A 38-year-old Japanese man with past medical history of central retinal vein occlusion at the age of 36 years presented with one-week duration of dyspnea, precipitated by common cold-like symptoms. He denied having any episodes of diarrhea. On examination, vital signs were within normal limits except for blood pressure of 172/90 mmHg. He was alert and oriented. His conjunctiva was pale and icteric. There were no other significant findings. The laboratory data revealed microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. Since thrombotic microangiopathy was suspected, urgent plasma exchange (PE) and corticosteroid were initiated, which was assessed to be ineffective. Later, it turned out that ADAMTS13 activity was normal and its inhibitor was negative, which excluded the possibility of thrombotic thrombocytopenic purpura. The negative stool culture ruled out Shiga-toxin-producing Escherichia coli
(STEC) – hemolytic uremic syndrome (HUS). Based on these results, atypical HUS (aHUS) was the working diagnosis and eculizumab was considered as the treatment of choice. However, at that time, eculizumab was not approved in Japan for treatment of aHUS. Therefore, the validity of using eculizumab was discussed by the ethical committee. Through approval by the committee, we decided to start eculizumab 29 days after the onset. The initiation of eculizumab decreased LDH and elevated hemoglobin and platelet count. However, renal function continued to worsen and hemodialysis was started shortly after initiation of eculizumab. While eculizumab was given, his renal function then gradually improved and dialysis was successfully discontinued 18 months after onset of aHUS.
Discussion: Twenty-five percent of cases of aHUS have resulted in death from thrombosis or acute renal failure. Even if death can be avoided, around half of the other cases develop end-stage renal disease (ESRD). However, based on previous reports, eculizumab can improve chronic kidney disease by more than one stage on average. In our case, we initially treated with PE, and belatedly started eculizumab. It has been reported that delayed initiation of eculizumab can be associated with a worse outcome of renal function. This initially seemed to be the case in our patient who progressed to requiring long-term dialysis. However, continued treatment with eculizumab resulted in discontinuing dialysis 18 months after onset. It is worth considering that long-term eculizumab may improve renal function even for patients with ESRD associated with aHUS, requiring dialysis.
Conclusions: Our case suggests that long-term eculizumab contributes to gradual recovery from kidney damage and ultimately to discontinuation of dialysis.
To cite this abstract:
Yamada, Y; Miyakawa, Y . LONG-TERM ECULIZUMAB TREATMENT CONTRIBUTES TO RECOVERY FROM END-STAGE RENAL DISEASE ASSOCIATED WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME. Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev.
Journal of Hospital Medicine.
2017; 12 (suppl 2).
https://www.shmabstracts.com/abstract/long-term-eculizumab-treatment-contributes-to-recovery-from-end-stage-renal-disease-associated-with-atypical-hemolytic-uremic-syndrome/. Accessed July 15, 2019.
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