Objective, Although Pneumocystis carinii pneumonia (PCP) is known to o
ccur in adults with systemic lupus erythematosus (SLE), this infection
has rarely been described in childhood SLE, We describe 3 children wi
th SLE who developed PCP and describe risk factors for this complicati
on. Methods. A retrospective case review. Results. All 3 children had
severe active SLE with organ involvement requiring immunosuppressive t
herapy, but the clinical presentations of PCP differed in each patient
. They shared some of the known risk factors for opportunistic infecti
on ih adults with SLE, including lymphopenia, but severe lymphopenia (
< 0.35 x 10(9)/1) was not seen. Conclusion. PCP is an uncommon but ser
ious complication of childhood SLE, and should be considered in the pr
esence of respiratory symptoms, however subtle. The role of oral chemo
prophylaxis is discussed.