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Kidney Week

Abstract: TH-PO0067

A Case of Severe AKI and Skin Involvement Without Crescents

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Starr, Tyler Chazan, UMass Memorial Health, Worcester, Massachusetts, United States
  • Le, Cindy, UMass Memorial Health, Worcester, Massachusetts, United States
  • Weatherly, Henry, UMass Memorial Health, Worcester, Massachusetts, United States
  • McClennen, Taylor, University of Massachusetts Chan Medical School, Worcester, Massachusetts, United States
  • Paulson, Jacob, UMass Memorial Health, Worcester, Massachusetts, United States
  • Yuan, Robert, UMass Memorial Health, Worcester, Massachusetts, United States
  • Vanguri, Vijay K., UMass Memorial Health, Worcester, Massachusetts, United States
  • Parada, Xavier F., UMass Memorial Health, Worcester, Massachusetts, United States
Introduction

Crescent involvement is highly predictive of the rare rapidly progressive form of IgA Nephropathy. Crescents are also associated with vasculitides, which share many dermatologic similarities with IgA Vasculitis. IgAN can occur concurrently with other glomerular diseases such as Anti-GBM and ANCA associated GN. This case presents a patient with rapidly progressive renal failure without crescent involvement and profound dermatologic symptoms.

Case Description

A 71 year old woman with HTN, HLD, and baseline Cr 0.9 presented with weakness, diarrhea, new palpable purpuric rash involving face and extremities and an AKI (Cr 1.85) with elevated CK (2773). Urine studies showed mild proteinuria, RBCs, WBCs, and rare granular casts. Normal complement levels, MPO & PR3 negative, and positive atypical P-ANCA. The patient initially elected to defer a skin biopsy. Her renal function continued to decline, and a renal biopsy showed interstitial nephritis with eosinophils, IgA nephropathy without glomerular involvement, and active arteritis. Pulse dose steroids improved her renal function rapidly; however, her skin lesions drastically evolved into bullae throughout her extremities and face. While the initial skin biopsy was more suggestive of medium vessel vasculitis, her subsequent skin biopsy revealed IgA Vasculitis. She was then started on Rituximab and IVIG.

Discussion

The lack of crescentic involvement suggests that this patient had a concurrent AIN and IgA Vasculitis. Both diseases are very sensitive to steroids, and with the ultimate skin biopsy showing IgA Vasculitis, it is possible the renal biopsy was performed early in the IgAN course. Even with the profound skin findings, the absence of crescents argued against ANCA induced renal failure. The case also emphasizes the importance of multiple skin biopsies when IgAN is on the differential. Furthermore, the treatment of rapidly progressing IgAN without crescents should be addressed in future KDIGO guidelines.

Digital Object Identifier (DOI)