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 X

Kidney Week

Abstract: TH-PO566

ANCA-Associated Pulmonary Renal Syndrome with Immune Complex Deposition

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Fleischhacker, Alexander Nathaniel, University of Miami Health System, Miami, Florida, United States
  • Quo, Shane W., University of Miami Health System, Miami, Florida, United States
  • Fernandez Bojanini, Carlos A., University of Miami Health System, Miami, Florida, United States
  • Padodara, Aakash, University of Miami Health System, Miami, Florida, United States
  • Ajuria, Jorge Luis, University of Miami Health System, Miami, Florida, United States
Introduction

Pulmonary renal syndrome (PRS) is a highly morbid condition in which rapid identification and empiric therapy is critical.

Case Description

A 55 year old female with a past medical history of hypertension, rheumatoid arthritis not on any active therapy, and CKD3 diagnosed 6 months ago, presented to the ED for 2 days of hemoptysis and hematuria. She denied any recent upper respiratory symptoms or fevers. She also developed constant, non radiating, epigastric pain with 2 episodes of blood streaked emesis. She denied any new medications. Because her symptoms were unremitting and she became dyspneic, she presented to the ED.
On arrival, she was tachypneic at 34 respirations per minute and hypoxic to 84%. She had a hemoglobin of 6.4. Her creatinine was 8.96 with a baseline of 1.5. On urinalysis, she had 3+ protein, 3+ blood, 6-9 WBCs, >100 RBCs, no casts or dysmorphic RBCs were noted. A chest x-ray showed severe, diffuse pulmonary infiltrates. Given concern for acute glomerulonephritis (GN) with associated PRS, she was started on empiric pulse dose steroids with 1 gram of methylprednisolone IV daily for 3 days as well as plasmapheresis with FFP and cyclophosphamide. Subsequent workup showed a positive MPO-ANCA serology. Complement c3 and c4 levels were low. Anti-GBM was negative. ANA was positive at 1:1280 with a speckled, nuclear pattern. Anti-DS DNA was not detected. Anti streptolysin O antibodies were in the normal range. Serologies for HIV and hepatitis were unremarkable.
Kidney biopsy revealed diffuse sclerosing and focal proliferative GN with 30 percent crescents and severe interstitial inflammation. There was also immune complex deposition.

Discussion

ANCA associated GN is most often pauci immune and concurrent immune complex GN is unusual but has been reported in 5-14 percent of patients with ANCA positive GN. When ANCA associated GN presents with immune complex deposition, vasculitis involvement is most often exclusively renal. In our case, the patient had both pulmonary and renal involvement. Recognition of MPO ANCA associated GN with concomitant immune complex deposition is important as it has been associated with worse renal outcomes. Moreover, while a link between ANCA positivity and rheumatoid arthritis has previously been established, RA related renal dysfunction is typically not rapidly progressive.