Relapse of atypical haemolytic uraemic syndrome after kidney transplantation: role of ATG and failure of mycophenolate mofetil as rescue therapy

Atypical haemolytic uraemic syndrome (aHUS) is a form of thrombotic microangiopathy precipitated by unopposed complement activation, the treatment of which has been revolutionised by the availability of the monoclonal anti-complement (C5) antibody, eculizumab. Historically, women with aHUS would be unable to achieve a successful pregnancy due to the severity of their renal disease and for the few who could conceive, recurrence of aHUS was a significant risk. In spite of this, parenthood remains a priority for many. Experience with eculizumab use in the management of aHUS during pregnancy is growing and with it comes a significant change in the course of the disease. We present the case of a 28-year-old woman diagnosed with severe aHUS in the first trimester of her first pregnancy. She received rescue therapy with eculizumab and had a return to normal renal function. While this pregnancy was lost, she strongly desired a family. We managed her through a subsequent pregnancy while receiving eculizumab. This pregnancy was uncomplicated and carried to term and she birthed a healthy 2500 g baby girl. The complexities of managing a pregnancy in a woman with a history of aHUS are vast but not insurmountable, as demonstrated by this case.

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Absence of thrombocytopaenia and/or microangiopathic haemolytic anaemia does not reliably exclude recurrence of atypical haemolytic uraemic syndrome after kidney transplantation

Nephrology ◽

10.1111/nep.12937 ◽

2017 ◽

Vol 22 ◽

pp. 28-31 ◽

Cited By ~ 2

Author(s):

Anoushka R Krishnan ◽

Brian Siva ◽

Aron Chakera ◽

Germaine Wong ◽

Daniel Wong ◽

...

Keyword(s):

Kidney Transplantation ◽

Haemolytic Anaemia ◽

Haemolytic Uraemic Syndrome ◽

Microangiopathic Haemolytic Anaemia ◽

Atypical Haemolytic Uraemic Syndrome

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Functional analyses indicate a pathogenic role of factor H autoantibodies in atypical haemolytic uraemic syndrome

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfp388 ◽

2009 ◽

Vol 25 (1) ◽

pp. 136-144 ◽

Cited By ~ 53

Author(s):

S. Strobel ◽

P. F. Hoyer ◽

C. J. Mache ◽

E. Sulyok ◽

W.-s. Liu ◽

...

Keyword(s):

Haemolytic Uraemic Syndrome ◽

Factor H ◽

Pathogenic Role ◽

Atypical Haemolytic Uraemic Syndrome ◽

Functional Analyses ◽

Factor H Autoantibodies

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Cost-effectiveness of eculizumab treatment after kidney transplantation in patients with atypical haemolytic uraemic syndrome

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfw353 ◽

2017 ◽

Vol 32 (suppl_1) ◽

pp. i115-i122 ◽

Cited By ~ 13

Author(s):

Jan AJG van den Brand ◽

Jacobien C Verhave ◽

Eddy M Adang ◽

Jack FM Wetzels

Keyword(s):

Kidney Transplantation ◽

Cost Effectiveness ◽

Haemolytic Uraemic Syndrome ◽

Atypical Haemolytic Uraemic Syndrome

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Functional studies indicate a direct pathogenic role of autoantibodies in atypical haemolytic uraemic syndrome by inhibiting factor H cell protective activity

Molecular Immunology ◽

10.1016/j.molimm.2008.08.016 ◽

2008 ◽

Vol 45 (16) ◽

pp. 4100

Author(s):

Stefanie Strobel ◽

Peter F. Hoyer ◽

Christoph J. Mache ◽

Endre Sulyok ◽

Peter F. Zipfel ◽

...

Keyword(s):

Haemolytic Uraemic Syndrome ◽

Factor H ◽

Protective Activity ◽

Pathogenic Role ◽

Functional Studies ◽

Inhibiting Factor ◽

Atypical Haemolytic Uraemic Syndrome

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Successful rescue therapy with mycophenolate mofetil in kidney transplantation improves the long-term graft survival

Medicina ◽

10.3390/medicina43120124 ◽

2007 ◽

Vol 43 (12) ◽

pp. 953 ◽

Cited By ~ 2

Author(s):

Jaanus Kahu ◽

Aleksander Lõhmus ◽

Madis Ilmoja ◽

Ülle Kirsimägi ◽

Gennadi Timberg ◽

...

Keyword(s):

Mycophenolate Mofetil ◽

Kidney Transplantation ◽

Graft Survival ◽

Cyclosporine A ◽

Negative Impact ◽

Rescue Therapy ◽

Graft Loss ◽

Graft Function ◽

Significant Difference

Objective. The aim of this study was to compare the graft survival after kidney transplantation in patients treated with azathioprine (AZA) or mycophenolate mofetil (MMF) and analyze the significance of different risk factors for graft survival. Material and methods. A total of 137 patients, transplanted between January 1996 and June 2001, were retrospectively divided into two groups: patients who received AZA together with cyclosporine A and methylprednisolone (AZA group, n=72) and patients who received MMF either immediately or were switched from AZA to MMF during 3 months (MMF group, n=65). Results. According to Kaplan-Meier analysis, a 1-year graft survival was 79% in the AZA group and 85% in the MMF group; a 6-year graft survival was 51% and 67%, respectively (P=0.046). Multivariate Cox survival model demonstrated that MMF therapy reduced the risk of graft loss by 34% (P=0.028), while delayed graft function increased the risk of graft loss (risk ratio 2.26, P=0.009). A statistically significant difference in total cholesterol level (6.7 vs. 5.7 mmol/L, respectively; P=0.002), mean systolic blood pressure (145 vs. 134 mmHg, P=0.009), and cyclosporine A daily dose (238 vs. 203 mg, P=0.015) between the AZA and MMF groups at 1 year was revealed. Conclusion. MMF rescue therapy improves the long-term graft survival compared to AZA despite high early rejection rate and avoids the negative impact of acute rejections on graft survival.

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