Successful use of eculizumab to treat atypical hemolytic uremic syndrome in patients with inflammatory bowel disease

Ramy M. Hanna, Noah Merin, Richard Burwick, Lama Abdelnour, Umut Selamet, Beshoy Yanny, Patrick Bui, Mary Fouad, Ira Kurtz

Research output: Contribution to journalArticle

Abstract

Background: Atypical hemolytic uremic syndrome is a rare group of disorders that have in common underlying complement amplifying conditions. These conditions can accelerate complement activation that results in a positive feedback cycle. The known triggers for complement activation can be diverse and include, infection, autoimmune disease, and malignancy. Recent reports suggest that certain autoimmune and rheumatological triggers of complement activation may result in atypical hemolytic uremic syndrome that does not resolve despite treating the underlying disorder. Specifically, patients with systemic lupus erythematosus and microangiopathic hemolysis may not respond to treatment of their underlying rheumatological trigger but responded to complement blockade. Case presentations: We report two patients with inflammatory bowel disease complicated by development of atypical hemolytic uremic syndrome. In both cases, patients were on treatment for inflammatory bowel disease, that was not well controlled/flaring at the time. The first patient is a male who developed Crohn's disease and microangiopathic hemolysis at age 5 and was treated with eculizumab successfully. Discontinuation of the medication led to multiple relapses, and the patient currently is being treated with eculizumab and has normal hematological and stable renal parameters. The second patient is a 49-year-old female with Ulcerative Colitis treated with 6-Mercaptopurine. She developed acute kidney injury and microangiopathic hemolysis. Prompt diagnosis and treatment with eculizumab resulted in the recovery of kidney injury along with a complete hematological response. Conclusions: These two cases are the fifth and sixth patients to be published in the literature with atypical hemolytic uremic syndrome and inflammatory bowel disease treated with complement blockade. This confirms that C5 complement blockade is effective in treating complement mediated thrombotic microangiopathy/atypical hemolytic uremic syndrome when it is triggered in patients with inflammatory bowel disease.

Original languageEnglish (US)
Article number18
JournalThrombosis Journal
Volume17
Issue number1
DOIs
StatePublished - Sep 9 2019

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Inflammatory Bowel Diseases
Complement Activation
Hemolysis
Complement C5
Thrombotic Microangiopathies
Kidney
6-Mercaptopurine
eculizumab
Atypical Hemolytic Uremic Syndrome
Ulcerative Colitis
Acute Kidney Injury
Crohn Disease
Systemic Lupus Erythematosus
Autoimmune Diseases
Therapeutics
Recurrence
Wounds and Injuries
Infection
Neoplasms

Keywords

  • Atypical hemolytic uremic syndrome
  • Complement
  • Complement blockade
  • Crohn's colitis/Crohn's disease
  • Inflammatory bowel disease
  • Thrombotic Microangiopathy
  • Ulcerative colitis

ASJC Scopus subject areas

  • Hematology

Cite this

Successful use of eculizumab to treat atypical hemolytic uremic syndrome in patients with inflammatory bowel disease. / Hanna, Ramy M.; Merin, Noah; Burwick, Richard; Abdelnour, Lama; Selamet, Umut; Yanny, Beshoy; Bui, Patrick; Fouad, Mary; Kurtz, Ira.

In: Thrombosis Journal, Vol. 17, No. 1, 18, 09.09.2019.

Research output: Contribution to journalArticle

Hanna, RM, Merin, N, Burwick, R, Abdelnour, L, Selamet, U, Yanny, B, Bui, P, Fouad, M & Kurtz, I 2019, 'Successful use of eculizumab to treat atypical hemolytic uremic syndrome in patients with inflammatory bowel disease', Thrombosis Journal, vol. 17, no. 1, 18. https://doi.org/10.1186/s12959-019-0207-7
Hanna, Ramy M. ; Merin, Noah ; Burwick, Richard ; Abdelnour, Lama ; Selamet, Umut ; Yanny, Beshoy ; Bui, Patrick ; Fouad, Mary ; Kurtz, Ira. / Successful use of eculizumab to treat atypical hemolytic uremic syndrome in patients with inflammatory bowel disease. In: Thrombosis Journal. 2019 ; Vol. 17, No. 1.
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