Early relapse rate determines further relapse risk: results of a 5-year follow-up study on pediatric CFH-Ab HUS
- PDF / 597,215 Bytes
- 9 Pages / 595.276 x 790.866 pts Page_size
- 15 Downloads / 207 Views
ORIGINAL ARTICLE
Early relapse rate determines further relapse risk: results of a 5-year follow-up study on pediatric CFH-Ab HUS Johannes Hofer 1,2,3 & Magdalena Riedl Khursigara 3,4 & Markus Perl 5 & Thomas Giner 3 & Alejandra Rosales 3 & Gerard Cortina 3 & Siegfied Waldegger 3 & Therese Jungraithmayr 3,6 & Reinhard Würzner 7 Received: 20 February 2020 / Revised: 19 July 2020 / Accepted: 31 August 2020 # The Author(s) 2020
Abstract Background The complement factor H antibody (CFH-Ab)–associated hemolytic uremic syndrome (HUS) forms a distinct subgroup within the complement-mediated HUS disease spectrum. The autoimmune nature of this HUS subgroup implies the potential benefit of a targeted immunosuppressive therapy. Data on long-term outcome are scarce. Methods This observational study evaluates the clinical outcome of 19 pediatric CFH-Ab HUS patients from disease onset until their 5-year follow-up. Results All but one relapse occurred during the first 2 years, and patients who had no relapse within the first 6 months were relapse-free until the end of the observation period. Kidney function at disease onset determines long-term kidney function: all individuals with normal kidney function at disease onset had normal kidney function after 5 years, and all patients with reduced kidney function at onset had impaired kidney function at the last follow-up. Level of CFH-Ab titer at disease onset was not correlated with a higher risk of recurrences or worse long-term outcome after 5 years. Resolution of CFH-Ab titers after 5 years was common. Conclusions CFH-Ab HUS patients have a varied overall long-term course. Early relapses are common, making close surveillance during the first years essential, regardless of the initial CFH-Ab titer. Keywords CFH-Ab . Hemolytic uremic syndrome . Immunosuppressive therapy
Introduction * Johannes Hofer [email protected] * Reinhard Würzner [email protected] 1
Institute of Neurology of Senses and Language, Hospital of St John of God, Linz, Austria
2
Research Institute of Developmental Medicine, Johannes Kepler University Linz, Linz, Austria
3
Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria
4
Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
5
Department of Pediatrics III, Medical University of Innsbruck, Innsbruck, Austria
6
Department of Pediatric Nephrology, Hospital Memmingen, Memmingen, Germany
7
Institute of Hygiene & Medical Microbiology, Medical University of Innsbruck, Innsbruck, Austria
Complement factor H antibody (CFH-Ab)–associated hemolytic uremic syndrome (HUS) forms a distinct subgroup of primarily complement-mediated thrombotic microangiopathies (TMA). CFH-Ab-associated HUS is reported in 6–25% of atypical HUS (aHUS) patients with pediatric onset of the disease [1–4]. Colleagues in India reported a much higher incidence (56%), which is so far not understood [5]. TMA is characterized by endothelial cell activation and secondary thrombus formation in th
Data Loading...
