Atypical Hemolytic Uremic Syndrome and Chronic Ulcerative Colitis Treated with Eculizumab.

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

doi:10.9734/ijmpcr/2015/18771

Abstract

BackgroundHemolytic-uremic syndrome (HUS) presents with hemolytic anemia, thrombocytopenia, 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 –TreatmentWe 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.ConclusionThis 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.

Highlights

INTRODUCTIONA 16-year-old Caucasian male with a 4-year history of chronic active ulcerative colitis presented with severe symptomatic anemia, thrombocytopenia, intravascular hemolysis, acute kidney injury, nephritis, and decreased C3 levels
Case Diagnosis –Treatment—We present a patient with low circulating complement (C) 3 levels who developed aHUS in the course of chronic active ulcerative colitis (UC)
A 16-year-old Caucasian male with a 4-year history of chronic active ulcerative colitis presented with severe symptomatic anemia, thrombocytopenia, intravascular hemolysis, acute kidney injury, nephritis, and decreased C3 levels

Summary

INTRODUCTION

A 16-year-old Caucasian male with a 4-year history of chronic active ulcerative colitis presented with severe symptomatic anemia, thrombocytopenia, intravascular hemolysis, acute kidney injury, nephritis, and decreased C3 levels Three months earlier he was hospitalized for UC flare (Fig. 1a) and worsening microcytic anemia (Hb 8.2 mg/dL) and his symptoms responded to treatment with intravenous (IV) methyprednisolone and packed red blood cells (RBC) transfusion. Soluble MAC and anti-CFH antibodies were not detected before starting eculizumab, genetic studies on Complement Factor (CF)H, CFI, CFB, CFHR1-3, MCP, C3 and thrombomodulin did not reveal disease causing mutations He was discharged home on prednisone, metronidazole, balsalazide, penicillin, iron, and multivitamin. He was able to resume full activity including school attendance and participation in sports

Findings

DISCUSSION

CONCLUSION