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: SA-PO649

Histopathological Findings and Clinical Outcomes of ANCA-Negative vs. ANCA-Positive Pauci-Immune Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Morris, Adam, Royal Preston Hospital, Lancashire NHS Foundation Trust, Preston, United Kingdom
  • Elsayed, Mohamed, Royal Preston Hospital, Lancashire NHS Foundation Trust, Preston, United Kingdom
  • Floyd, Lauren, Royal Preston Hospital, Lancashire NHS Foundation Trust, Preston, United Kingdom
  • Brady, Mark, Royal Preston Hospital, Lancashire NHS Foundation Trust, Preston, United Kingdom
  • Ponnusamy, Arvind, Royal Preston Hospital, Lancashire NHS Foundation Trust, Preston, United Kingdom
  • Dhaygude, Ajay Prabhakar, Royal Preston Hospital, Lancashire NHS Foundation Trust, Preston, United Kingdom
Background

It has been suggested that pauci-immune small vessel vasculitis exhibits a different spectrum of disease in the absence of detectable ANCA. We investigated differences in clinical phenotypes, renal histology and clinical outcomes amongst patients with or without ANCA positive disease.

Methods

A cohort with biopsy proven pauci-immune glomerulonephritis was constructed from a single centre between 2006-2016 and followed until December 2018. Patients were stratified by ANCA status at the time of diagnosis with comparative analysis of demographics, clinical characteristics and histopathological variants. Multivariate survival and logistic models compared remission rates, ESRD and mortality risk.

Results

From 143 patients, 20% (n=29) had ANCA negative disease with a male predominance of 66% (n=19) and a younger mean age at diagnosis compared to those with positive ANCA serology; 53+19 years vs. 66+12 years respectively (p=<0.001). The presence of extra-renal disease with ENT and constitutional symptoms occurred more frequently in ANCA positive patients; 25% (n=27) vs. 0% (p=0.0012) and 27% (n= 30) vs. 3% (n=1) (p=0.0049) respectively. Conversely, ANCA negative serology was associated with renal limited disease (p=0.039). There was no association between histological features on biopsy and ANCA serology (Table 1). ANCA positive disease was associated with a significantly higher remission rate [OR 3.9 (95% CI 1.12-13.5)]. However, this did not confer any renal or overall survival benefit; [HR 0.45 (95% CI 0.15 – 1.33)] for ESRD and [HR 0.68 (95% CI 0.25 – 1.81)] for death.

Conclusion

Our single centre experience suggests that ANCA negative disease tends to occur in younger patients, with a higher rate of renal limited disease and no significant differences in histopathologial variants. Seronegative disease is less likely to remit, but is associated with similar survival risks in compassion to ANCA positive disease.

Table 1: Histopahtological Variants by ANCA status
Histological featureANCA negativeANCA positivep-value
< 10% Normal Glomeruli10 (34.48%)22 (19.30%)0.22
Berden Classification:
Crescentic class

Sclerosed class

Focal class

Mixed class
.
3 (10.34%)

2 (6.90%)

13 (44.83%)

11 (37.93%)
.
21 (18.42%)

5 (4.39%)

47 (41.23%)

41 (35.96%)
.
0.73

0.73

0.73

0.73
Extra-glomeruli arteritis4 (13.79%)16 (14.04%)1
Vessel wall necrosis4 (13.79%)11 (9.65%)0.51
>50% IFTA03 (2.63%)0.53