Kidney Week

Abstract: SA-PO1155

Leukocyte Chemotactic Factor 2-Related Amyloidosis Presenting with a Rash and Unexplained CKD

Session Information

• CKD: Patient-Oriented Care and Case Reports
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: CKD (Non-Dialysis)

• 2302 CKD (Non-Dialysis): Clinical, Outcomes, and Trials

Authors

• Gholizadeh Ghozloujeh, Zohreh, Loma Linda University, Loma Linda, California, United States

Zohreh Gholizadeh Ghozloujeh, MD

• Chen Wongworawat, Yan, Loma Linda University, Loma Linda, California, United States

Yan Chen Wongworawat

• Abdi Pour, Amir, Loma Linda University, Loma Linda, California, United States

Amir Abdi Pour, MD, FASN

• Ebrahimi, Niloufar, Loma Linda University, Loma Linda, California, United States

Niloufar Ebrahimi, MD

• Norouzi, Sayna, Loma Linda University, Loma Linda, California, United States

Sayna Norouzi, MD, FASN

Introduction

Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a recently described type of amyloidosis predominantly affecting the kidneys. ALECT2 has a relatively benign course, rare cardiac involvement, and a specific ethnic distribution. Recognizing ALECT2 is crucial to avoid unnecessary and potentially harmful treatments intended for more common subtypes like AL or AA amyloidosis. We present a case of ALECT2 with unusual clinical presentation and renal histology.

Case Description

A 60-year-old Hispanic female with a history of prediabetes (HbA1c = 5.7%) and hypertension presented with a pruritic skin rash on her hands. Upon reviewing historical laboratory findings, the patient was found to have elevated serum creatinine (Cr = 1.20 mg/dL) with an estimated glomerular filtration rate of 48 mL/min/1.73m² (CKD stage 3) without proteinuria for the past 20 months. Renal ultrasound showed increased echogenicity consistent with CKD. Despite controlling her hypertension, her serum creatinine continued to rise (1.39 mg/dL) over the next 2 months, necessitating a biopsy to investigate the underlying etiology of her CKD.
The kidney biopsy revealed LECT2 amyloidosis, predominantly glomerular and less dominant interstitial amyloid deposition, which was Congo red-positive with light green birefringence under polarized light (Image1). No diagnostic features of paraprotein-associated renal deposition disease, immune complex-mediated glomerulonephritis, or interstitial nephritis were observed, and urine protein electrophoresis was negative. The echocardiography did not suggest cardiac involvement with an ejection fraction of 60-65%.

Discussion

We report an unusual case of biopsy proven ALECT2 presenting with skin rashes resembling other autoimmune diseases.