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Kidney Week

Abstract: TH-PO126

Kidneys, Windows to the Liver: A Case of Autoimmune Hepatitis With Lupus Nephritis

Session Information

  • AKI: Mechanisms - I
    November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Brown, Leanne, UConn Health, Farmington, Connecticut, United States
  • Singh, Aditi, UConn Health, Farmington, Connecticut, United States
Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease with a wide range of systemic manifestations. However, primary involvement of the liver is rare and is not part of the diagnostic criterion. We present a case of biopsy proven primary biliary cholangitis (PBC), later diagnosed as lupus nephritis with suspected autoimmune hepatitis (AIH) given remission with immunosuppressive therapy.

Case Description

An Asian-American 51 year-old male presented to the hospital with worsening lower extremity edema. Laboratory investigations revealed normal renal function, persistently elevated liver enzymes and equivocal antimitochondrial antibody (AMA) titers. PBC was diagnosed two years prior to presentation with liver biopsy demonstrating interface hepatitis with chronic inflammatory infiltrates and bile duct injury. He was started on ursodiol without significant improvement in his liver dysfunction. He subsequently developed an acute kidney injury (AKI) and was found to have both hematuria and proteinuria. Further work up demonstrated positive antinuclear antibodies in high titres and positive double stranded DNA antibodies with renal biopsy consistent with Class IV Lupus nephritis. He was induced with pulse dose steroids followed by maintenance with cyclophosphamide with later transitioned to mycophenolate mofetil. He achieved clinical and serological remission with improvement in his renal function and complete resolution of liver dysfunction on immunosuppressive regimen for SLE.

Discussion

Although the liver is not a major target for damage in SLE, clinical and biochemical evidence of liver dysfunction is common. This can pose great diagnostic challenges given a considerable degree of overlap in the presentation of lupus hepatitis, AIH and PBC. In our case a diagnosis of AIH was made after lupus nephritis was detected on renal biopsy and its response to immunosuppressive therapy. A high degree of clinical suspicion is required, especially in patients of Asian descent where AIH is more prevalent.