Late-onset Pneumocystis jirovecii pneumonia (PJP) in patients with ANCA-associated vasculitis

Abstract

Immunosuppression in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is complicated by increasing risk of infections including opportunistic infections like Pneumocystis jirovecii pneumonia (PJP). Available evidence on risk factors and indications for prophylaxis in AAV is derived from PJP occurring early in the course of AAV. In this retrospective study, we characterized the profile of PJP in patients with AAV. PJP cases were identified retrospectively based on positive polymerase chain reaction test from electronic record followed by confirmation from medical records over a 10-year period. AAV patients without PJP over the same period were used as control group. Sixteen PJP+AAV+ were identified; in 14 of them, we were able to confirm they received PJP prophylaxis during induction therapy, while in two cases, data were missing. The onset of the infection was after 6 months from AAV diagnosis in 80% of cases. Escalations in immunosuppression prior to PJP were observed in six cases within 3 months prior to PJP onset. Overall mortality was 12.5%. By univariate analysis, renal involvement at AAV diagnosis was associated with PJP. These results indicate that PJP is not limited to the first 6 months following AAV diagnosis. Late-onset infection can occur in context of augmented immunotherapy, particularly with concurrent lymphopenia. Other risk factors that can independently predict late-onset PJP remain to be identified.

Duration of prednisone treatment before development of Pneumocystis jirovecii pneumonia in patients with vasculitis: A case series

“…Mortality from PCP was 29%, in keeping with estimates of 30%-60% among non-HIV-infected immunosuppressed are reported and often follow a recent flare and escalation of therapy without re-evaluation of PCP prophylaxis (9). There is also increasing evidence that rituximab is an independent risk factor for PCP (10).…”

Risk of Pneumocystis jirovecii Pneumonia among Solid Organ Transplant Recipients: A Population-Based Study

“…Patients with hematologic malignancies are known to have higher levels of immunosuppression [ 46 ], and the incidence of PJP has been increasing in subjects with hematologic malignancy [ 47 ]. Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were at higher risk for PJP infection [ 48 ].…”

Overview of infections as an etiologic factor and complication in patients with vasculitides

“…The distribution of infective sites includes primarily the lungs and the skin [7]. Opportunistic infections like Pneumocystis jirovecii pneumonia (PJP) occur early in the course of AAV, and it is frequently associated with the induction of immunosuppression [83]. However, PJP is not limited to the first six months following AAV diagnosis, and late-onset infection can occur in the context of augmented immunotherapy, particularly with concurrent lymphopenia [83].…”

“…Opportunistic infections like Pneumocystis jirovecii pneumonia (PJP) occur early in the course of AAV, and it is frequently associated with the induction of immunosuppression [83]. However, PJP is not limited to the first six months following AAV diagnosis, and late-onset infection can occur in the context of augmented immunotherapy, particularly with concurrent lymphopenia [83]. It should be noted that AAV treatment with rituximab has been associated with a high frequency of bacterial infections in a recently reported meta-analysis [84].…”