Managing hypertriglyceridemia in children with systemic lupus erythematosus: two sides of the same coin / Manejo de la hipertrigliceridemia en niños con lupus eritematoso sistémico: 2 caras de la misma moneda

Hypertriglyceridemia is common in children with systemic lupus erythematosus (SLE). A retrospective analysis of the baseline clinical-pathological presentation and treatment outcome (status of lipid profiles) was performed in two children with SLE, who presented with extreme hypertriglyceridemia over a follow-up period of four weeks. The children were treated with prednisolone, mycophenolate mofetil (MMF), hydroxychloroquine and hypolipidemic agents, depending on their disease status. On serial follow-up, the first child showed a significantly raised serum triglyceride level after receiving one week of oral prednisolone therapy. Anti-lipoprotein-lipase (LPL) autoantibody was absent. Lipid profile levels of this child gradually improved after replacing oral prednisolone with another immunosuppressant, namely MMF. The second child presented with extreme hypertriglyceridemia with positive anti-LPL autoantibody. She responded to plasmapheresis followed by increasing the dose of immunosuppressant. So, extreme hypertriglyceridemia in children with SLE may be steroid induced or due to presence of anti-LPL auto antibody. Management should be individualized depending on the etiology (AU)

La hipertrigliceridemia es común en niños con lupus eritematoso sistémico. Un análisis retrospectivo de la presentación clínico-patológica al inicio del estudio y los resultados del tratamiento (estado de los perfiles lipídicos) se llevó a cabo en 2 niños con lupus eritematoso sistémico, que presentaron hipertrigliceridemia extrema durante un período de seguimiento de 4 semanas. Los niños fueron tratados con prednisolona, micofenolatomofetil, hidroxicloroquina y agentes hipolipidemiantes, dependiendo de su estado de salud. En el seguimiento, el primer niño mostró un nivel de triglicéridos en suero significativamente elevado después de recibir una semana de tratamiento con prednisolona oral. No existían anticuerpos antilipoproteína lipasa. Los niveles de perfil de lípidos de este niño mejoraron gradualmente después de sustituir la prednisolona oral con otro inmunosupresor, a saber micofenolatomofetil. El segundo niño presentó hipertrigliceridemia extrema con autoanticuerpos antilipoproteína lipasa-positivos. Ella respondió a la plasmaféresis seguida del incremento de la dosis de inmunosupresores. Por lo tanto, la hipertrigliceridemia extrema en niños con lupus eritematoso sistémico puede ser inducida por esteroides o debido a la presencia de autoanticuerpos antilipoproteína lipasa. El manejo debe ser individualizado en función de la etiología (AU)

Managing hypertriglyceridemia in children with systemic lupus erythematosus: Two sides of the same coin.

Hypertriglyceridemia is common in children with systemic lupus erythematosus (SLE). A retrospective analysis of the baseline clinical-pathological presentation and treatment outcome (status of lipid profiles) was performed in two children with SLE, who presented with extreme hypertriglyceridemia over a follow-up period of four weeks. The children were treated with prednisolone, mycophenolate mofetil (MMF), hydroxychloroquine and hypolipidemic agents, depending on their disease status. On serial follow-up, the first child showed a significantly raised serum triglyceride level after receiving one week of oral prednisolone therapy. Anti-lipoprotein-lipase (LPL) autoantibody was absent. Lipid profile levels of this child gradually improved after replacing oral prednisolone with another immunosuppressant, namely MMF. The second child presented with extreme hypertriglyceridemia with positive anti-LPL autoantibody. She responded to plasmapheresis followed by increasing the dose of immunosuppressant. So, extreme hypertriglyceridemia in children with SLE may be steroid induced or due to presence of anti-LPL auto antibody. Management should be individualized depending on the etiology.

Efficacy and safety of rituximab in comparison with common induction therapies in pediatric active lupus nephritis.

BACKGROUND: Childhood-onset lupus nephritis (LN) is one of the most severe manifestations of systemic lupus erythematosus (SLE). Despite treatment-related toxicities, cyclophosphamide (CYC) and glucocorticoid-based treatment protocols are still considered standard therapy in managing this multisystem disorder. An effective and safe alternative induction regimen is needed. METHODS: Forty-four pediatric patients with active LN aged 3.5-13.8 (median 8.4) years, of whom 32 entered the study at diagnosis of SLE, were followed over 36 months. Induction therapy consisted of methylprednisolone pulses followed by either rituximab (RTX) (n = 17), mycophenolate mofetil (MMF) (n = 12) or pulse-CYC (n = 15), with tapering dose of prednisolone orally. MMF was added as maintenance immunosuppressant (800 mg/m2 daily) in all children from the third month onward. RESULTS: Flare-free survival was significantly higher at 36 months with RTX compared with MMF and CYC (100% for RTX vs. 83% for MMF. and 53% for CYC, p = 0·006). Twelve patients (76.5%) achieved complete remission with RTX compared with five (41.7%) and seven (46.7%) with MMF and CYC, respectively, at last follow-up. Requirement of mean daily dosage of prednisone was significantly lower in RTX group [p = 0.005 (RTX vs MMF); 0.0001 (RTX vs CYC) at 36 months] compared with other groups after the 3-month follow-up. In comparison with few minor adverse events in the other two cohorts, several serious adverse events occurred in the CYC group. CONCLUSIONS: Efficacy and medium-term safety of RTX induction followed by MMF maintenance therapy in inducing and maintaining remission among children with LN were evident in this study.

Long-term efficacy and safety of common steroid-sparing agents in idiopathic nephrotic children.

BACKGROUND: Calcineurin inhibitors (CNI), mycophenolate mofetil (MMF), and levamisole are common treatment choices for patients with frequently relapsing (FRNS) and steroid-dependent nephrotic syndrome (SDNS). METHODS: In this retrospective cohort study, we analyzed the relative efficacy and safety of tacrolimus, MMF, and levamisole over a period of 30 months, in treating 340 children with idiopathic FRNS/SDNS. The children received either MMF 1200 mg/m2 daily, or levamisole 2.5 mg/kg on alternate days, or tacrolimus 0.1-0.2 mg/kg daily along with tapering doses of alternate-day prednisolone. RESULTS: Tacrolimus was associated with a higher rate of 30-month relapse-free survival when compared to MMF (61.7 vs. 38.5 %, p < 0.001), or levamisole (61.7 vs. 24 %, p < 0.001). However, relapse rate increased almost threefold once tacrolimus was stopped, resulting in a higher relapse rate per patient-year when compared to the MMF group (2.0 vs. 1.5, p = 0.013). The cumulative prednisolone dose per patient during the last year of the study period was also increased among tacrolimus group in comparison with MMF group (96.4 vs. 74.4 mg/kg/year, p = 0.004). Independent of the impact of drug choice, the relapse risk was higher in patients with steroid dependency at baseline (HR 2.14, 95 %CI 1.79-2.96, p < 0.0001). In comparison with few minor adverse events in other two cohorts, several serious adverse events were documented in the tacrolimus group. CONCLUSIONS: Although there are serious safety concerns regarding tacrolimus, it is more effective than MMF or levamisole in maintaining relapse-free survival. However, unlike MMF, the relative efficacy of tacrolimus in preventing further relapses is seen only when the patient is on the drug. Taking together the long-term efficacy and safety data observed, MMF appears as a safe and effective alternative to tacrolimus in managing pediatric FRNS/SDNS.