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Kidney Week

Abstract: SA-PO055

Pancreatitis-Associated Atypical Hemolytic Uremic Syndrome

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical‚ Outcomes‚ and Trials

Authors

  • Sinha, Avilasha, Baylor College of Medicine Margaret M and Albert B Alkek Department of Medicine, Houston, Texas, United States
  • Yan, Jingyin, Baylor College of Medicine Margaret M and Albert B Alkek Department of Medicine, Houston, Texas, United States
Introduction

Atypical hemolytic uremic syndrome (aHUS) is a rare form of TMA that can present as AKI and can progress to ESRD and even be fatal making prompt diagnosis and treatment very important.

Case Description

A 41-year-old male with a history of recurrent, non-alcoholic pancreatitis presented with epigastric pain. His lipase level was elevated and CT showed acute pancreatitis. Exam was notable only for epigastric tenderness. Labs (Table 1) included an elevated creatinine (Crt) of 14.7 (baseline was 1.0) and were suggestive of hemolysis although ADAMTS13 activity level and platelet count were normal. As the cause of his AKI could not be elucidated and his Crt remained elevated, renal biopsy was performed and showed fibrin thrombi and fragmented red blood cells consistent with TMA. A genetic panel found several polymorphisms strongly associated with aHUS. He was started on eculizumab and is continuing treatment with hopes of renal recovery although he is currently dialysis-dependent.

Discussion

The differential for this patient’s AKI included ATN, AIN, and IgG4-related membranous nephropathy. As there were many potential etiologies, biopsy was helpful to definitively determine the cause of his AKI especially because the lack of thrombocytopenia made TMA seem unlikely. The trigger for aHUS in this case was most likely pancreatitis as the inflammation activates complement which compounds the complement overactivation in patients genetically predisposed to produce factor H autoantibodies. Eculizumab, which prevents formation of the membrane attack complex, has shown great efficacy in aHUS and early initation is associated with better outcomes underscoring the importance of early diagnosis. This case highlights the association of aHUS with pancreatitis and demonstrates how lack of thrombocytopenia does not rule out HUS.

 Measured ValueNormal Range
Lipase304 U/L8 – 78 U/L
Creatinine14.7 mg/dL0.57 – 1.25 mg/dL
Blood Urea Nitrogen107 mg/dL7 – 21 mg/dL
Hemoglobin5.7 g/dL13.7 – 17.5 g/dL
Haptoglobin< 8 mg/dL14 – 258 mg/dL
Lactate Dehydrogenase1,008 U/L125 – 220 U/L
IgG4125.2 mg/dL4 – 86 mg/dL
Reticulocyte Count7.3%0.5 – 1.8%
Total Bilirubin0.6 mg/dL0.2 – 1.2 mg/dL
Platelet Count200,000/μL150,000 – 400,000/μL

Table 1: Notable lab values on admission