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Research Article Open access CC BY 4.0

Atypical Hemolytic Uremic Syndrome and Chronic Ulcerative Colitis Treated with Eculizumab

Tennille N. Webb, Heidi Griffiths, Yosuke Miyashita, Riha Bhatt, Ronald Jaffe, Michael Moritz, Johannes Hofer, Agnieszka Swiatecka-Urban

International Journal of Medical and Pharmaceutical Case Reports · pp. 105–112 · Published 19 Jun 2015

10.9734/IJMPCR/2015/18771

Abstract

Background: Hemolytic-uremic syndrome (HUS) presents with hemolytic anemia, thrombo-cytopenia, and thrombotic microangiopathy of the kidney and usually results from Shiga-toxin induced activation of the alternative complement pathway. Gastroenteritis is a common feature of the Shiga-toxin producing Escherichia coli HUS, referred to as STEC-HUS. An inherited or acquired complement dysregulation may lead to HUS referred to as non-STEC or atypical (a)HUS. Although gastroenteritis is not a common presentation of aHUS, some patients develop ischemic colitis and may be misdiagnosed as acute appendicitis or acute ulcerative colitis (UC). Case Diagnosis –Treatment: We present a patient with low circulating complement (C) 3 levels who developed aHUS in the course of chronic active UC. Resolution of renal and gastrointestinal manifestations in response to treatment with eculizumab, a humanized monoclonal antibody against terminal C5 protein suggests the role of alternative complement in the pathogenesis of both, aHUS and UC. Conclusion: This case illustrates that dysregulation of the alternative complement pathway may manifest in other organs besides the kidney and that the circulating C3 levels do not correlate with the disease activity or the clinical response to eculizumab.

Hemolytic–uremic syndrome (HUS) thrombotic microangiopathy (TMA) ulcerative colitis (UC) inflammatory bowel disease (IBD) acute kidney injury (AKI) membrane attack complex (MAC).

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Cited by 28

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Ozer H, Baloglu İ, Turkmen K · Gastroenterol Hepatol Bed Bench · 2023

Pediatric Atypical Hemolytic Uremic Syndrome Advances.

Raina R, Vijayvargiya N, Khooblall A · Cells · 2021

Deposition of the Membrane Attack Complex in Healthy and Diseased Human Kidneys.

Koopman JJE, van Essen MF, Rennke HG · Front Immunol · 2020

Ulcerative Colitis and Atypical Hemolytic-Uremic Syndrome: An Unusual But Potentially Life-threatening Complication.

Viada Bris JF, Velasco Rodríguez-Belvís M, de Lucas Collantes C · Inflamm Bowel Dis · 2019

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