Abstract: SA-PO421

Results from the Cross-Sectional Evaluation of Clinical Symptoms and Epidemiologic Parameters in Patients with TMA, Differentiated by Laboratory Parameters Study (CESAR)

Session Information

Category: Chronic Kidney Disease (Non-Dialysis)

  • 302 CKD: Estimating Equations, Incidence, Prevalence, Special Populations

Authors

  • Schoenermarck, Ulf, University Hospital Munich-Grosshadern, D-81377 Muenchen, Germany
  • Ries, Wolfgang, Diako Flensburg, Flensburg, Germany
  • Schroppel, Bernd, University of Ulm, Ulm, Germany
  • Jeglitsch, Michael, Alexion, Munich, Germany
Background

Atypical hemolytic uremic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP) and HUS caused by Shiga toxin-producing Escherichia coli (STEC-HUS) are diseases with thrombotic microangiopathy (TMA) and similar clinical presentations. Epidemiological data on TTP and aHUS in Germany are lacking.

Methods

CESAR is a German prospective, multicenter, cross-sectional, epidemiological study of the relative incidences (RI) of aHUS, TTP and STEC-HUS in patients (pts) presenting with TMA. ADAMTS13 activity and presence of STEC were tested. Treating clinicians diagnosed pts as either aHUS, TTP, STEC-HUS or “other”.

Results

From 04/2014 to 03/2017, 232 pts were enrolled in 22 German centers. RIs and clinical data are shown in the table. 104 (74%) pts with aHUS had at least one complement-amplifying condition.

Conclusion

aHUS was the most common diagnosis in pts with clinical TMA. Less frequent differential diagnoses including TTP should be rapidly ruled out through specific diagnostic tests. Pts with aHUS or STEC-HUS were less thrombocytopenic and experienced more severe acute kidney injury than pts with TTP.

Funding

  • Commercial Support