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Abstract: SA-PO444

Clinicopathological Significance in Juvenile-Onset Silent Lupus Nephritis

Session Information

  • Pediatric Nephrology - II
    October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1600 Pediatric Nephrology

Authors

  • Shima, Yuko, Wakayama Medical University, Wakayama City, Japan
  • Nakanishi, Koichi, Graduate School of Medicine, University of the Ryukyus, Nishihara-cho, Japan
  • Hama, Taketsugu, Wakayama Medical University, Wakayama City, Japan
  • Sato, Masashi, Wakayama Medical University, Wakayama City, Japan
  • Mukaiyama, Hironobu, Wakayama Medical University, Wakayama City, Japan
  • Tanaka, Yu, Wakayama Medical University, Wakayama City, Japan
  • Kaito, Hiroshi, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyōgo, Japan
  • Nozu, Kandai, Dept. of Pediatrics, Kobe Univ. Graduate School of Medicine, Kobe, Japan
  • Tanaka, Ryojiro, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyōgo, Japan
  • Suzuki, Hiroyuki, Wakayama Medical University, Wakayama City, Japan
  • Iijima, Kazumoto, Dept. of Pediatrics, Kobe Univ. Graduate School of Medicine, Kobe, Japan
  • Yoshikawa, Norishige, Wakayama Medical University, Wakayama City, Japan
Background

In patients with juvenile-onset systemic lupus erythematosus (SLE), there have been reports of silent lupus nephritis (SLN) patients without abnormal urinalysis and renal impairment. Currently, renal biopsies are the gold standard for diagnosis and monitoring of LN. The purpose of this study is to clarify the clinicopathological significance of juvenile-onset SLN.

Methods

Retrospective analysis of 57 patients who underwent renal biopsy among 59 patients diagnosed as juvenile-onset SLE who met the American College of Rheumatology criteria for the classification of SLE between December 2000 and March 2017 to compare clinical and pathological findings between SLN and others.

Results

There were 27 patients (47.4%) with SLN. Clinical findings showed significant differences (SLN vs. non-SLN) in eGFR (174.9 vs 147.5 ml/min/1.73m2, p=.02) and serum C3 level (76.0 vs 46.5 mg/dl, p=.04). The distribution of ISN/RPS classes of LN was as follows: class I: II: III: IV: V (12: 11: 3: 1: 0 vs 2: 7: 6: 14: 1; p=.003). Though SLN group showed milder pathological presentation, 4 cases with SLN showed severe pathological findings such as class III or IV. The 4 patients with class III and IV had significantly higher dsDNA antibody titer (159.2 vs 42.1 IU/ml, p =.02) and lower serum C3 level (37 vs 77 mg/dl, p =.03) than the other SLN cases. As to immunofluorescence, all patients showed the deposition of immunoglobulins and the degree of deposition of IgG and C3 in SLN was significantly weaker than in non-SLN. In electron microscopy, the degree of subendothelial and subepithelial EDD was significantly weaker in SLN.

Conclusion

Most of the patients with SLN showed milder pathological presentation, but some cases showed severe pathological presentation regardless of minor clinical presentations. We confirmed an importance of renal biopsies for juvenile-onset SLE. Serum C3 level might be an important marker of the severity of SLN.