Abstract: SA-PO258

Associated Clinical Characteristics in Anti-Neutrophil Cytoplasmic Antibody Negative, Pauci-Immune Crescentic Glomerulonephritis: Results of a Case Series at a Tertiary Care Center

Session Information

Category: Glomerular

  • 1005 Clinical Glomerular Disorders

Authors

  • Amaechi, Prince, Medical University of South Carolina, Charleston, South Carolina, United States
  • Bruner, Evelyn, Medical university of south carolina, Charleston, South Carolina, United States
  • Budisavljevic, Milos N., Medical University of South Carolina----, Charleston, South Carolina, United States
Background

Pauci-immune crescentic glomerulonephritis (PCGN) is the most common cause of rapidly progressive glomerulonephritis in adults and elderly patients. Approximately 10-30% of patients with PCGN have anti-neutrophil cytoplasmic antibody (ANCA) negativity. However the etiology of idiopathic ANCA-negative PCGN has never been clearly characterized besides a few case reports. We therefore conducted a case series of patients in our tertiary care center diagnosed with pauci-immune crescentic glomerulonephritis in the last 33 years to see if there was any specific associated factors.

Methods

We looked at all the patients in our hospital that had a diagnosis of pauci-immune crescentic glomerulonephritis from November 1994- June 2017. Those with additional diagnosis such as glomerular basement membrane disease or other autoimmune disorders were excluded. We identified 59 patients however, some had incomplete records and they were further excluded. The final number was 14 patients, of which 10 were ANCA- positive and 4 were ANCA-negative. Their medical records were evaluated for any associated medical conditions.

Results


Out of the 4 patients who were ANCA-negative, all of them had an infection that preceded their illness and that was clearly present at the time of renal failure diagnosis (100%); whereas, only 2 out of the 10 who were ANCA-positive had an associated infection with their illness (20%). In this small series of patients, the two categories of patients had a similar severity of illness as estimated by their Sequential Organ Failure (SOFA) Assessment Scores (averaging 3.6 and 3 respectively).

Conclusion

100% of our patients with a diagnosis of ANCA-negative PCGN had associated infections preceding their illness, whereas this was true of only 20% of the ANCA-positive ones. The infections involved included BK virus, Parvovirus, Staph aureus pneumonia and streptococcal pneumonia. Our results suggest that there is a positive link between otherwise idiopathic ANCA-negative crescentic pauci-immune glomerulonephritis and a preceding episode of infection.

Relationship between ANCA positive vs negative with infection
ANCA- positive (+) infection = 2ANCA- negative (+) infection= 4
ANCA-positive (-) infection = 8ANCA- negative (-) infection = 0

ANCA- positive total n= 10, ANCA-negative total n= 4