A CASE OF ELDERLY-ONSET AHUS SUCCESSFULLY TREATED WITH COMPLEMENT INHIBITION, RESULTING IN RENAL RECOVERY

 

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A CASE OF ELDERLY-ONSET AHUS SUCCESSFULLY TREATED WITH COMPLEMENT INHIBITION, RESULTING IN RENAL RECOVERY

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Ryuichi
Yoshii
Ryuichi Yoshii yoshii.ryuichi@gmail.com Kumamoto Medical Center Nephrology Kumamoto Japan *
Kengo Kajiwara kengoffice@yahoo.co.jp Kumamoto Medical Center Nephrology Kumamoto Japan -
Toshiki Hatanaka taiki122001@yahoo.co.jp Kumamoto Medical Center Nephrology Kumamoto Japan -
Tomohumi Nakamura mimo_yusa@yahoo.co.jp Kumamoto Medical Center Nephrology Kumamoto Japan -
Masao Tomita mmuko@kumamoto-u.ac.jp Kumamoto Medical Center Nephrology Kumamoto Japan -
Hideki Yokoi hyokoi@kumamoto-u.ac.jp Kumamoto University Hospital Nephrology Kumamoto Japan -
 
 
 
 
 
 
 
 
 

Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated thrombotic microangiopathy (TMA) that can lead to acute kidney injury (AKI). Although often considered a disease of younger individuals, recent reports indicate that aHUS can also develop in the elderly. Early recognition and treatment are critical, particularly in cases of TMA with unexplained AKI.

An 85-year-old male presented with generalized edema and dyspnea. Initial evaluation revealed AKI, severe hypertension, and pleural effusion, prompting referral to our hospital. Laboratory tests showed schistocytes, elevated lactate dehydrogenase, hyperbilirubinemia, and thrombocytopenia, suggestive of TMA. Renal biopsy confirmed TMA. Immunofluorescence demonstrated strong C3 deposition along glomerular capillary walls and mesangium, while electron microscopy revealed no electron-dense deposits. Genetic testing for aHUS-related mutations was negative; however, anti-factor H antibody was positive. Secondary causes of TMA were excluded.

The patient was diagnosed with aHUS based on clinical, histopathological, and immunological findings. Hemodialysis was initiated on day 1 of admission. Ravulizumab therapy was started on day 10, resulting in marked improvement of renal function. By day 18, the patient was successfully weaned off dialysis.

This case highlights that aHUS can occur in elderly patients and should be considered in the differential diagnosis of TMA with unexplained AKI. Prompt diagnosis and early initiation of complement inhibition can lead to significant renal recovery, even in advanced age.

The content presented in this abstract was submitted for The 55th Western Regional Meeting of the Japanease Society of nephrology. I also declare that re-submitting the abstract is permitted by the organizers of the original meeting.

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