ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

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-PO439

Granulomatosis With Polyangiitis (GPA) Presenting as a Renal Mass in a Young Man With a Solitary Kidney

Session Information

Category: Glomerular Diseases

  • 1301 Glomerular Diseases: Fibrosis and Extracellular Matrix

Authors

  • Abudaff, Naief N., Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Patel, Sagar, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Fogo, Agnes B., Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Sanghani, Neil S., Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction

GPA is a rare autoimmune ANCA-associated vasculitis. GPA has varied extrarenal presentations including fevers, arthralgias, sinusitis, and respiratory failure from diffuse alveolar hemorrhage. In the kidney, it classically presents as RPGN with the quadrad of increased creatinine, microscopic hematuria, proteinuria and hypertension. Here, we describe a rare presentation of GPA with a renal mass.

Case Description

A 20M with a solitary left kidney presented due to persistent fevers and AKI. He had a one month history of fevers, arthralgias, nosebleeds, left flank pain, weight loss, and had been treated for presumed UTI.
Vital signs were T 101.7F, HR 109 bpm, and BP 148/78mmHg. Urinalysis was positive for heme and albumin without any nitrites with 38 WBCs and 138 RBCs, urine protein/creatinine ratio was 1, creatinine 2.7 mg/dL (from 1.1 mg/dL), CRP 173 mg/L, ESR 140 mm/hr.
Renal ultrasound showed a 16.5cm left kidney with a small amount of perinephric fluid and a complex fluid collection, 8.5x7.3x4.9cm, in the left upper pole, concerning for pyelonephritis and abscess. He was treated with IV antibiotics and percutaneous abscess drainage was planned. CT showed an atrophic right kidney with chronic UPJ obstruction and a left upper pole lesion that appeared more mass-like [A].
Biopsy of the mass was performed, revealing extensive necrosis, including vasculitis of arteries [B] and pauci-immune necrotizing crescentic glomerulonephritis [C]. Anti-PR3 was then detected. Urine, blood and tissue cultures returned negative.
The patient was diagnosed with GPA and treated with rituximab and prednisone and defervesced. Six months later, creatinine was stable at 1.5 mg/dL from a peak of 3.8 mg/dL

Discussion

Renal masses are a rare presentation of GPA. The diagnosis is often made incidentally after partial or radical nephrectomy. This case exemplifies the utility of percutaneous kidney biopsies as a nephron-sparing procedure, particularly useful in patients with reduced nephron mass, and the importance of histopathology in arriving at a diagnosis.