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Abstract: FR-PO198

Plasma Cell-Rich Acute Interstitial Nephritis in VEXAS: An Under-Recognized Disease Feature

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Kalantari, Kambiz, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Herrera Hernandez, Loren Paola, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Bu, Lihong, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Nasr, Samih H., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Cornell, Lynn D., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Warrington, Kenneth, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Mangaonkar, Abhishek, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Patnaik, Mrinal, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Koster, Matthew J., Mayo Clinic Minnesota, Rochester, Minnesota, United States
Introduction

VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently identified autoinflammatory disease with a large variety of disease manifestations. While recurrent fever, auricular chondritis, recurrent venous thromboembolism/thrombophlebitis, inflammatory skin lesions, ocular inflammation and cytopenias have been well-described, understanding of kidney manifestations is limited.

Case Description

Methods: Medical records of all patients with genetically confirmed VEXAS syndrome were reviewed for evidence of AKI or abnormal urinalysis. Patients who met the KDIGO criteria for AKI for at least two consecutive measurements of serum creatinine or cystatin C were considered as having AKI. Biopsy specimens (n = 4) were reviewed by four experienced nephropathologists. Clinical and laboratory features at disease onset and at time of AKI diagnosis were abstracted from direct chart review.
Results: Among a cohort of 69 patients (all men, mean age 71 ± 9) with VEXAS syndrome, 16 (23%) developed AKI (mean age 75 ± 9) at some point during their follow up. A review of urinary findings revealed microscopic hematuria, mild proteinuria, and pyuria in 100%, 100% and in 82% of cases, respectively. Four patients had undergone renal biopsy for AKI. One patient had features of peri-tubular capillaritis and has been previously described. Three patients were found to have biopsy confirmation of interstitial nephritis (IN). All three patients had IN (acute in 2 and active chronic in 1) with plasma cell-rich infiltrate and tubulitis. Immunofluorescence was negative and electron microscopy did not show glomerular or extraglomerular immune deposits. Prednisone was initiated in all patients with biochemical improvement in renal function and resolution of urinary findings, however, AKI recurred following reduction in prednisone dose below 10-15 mg/day.

Discussion

AKI from Plasma cell-rich IN is an under-recognized feature in patients with VEXAS. AKI in VEXAS responds well to treatment with corticosteroids. Recurrence is common with lowering corticosteroids dose. Further investigations to Identify targeted, effective therapies is necessary.