Causes of death in pediatric systemic lupus erythematosus.

Causes of death in pediatric systemic lupus erythematosus Sirs, Pediatric systemic lupus erythematosus (pSLE) remains a severe disease although its prognosis has improved with time. Use of new therapies may result in modifying the causes of mortality. Death was mostly due to renal insufficiency and infections (1-4) during the 1980-1990 decade, while no death was recorded in the sole study enrolling children strictly after 1990 (5). The purpose of this study is to document the changes in causes of mortality in pSLE over the past 10 years in order to better prevent them. This French retrospective multicenter study included all pSLE patients followed in pediatric centers who died before the age 18 years during the 1996-2006 decade. All of them fulfilled the American College of Rheumatology (ACR) classification criteria for SLE, and were diagnosed before 16 years of age. A questionnaire was sent to the 44 pediatric nephrology and rheu-matology units of French university hospitals. The authors classified retrospectively the cause of death as organ involvement resulting from active uncontrollable SLE, thrombosis, therapy-related or infection-related event; the predominant cause was considered when there was some possible overlapping causes of mortality. Thirty-nine of the 44 (88%) centers responded to our questionnaire and recorded death in 12 patients, whom main demographic and clinical features are summarized in Table I. Death resulted from SLE organ involvement, including three cases of pancreatitis, thrombosis, infection in 5, 4 and 3 patients respectively. A therapy-related event was assessed in one patient who died during a conditioning regimen before autologous bone marrow transplantation (ABMT), and was suspected in another who had cerebral histoplasmosis, 5 months after being treated sequentially with rituxi-mab (RTX) and cyclophosphamide (Table I). His serum immunoglobulin G level (400 mg/dL) and lymphocyte count (1.1 x 10 9 /L) were low at time of death. The twelfth patient committed suicide. The present series emphasizes that pSLE remains a potentially fatal disease during the pediatric age. Unfortunately, the retrospective design of this multicenter study did not allow us to determine the mortality rates. Deaths were due to SLE exacer-bation, thromboses, infections, but also to new therapies related events. The most significant change in survival over time occurred in patients with renal involvement. Renal failure, responsible for most of the deaths before 1990 (1-5), was not a cause of mortality anymore. Conversely, within the last decade, new therapies proposed to SLE patients, including RTX and ABMT, result …