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Kidney Week

Abstract: SA-PO0375

Acute Renal Failure in Idiopathic Necrotizing Lymphadenitis

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Gao, Sheng, Virginia Mason Franciscan Health, Seattle, Washington, United States
  • Strobel, Brian K., Virginia Mason Franciscan Health, Seattle, Washington, United States
  • Haggerty, Lauren, Virginia Mason Franciscan Health, Seattle, Washington, United States
  • Walia, Ritika, Virginia Mason Franciscan Health, Seattle, Washington, United States
Introduction

Necrotizing lymphadenitis is a self-limiting, inflammatory condition which presents as fever, fatigue and cervical lymphadenopathy. We report an atypical case where acute renal failure (ARF) presents early in the disease course.

Case Description

A 33 year old woman with recent upper respiratory symptoms presents to the ED with cervical lymphadenopathy, diffuse maculopapular rash, and anuria. Vital signs are unremarkable. Laboratory workup reveals leukocytosis, serum creatinine 5.9, hyperphosphatemia, hypercalcemia, elevated uric acid and LDH. Proteinuria is present. Urine microscopy shows hematuria, pyuria and renal tubular epithelial cells. Broad viral and autoimmune testing is negative other than low C4 and EBV PCR of 207 IU/mL. Excisional lymph node biopsy shows necrotizing lymphadenitis with no evidence of malignancy. Kidney biopsy shows acute tubular injury with mild interstitial nephritis. The patient is started on steroids and hemodialysis. Dialysis is weaned 6 weeks later.

Discussion

Idiopathic necrotizing lymphadenitis is commonly diagnosed as Kikuchi-Fujumoto disease (KFD), but there are several features in our patient’s lymph node biopsy that were atypical: coagulative-type necrosis with presence of neutrophils, fewer histiocytes and CD4 T-cell predominance with numerous plasma cells in viable areas. However, the disease was steroid responsive and other diagnoses were ruled out, favoring a KFD-like process. Differentials considered include lupus, hemophagocytic lymphohistiocytosis, malignancy, and infection. Using KFD as the closest analogue, we searched the literature for associations of ARF and KFD. Prior reports are rare and all have been with severe multi-organ processes or explicit additional diagnoses such as lupus. In our case, the most likely explanation was high cell turnover (i.e. tumor-lysis physiology) leading to uric acid nephropathy. To our knowledge, this is the first report of an idiopathic necrotizing lymphadenitis with acute tubular injury at initial presentation.

a. Coagulative necrosis with neutrophils
b. CD138 IHC with numerous plasma cells
c. CD4 T cell predominance
d. Fewer CD8-T cells