Pneumocystis jiroveci pneumonia following rituximab treatment in Wegener's granulomatosis.
OBJECTIVE
Wegener's granulomatosis (WG) is a devastating small-vessel vasculitis in children. Standard treatment consists of immunosuppressive medications with cyclophosphamide potentially associated with significant infectious side effects, including Pneumocystis jiroveci pneumonia (PCP). Recently, rituximab, a monoclonal antibody against B cells, has successfully been used in refractory disease.


METHODS
We describe the first pediatric patient with refractory WG with sinus and lung disease who developed PCP 6 months after treatment with rituximab, while being treated with methotrexate and prednisone. This 9-year-old child had no CD20+ B cells at time of infection, with normal lymphocyte and CD4 counts.


RESULTS
This study provides a review of the published literature, including current protocols, which suggest chemoprophylaxis only in WG patients receiving T cell-targeted immunosuppression such as cyclophosphamide. However, clinical and laboratory evidence points toward a possible role of B cells in the defense against PCP.


CONCLUSIONS
Routine PCP chemoprophylaxis should be strongly considered in patients with WG treated with rituximab.
Hugle
Boris
B
The Hospital for Sick Children, University of Toronto, Ontario, Canada.
Solomon
Melinda
M
Harvey
Elizabeth
E
James
Adrian
A
Wadhwa
Anupma
A
Amin
Reshma
R
Bell-Peter
Audrey
A
Benseler
Susanne
S
D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects*; D002648:Child; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D006801:Humans; D045363:Pneumocystis carinii; D011020:Pneumonia, Pneumocystis / Q000139:chemically induced* / Q000175:diagnosis / Q000628:therapy; D000069283:Rituximab
D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab