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Patients with atypical hemolytic uremic syndromes (HUS) have genetic mutations in the alternate complement pathway and a high rate of recurrence post renal transplantation. Renal transplantation in this group of patients carries a high risk of graft failure and mortality. Eculizumab has been used as a prophylactic measure, but is expensive, frequently inaccessible and has no definite recommendations for cessation of use.
We describe a set of patients with atypical HUS who underwent live donor renal transplantation at our centre with eculizumab sparing protocols. The management strategies incorporated have also been analysed. We followed up this group of patients for a year. This is the first study of its kind in South Asia to our knowledge. It highlights the possibility of avoiding or reducing the use of eculizumab in patients with atypical HUS, thus providing hope to many with this difficult disease.
Patients with atypical HUS/ microangiopathy who underwent live donor renal transplantation at our centre were included in the study. Immunosuppressive regimens, post transplant complications, 1 year graft and patient survival were analysed.
Eculizumab sparing protocol used:
Out of 450 renal transplants done from January 2021 to August 2023, 6(1.33%) patients had atypical HUS. 4 were males and 2 were females. Immunological profile: All patients were CDC cross match negative, had permissible DSA titres and negative flow cross match. 2 patients needed pre-transplant nephrectomy. Mean age was 30 years. 4 (66%)patients had biopsy proven thrombotic microangiopathy; the other two had genetic mutations. Genetic analysis: 3 patients had CFHR1 and CFHR3 mutation, 1(16%) had CFH exon 17 mutation and 1(16% ) had DGKE mutation. Anti factor H antibody was positive in 1(16%) patient. 1/6 patient was given eculizumab prohylaxis. Incidence of delayed graft function and vascular thrombosis was nil. 2 (33%)patients had acute rejection (T- Cell mediated) which was managed with steroids. 1 patient had biopsy proven acute tubular injury. 1 patient developed cytomegalovirus hepatitis. None had BK virus infection. Average number of pre transplant antihtpertensives was 4, which reduced to 1 post transplant. At 1 year follow up, rate of recurrence was 0. Graft and patient survival at 1 year are 100%.
Renal transplantation is possible with eculizumab sparing protocols in cases of atypical hemolytic uremic syndrome. 1 year graft and patient survival outcomes are encouraging. Eculizumab as salvage therapy may be needed at any time. Long term follow up is essential. Methods to reduce activation of alternate pathway include strict blood pressure control, low dose calcineurin inhibitors, angiotensin receptor blockers and statins.