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: FR-PO977

Renal Necrotizing Histiocytic Lesions in a Patient with Systemic Lupus Erythematosus (SLE)

Session Information

Category: Pathology and Lab Medicine

  • 1800 Pathology and Lab Medicine

Authors

  • Raslan, Rasha, Duke University Health System, Durham, North Carolina, United States
  • Ortiz Kaemena, Maria Fernanda, Duke University Health System, Durham, North Carolina, United States
  • Lagoo, Anand S., Duke University Health System, Durham, North Carolina, United States
  • Howell, David Noble, Duke University Health System, Durham, North Carolina, United States
Introduction

Patients with SLE can develop lupus lymphadenitis (LL) with necrotizing lesions in lymph nodes and is difficult to distinguish from Kikuchi-Fujimoto Disease (KFD). There have been no cases depicting histological evidence of necrotizing lymphadenitis in the kidney

Case Description

A 35 year old female with SLE presented with fevers & cervical lymphadenopathy. Extensive workup for infections was negative. A lymph node and native kidney biopsy were performed (figures 1 & 2). These showed a necrotizing inflammatory process with CD8 + T cells, plasma cells and macrophages

Discussion

KFD & LL have both distinct and overlapping features. On histology, KFD is characterized by patchy areas of necrosis with abundant karyorrhectic nuclear debris. Neutrophils & eosinophils are typically absent. Immunohistochemical testing often shows CD3 & CD8 T cells. Presence of neutrophils, hematoxylin bodies, & abundant plasma cells is more consistent with LL. C4d staining (more common in LL) may be useful in distinguishing the two. There are reports of AKI in patients with KFD, however, direct involvement of renal parenchyma by necrotizing inflammation recapitulating lymphadenitis is not documented. One explanation is an over-active immune response that leads to localized inflammation in nearby tissues. KFD is typically limited to lymph nodes, so how it spreads to distant organs is less clear. Possible explanations include migration of inflammatory cells from elsewhere, or de novo inflammatory process that initiated in the kidney. It is unclear why this necrosis predominantly involves histiocytes and not neutrophils. Our case highlights another entity that can be encountered in a patient with SLE & the need for renal biopsies when a diagnosis is not clear. Obtaining an adequate sample is important to not miss other pathological processes