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: PO0186

Recurrent Stage 3 AKI Resolves with Establishing the Diagnosis of TAFRO Syndrome and Treatment with Anti-IL-6 Antibody

Session Information

  • AKI Mechanisms - 2
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

  • 103 AKI: Mechanisms

Authors

  • Tawhari, Ibrahim, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
  • Kanwar, Yashpal S., Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
  • Aggarwal, Vikram, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
Introduction

TAFRO syndrome, a unique variant of idiopathic multicentric Castleman’s disease (iMCD), is a syndrome with a constellation of Thrombocytopenia, Anasarca, Fever, Reticulin myelofibrosis/renal insufficiency, and Organomegaly. Its pathogenesis is driven by excessive cytokine storm, most notably from IL-6, causing multiorgan failure. 30% of patients with TAFRO-iMCD patients require dialysis. Glomerular thrombotic microangiopathy (TMA) and membranoproliferative glomerulonephritis are the most characteristic lesions. We present a challenging case of recurrent dialysis requiring AKI in whom recognition of TAFRO syndrome and glomerular TMA-like lesion led to successful treatment with anti-IL-6 therapy and subsequent definitive diagnosis of iMCD.

Case Description

A 35-year-old Hispanic female presented in May 2019 with fever, hypotension, anemia, thrombocytopenia, anasarca, hepatosplenomegaly, generalized lymphadenopathy, and anuric AKI. She was admitted to the intensive care unit, required vasopressors, mechanical ventilation, and initiation of continuous renal replacement therapy (CRRT). A comprehensive workup for infectious and autoimmune etiologies was unrevealing. This puzzling presentation was presumed to be driven by an unidentified viral illness. She received an empiric course of IVIG and steroids with mild improvement in anemia, thrombocytopenia, and complete renal recovery. A month later, she was admitted with a similar presentation and proteinuria. She once again required CRRT. Lab work revealed elevated IL-6. Lymph node and bone marrow biopsy were non-contributary. A renal biopsy revealed glomerular capillary endotheliosis. This time it became clear that she had a TAFRO phenotype and decided to treat her with tocilizumab (anti-IL-6 antibody) every two weeks. She had complete recovery of AKI, anemia, and thrombocytopenia within four weeks. She has been on anti-IL6 antibody for one year, and the disease is in remission. An excisional lymph node biopsy in May 2020 confirmed the diagnosis of Castleman’s disease.

Discussion

TAFRO syndrome-iMCD related renal involvement has been reported from Japan and France. For the first time, we describe these findings from a North American center. This case highlights that knowledge of TAFRO syndrome as a cause of AKI, its diagnostic approach, and renal histology is valuable for Nephrologists.