ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: TH-PO104

The Oxford IgA MEST Score Is Associated with Worse Kidney Outcomes in Childhood Onset Henoch Schönlein Purpura Nephritis

Session Information

Category: Glomerular

  • 1004 Clinical/Diagnostic Renal Pathology and Lab Medicine

Authors

  • Kellett, Sally Anne, The Hospital For Sick Children, Toronto, Ontario, Canada
  • Thorner, Paul S., The Hospital For Sick Children, Toronto, Ontario, Canada
  • Chami, Rose, The Hospital For Sick Children, Toronto, Ontario, Canada
  • Jawa, Natasha, The Hospital for Sick Children, Toronto, Ontario, Canada
  • Parekh, Rulan S., The Hospital For Sick Children, Toronto, Ontario, Canada
  • Noone, Damien Gerard, The Hospital for Sick Children, Toronto, Ontario, Canada
Background

Henoch-Schönlein Purpura (HSP) is one of the commonest forms of systemic small vessel vasculitis in children, with kidney involvement occurring in 40-50% of children. Current pathology classification systems have not proved useful in determining long-term kidney outcomes in children with HSP nephritis.

Aim: Determine if Oxford IgA MEST score is associated with long-term outcomes in childhood onset HSP.

Methods

This was a single-center, retrospective cohort study of children aged 0 to 18 yrs who presented with HSP nephritis between 2002 and 2016. Initial renal biopsies were scored based on the MEST criteria (M=mesangial hypercellularity, E=endocapillary hypercellularity, S=segmental glomerulosclerosis, T=tubular atrophy/interstitial fibrosis) and also for presence of crescents (C=cellular or fibrocellular crescents) and level of C3 deposition. Analyzed by logistic regression, we determined if MEST score was associated with clinical outcomes at last clinical follow-up with a composite outcome of proteinuria (protein:creatinine ratio >20mmol/mmol and/or eGFR <90ml/min/1.73m2) controlling for age at presentation and sex.

Results

We identified 44 children (F=63.6%) with biopsy data who were followed for at least 6 months. At presentation, median age was 8.3 yrs (IQR 5.8 – 11.5yrs) median protein:creatinine ratio was 239.6mg/mmol (IQR 151.5 – 608.9 mg/mmol). Median length of follow-up was 3 yrs (0.5 to 11yrs). Pathology review determined 34.1% had a combined MEST score ≥3, 63.6% had crescents (of those, 85.7% <50%) and 61.4% C3 staining >1+. A total of 79.5% were treated with steroids and 45.5% with an ACE inhibitor or angiotensin II receptor blocker. Median GFR at last follow up was 107.4ml/min/1.73m2 (IQR 83.6 to 146.2). A MEST score ≥3 had an adjusted odds of 8.3 [95% CI 1.3-52.2] of having a poor kidney outcome when adjusted for age and sex. The presence of crescents gave an adjusted odds of 5.4 [95% CI 1.2-25.5] of reaching the composite outcome. C3 was not significantly associated with the composite outcome with adjusted odds of 1.9 [95% CI 0.5-7.3].

Conclusion

MEST pathological score of ≥3 and presence of crescents on first biopsy in children with HSP nephritis is associated with a worse outcome by 3 years. This study suggests that Oxford IgA MEST score may be useful for disease prognosis.