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Abstract: PO1788

Lupus Nephritis Classification Should Consider Lupus Arteritis in the Activity Score

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Alzahrani, Nora Mohsin M, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States
  • Almokyad, Ismail K., The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States
  • Cohen, Scott D., The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States
  • Regunathan-Shenk, Renu, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States
  • Sethi, Sanjeev, Mayo Clinic Department of Laboratory Medicine and Pathology, Rochester, Minnesota, United States
Introduction

Lupus nephritis (LN) complicates 20-49% of systemic lupus erythematosus (SLE) patients. Vascular lesions are not considered in the activity index of LN pathology classification. We report a case of LN with severe necrotizing arteritis without proliferative glomerular lesions, prompting a more aggressive intervention.

Case Description

A 36-year-old woman with known SLE with Class II LN, as well as vasculitis, resulting in multiple digital amputations presented with eight days of abdominal pain, vomiting, fever, and tea-colored urine. Labs showed creatinine of 1.4 mg/dL, microscopic hematuria, urine protein/creatinine of 5 g/g, low complements, positive anti-double-stranded DNA of 14, an elevated antinuclear antibody of 1:320, elevated Myeloperoxidase (MPO)–Antineutrophil Cytoplasmic Antibody (ANCA) of 59. She received induction therapy with intravenous (IV) methylprednisolone 1g daily x3, followed by prednisone 60 mg daily and mycophenolate mofetil (MMF) 500 mg twice daily. A repeat kidney biopsy was performed, and 12 glomeruli showed only mesangial proliferation. She was classified as International Society of Nephrology and the Renal Pathology Society (ISN/RPS) class IIIA LN based on two arteries revealing severe arteritis with transmural necrosis causing occlusion, inflammation, and rupture of the vessel walls. Consequently, we switched MMF to IV cyclophosphamide 1g/m2 monthly. Creatinine improved to 1 mg/dl on discharge and 0.9 mg/dl two months later.

Discussion

There is limited attention to non-glomerular vascular lesions among patients with LN. Prior case reports show that LN patients with vascular involvement have worse outcomes and may require more aggressive treatment. The vasculitis in this case was attributed to the MPO Antibody. Given the potential prognostic and therapeutic implications of vascular involvement in LN patients, we suggest that lupus arteritis be considered in the LN pathology classification.