New Treatment Options for Atypical Hemolytic Uremic Syndrome with the Complement Inhibitor Eculizumab

Our case series showed that eculizumab is efficacious and safe in treating atypical hemolytic-uremic syndrome, as well as it has positive effects on quality of life. Further extensive studies are required to develop unified treatment guidelines.

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Kidney transplantation using complement inhibitor in a patient suffering from atypical hemolytic-uremic syndrome associated with factor H antibodies: successful prevention of recurrence of the underlying disease

Russian Journal of Transplantology and Artificial Organs ◽

10.15825/1995-1191-2020-2-132-138 ◽

2020 ◽

Vol 22 (2) ◽

pp. 132-138

Author(s):

E. I. Prokopenko ◽

S. A. Pasov ◽

A. V. Vatazin ◽

A. Ya. Tsalman ◽

T. E. Pankratenko ◽

...

Keyword(s):

Kidney Transplantation ◽

Hemolytic Uremic Syndrome ◽

Complement Activation ◽

Atypical Hemolytic Uremic Syndrome ◽

Underlying Disease ◽

Factor H ◽

Inhibitor Therapy ◽

Complement Inhibitor ◽

Post Transplant ◽

Uremic Syndrome

Atypical hemolytic-uremic syndrome (aHUS) is an extremely rare complement-mediated disease that belongs to the group of thrombotic microangiopathies (TMA). It often reoccurs after kidney transplantation (KT). Previously, KT was considered contraindicated in both children and adults with aHUS due to high (up to 50% and above) incidence of early graft loss associated with post-transplant recurrent TMA. Introduction of specific complement inhibitor therapy into clinical practice has improved outcomes in patients with aHUS and has significantly reduced the risk of post-transplant recurrence of underlying disease. We describe the clinical observation of a 20-year-old female patient with aHUS associated with antibodies to factor H, a major regulator of complement activation. The patient underwent KT and eculizumab was used for prophylactic purposes. In the postoperative period, the patient developed ureteral necrosis that required reconstructive surgery, followed by graft pyelonephritis. Despite postoperative complications, which were highly likely to trigger uncontrolled complement activation, TMA recurrence was avoided due to early treatment of the complications and prophylactic use of complement inhibitor therapy.

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ATYPICAL HEMOLYTIC UREMIC SYNDROME AND C3 GLOMERULOPATHY: CONCLUSIONS FROM A «KIDNEY DISEASE: IMPROVING GLOBAL OUTCOMES» (KDIGO) CONTROVERSIES CONFERENCE

Nephrology (Saint-Petersburg) ◽

10.24884/1561-6274-2018-22-4-18-39 ◽

2018 ◽

Vol 22 (4) ◽

pp. 18-39

Author(s):

Timothy H.J. Goodship ◽

H. Terence Cook ◽

Fadi Fakhouri ◽

Fernando C. Fervenza ◽

Veronique Fremeaux-Bacchi ◽

...

Keyword(s):

Kidney Disease ◽

Hemolytic Uremic Syndrome ◽

Treatment Options ◽

Atypical Hemolytic Uremic Syndrome ◽

Treatment Strategies ◽

Evidence Base ◽

Current Treatment ◽

Primary Role ◽

C3 Glomerulopathy ◽

Uremic Syndrome

In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.

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