title: Incidence and prognosis of systemic lupus erythematosus in a 35 year period in Fukushima, Japan.
abstract: BACKGROUND
  We examined the epidemiology, clinical manifestations, and prognosis of pediatric systemic lupus erythematosus (SLE) in Fukushima Prefecture, Japan over a 35 year period.
  
  
  METHODS
  We collected the medical records of 37 patients diagnosed with SLE between 1977 and 2013. These children were divided into two groups. group 1 consisted of 19 patients who were diagnosed between 1977 and 1995, and group 2 consisted of 18 patients diagnosed between 1996 and 2013. The epidemiology, clinical features, and prognosis were retrospectively compared between the two groups.
  
  
  RESULTS
  The mean number of patients per 100,000 children per year for group 1 and group 2 was 0.33 ± 0.25 and 0.35 ± 0.30, respectively. The duration from onset of symptoms to treatment in group 2 was shorter than that in group 1, but the clinical and laboratory findings at onset did not differ between the two groups. All patients were treated with prednisolone, and 17 patients in group 1 and 18 in group 2 were treated with methylprednisolone pulse therapy. The frequency of cyclophosphamide treatment decreased whereas the frequency of cyclosporine, tacrolimus and mizoribine pulse therapy increased in group 2. SLE disease activity index (SLEDAI) score at the latest follow up in group 2 was lower in group 1. The survival rate was 84% in group 1 and 100% in group 2.
  
  
  CONCLUSIONS
  The frequency and severity of SLE in group 1 were similar to those in group 2, and the prognosis of SLE in group 2 was better than that in group 1.
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Kawasaki
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Ohara
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Kyohei
authors.initials: K
authors.lastname: Miyazaki
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Shuto
authors.initials: S
authors.lastname: Kanno
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Ono
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Suyama
authors.affiliation: Department of Pediatrics, Some General Hospital, Fukushima, Japan.
authors.forename: Masatoki
authors.initials: M
authors.lastname: Sato
authors.affiliation: Department of Pediatrics, Ohara General Hospital, Fukushima, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Suzuki
authors.affiliation: Department of Pediatrics, Hoshi General Hospital, Fukushima, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Maeda
authors.affiliation: Department of Pediatrics, Takeda General Hospital, Fukushima, Japan.
authors.forename: Katsutoshi
authors.initials: K
authors.lastname: Nagasawa
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Hosoya
mesh_terms: D017668:Age of Onset; D001706:Biopsy; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate / Q000639:trends
keywords: Bredinin; clinical manifestation; epidemiology; methylprednisolone; systemic lupus erythematosus