ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO2262

A Chimeric Case of AKI

Session Information

Category: Trainee Case Report

  • 1602 Pathology and Lab Medicine: Clinical

Authors

  • Kharadjian, Talar, University of California San Diego, La Jolla, California, United States
  • Miracle, Cynthia, University of California San Diego, La Jolla, California, United States
  • Zhang, Haiyan, University of California San Diego, La Jolla, California, United States
Introduction


New-onset nephrotic syndrome often requires kidney biopsy for diagnosis. Pathology is at times insufficient.

Case Description

An 81-year-old Hispanic female with HTN, CKD stage III presented with rash, fever, peripheral eosinophilia, AKI and diagnosed with DRESS syndrome due to allopurinol started 3 weeks ago. Urinalysis showed pyuria, hematuria. Spot urine Pr/Cr was 3.8 with serum albumin 2.5g/dL. Admission creatinine was 4.3mg/dL, increased to 6.5mg/dL prior to starting dialysis. Serologic findings shown below.

Steroids were started for DRESS and suspected AIN. Genetic testing for HLA-B*5801, seen in severe cutaneous adverse reactions to allopurinol, was positive. Skeletal survey demonstrated no lesions. Blood counts revealed anemia and thrombocytopenia.

Bone marrow biopsy did not show a plasma cell dyscrasia. Kidney biopsy noted amyloidosis (Congo red positive deposits in glomeruli, vessels, interstitium) with 56% global glomerulosclerosis and >60% severe IFTA. Mild interstitial inflammation with occasional eosinophils suggested resolving AIN. Severe findings of hypertensive sequelae noted. Immunofluorescence was 2+ for IgG and kappa. Electron microscopy showed 10nm fibrils and severe podocyte foot process effacement. Mass spectrometry diagnosed ALECT2 amyloidosis.

Discussion

Our case is unusual in having three simultaneous pathologies, namely AIN and ALECT2 amyloidosis superimposed on a background of hypertensive changes; the recognition of disproportionate proteinuria was essential to question the diagnosis and avoid early closure on AIN. Concurrent renal pathologies are a common finding in patients with ALECT2 amyloidosis, underscoring the utility of kidney biopsy in these patients who often have mixed clinical pictures.

Circumstances leading to this case raise suspicion for preceding immune dysregulation, in the form of DRESS/steroids, that may have triggered amyloidogenesis. A prior case report describes a patient who developed ALECT2 amyloid after steroids for allergic symptoms. This potential interplay between genetic and environmental factors requires further investigation.

UPEP, urine immunofixationNo monoclonal protein
SPEP, serum immunofixation Polyclonal
Kappa/lambda light chains8.42
Beta-2 microglobulin20.2 mg/dL
ANA, ANCA, anti-GBMnegative
Hepatitis B and C, cryoglobulinnegative
Complementsnormal