title: Experience with mycophenolate mofetil as maintenance therapy in five pediatric patients with severe systemic lupus erythematosus.
abstract: Maintenance therapy of severe pediatric systemic lupus erythematosus (SLE) usually consists of azathioprine and prednisone . In adult SLE patients mycophenolate mofetil (MMF) is successfully used, superiority to azathioprine has not been shown yet. We hypothesized that a maintenance therapy with MMF is able to decrease disease activity as well as the dose of glucocorticoid needed in children and adolescents with SLE. Five girls with a mean age of 13.9 (range 12-15) years were treated with 1.2+/-0.20 g/m (2) MMF daily on individual medical decision. Three patients had severe renal (WHO IV) and one severe cerebral involvement. Three patients with frequent flares on azathioprine maintenance therapy were switched to MMF, two patients with severe renal and cerebral manifestation received MMF additionally after induction therapy. Flares, steroid dosage, and disease activity (SLEDAI) were monthly registered in all patients. The number of flares decreased from 1.28 to 0.25 episodes per patient year during a mean follow-up period of 39 (range 36-42) months after MMF initiation. In parallel prednisone dose could be reduced from 10.80+/-5.25 to 3.25+1.18 mg/d (p<0.01). SLEDAI score dropped from 15.20+/-2.8 before MMF to 3.60+/- 0.9 at the last visit under MMF (p<0.001). No severe adverse event occurred. In our cohort of five pediatric patients MMF was effective and safe for maintenance therapy of SLE over a period of 3.5 years. MMF seems to be successful in preventing flares even in adolescents having unfavorable course on azathioprine treatment before. This observation should be confirmed by a randomized multicenter clinical trial.
authors.affiliation: Department of Pediatric Nephrology, University of Erlangen-Nürnberg, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Dittrich
authors.forename: S
authors.initials: S
authors.lastname: Ross
authors.forename: K
authors.initials: K
authors.lastname: Benz
authors.forename: K
authors.initials: K
authors.lastname: Amann
authors.forename: J
authors.initials: J
authors.lastname: Dötsch
mesh_terms: D000293:Adolescent; D001379:Azathioprine / Q000008:administration & dosage / Q000009:adverse effects; D002648:Child; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D008134:Long-Term Care; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000188:drug therapy*; D008181:Lupus Nephritis / Q000175:diagnosis / Q000188:drug therapy; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis / Q000188:drug therapy; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000009:adverse effects / Q000031:analogs & derivatives*; D011241:Prednisone / Q000008:administration & dosage / Q000009:adverse effects; D055502:Secondary Prevention
chemical_list: D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D001379:Azathioprine; D011241:Prednisone