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Kidney Week

Abstract: TH-PO725

Gender Differences in Presentation and Outcomes Among Patients with Atypical Hemolytic Uremic Syndrome (aHUS)

Session Information

Category: Women’s Health and Kidney Diseases

  • 2000 Women’s Health and Kidney Diseases

Authors

  • Aigner, Christof, Medical University of Vienna, Vienna, Austria
  • Gaggl, Martina M., Medical University of Vienna, Vienna, Austria
  • Kain, Renate, Medical University of Vienna, Vienna, Austria
  • Prohaszka, Zoltan, Semmelweis University, Budapest, Hungary
  • Sunder-Plassmann, Raute, Medical University of Vienna, Vienna, Austria
  • Haninger-Vacariu, Natalja, Medical University of Vienna, Vienna, Austria
  • Schmidt, Alice, Medical University of Vienna, Vienna, Austria
  • Sunder-Plassmann, Gere, Medical University of Vienna, Vienna, Austria
Background

Gender differences among patients with aHUS are not well established.

Methods

We describe and compare demographic, clinical and genetic data from female and male patients with a history of aHUS enrolled in the Vienna thrombotic microangiopathy (TMA) cohort.

Results

In this single center study, we identified 51 patients with a first manifestation of aHUS between 1981 and 2019. The median age at diagnosis was 28 years and 63% were female. Kidney biopsies were available from 32 patients (63%) and all but 1 showed features of TMA. At time of presentation 31 patients were dialysis dependent (no data for 3 patients). 23 received plasma and 7 eculizumab therapy. 7 recovered kidney function with therapy (3 eculizumab, 3 plasma exchange, 1 supportive). At last follow-up, 9 were deceased, 3 on dialysis, and 17 had a renal graft. Among the remaining 22 patients, 13 had an eGFR ≥ 60 ml/min per 1.73m2. Gender specific results are indicated in Table 1.

Conclusion

The majority of aHUS patients enrolled in the Vienna TMA cohort were female. Women presented at younger age, more often harbored disease-causing genetic variants or a CFH- or CD46-risk haplotype, and had better kidney function at last follow-up as compared to males.

Table 1.
 FemaleMaleP
Patients, n (%)32 (63)19 (37)0.09
Age at diagnosis, years (median)27.529.00.96
Treatment at presentation  0.63
-Plasma-exchange, n (%)15 (46.9)8 (31.6) 
-Eculizumab, n (%)5 (15.6)2 (10.5) 
-Supportive, n (%)11 (34.4)6 (42.1) 
Age at last follow-up/death, years (median)35.534.00.87
Kidney function at last follow-up  0.05
-eGFR ≥ 60ml/min, n (%)10 (31)3 (16) 
-eGFR <60ml/min, n (%)6 (19)3 (16) 
-Dialysis, n (%)2 (6)1 (5) 
-Kidney transplant, n (%)10 (31)7 (37) 
-Deceased, n (%)4 (13)5 (26) 
Maintenance treatment at last follow-up, n (%)9 (28)3 (16)0.16
-Plasma, n (%)2 (6)2 (11) 
-Eculizumab, n (%)7 (22)1 (5) 
Disease-causing variants*, n (%)17 (53)7 (37)0.33
Variants of unknown significance, n (%)2 (6)1 (5) 
CFH-H3 / CD46ggaac, n (%)31 (97)13 (68)0.01

*no data available in 1, 5 showed no genetic variants