of prophylaxis in these patients. Available survey data, however, suggest that routine PJP prophylaxis is not a common practice among neurologists 16 . In large clinical trials evaluating the effectiveness of immunomodulating therapies in patients with MG (ie, REGAIN, ADAPT, and MGTX) and chronic inflammatory demyelinating polyneuropathy (CIDP; ie, ICE and PATH), not a single case of PJP was reported, which we have further confirmed in personal correspondences with study authors and sponsors.…”

“…The presence of a comorbid disease, such as other organ dysfunction, active cancer status, advanced age, and especially interstitial lung disease, appears to heighten PJP risk 16,31,40 . A high prevalence of parenchymal organ failure was observed in patients with PJP 16 . Coexisting pulmonary diseases, especially interstitial pulmonary fibrosis, appear to increase PJP risk in immunocompromised patients 36,39,40,44 .…”

“…16,31,40 A high prevalence of parenchymal organ failure was observed in patients with PJP. 16 Coexisting pulmonary diseases, especially interstitial pulmonary fibrosis, appear to increase PJP risk in immunocompromised patients. 36,39,40,44 The noted high incidence of coexisting infections (eg, cytomegalovirus infection) in patients with PJP as well as low albumin levels also argues that patients at risk for PJ infections are generally systemically ill. 16,33,44 6 | RISKS OF PJP PROPHYLAXIS Prophylaxis with TMP-SMX is generally well tolerated and effective, reducing occurrence of PJP by 85% or more.…”

“…In rheumatologic patients, the estimated overall PJP incidence is approximately 2% to 3%, being highest for systemic vasculitic conditions like granulomatosis with polyangiitis (1.5% to 30%) and much lower for rheumatoid arthritis (0.1% to 0.3%) 10‐15 . Available data suggest that the incidence of PJP in patients with myasthenia gravis (MG) and nonsystemic autoimmune neuropathies is less than 1%, whereas the incidence in autoimmune myopathies is less than 2% 16‐22 …”