Original Article

Pediatric Nephrology

, Volume 26, Issue 3, pp 401-410

First online:

Markers of childhood lupus nephritis indicating disease activity

  • Monika EdelbauerAffiliated withDepartment of Pediatrics I, Innsbruck Medical University Email author 
  • , Sudhir KshirsagarAffiliated withDepartment of Pediatrics I, Innsbruck Medical University
  • , Magdalena RiedlAffiliated withDepartment of Pediatrics I, Innsbruck Medical University
  • , Dieter HaffnerAffiliated withUniversitäts-Kinder- und Jugendklinik, Universität Rostock
  • , Heiko BillingAffiliated withZentrum für Kinder- und Jugendmedizin, Universitätsklinikum Heidelberg
  • , Burkhard TönshoffAffiliated withZentrum für Kinder- und Jugendmedizin, Universitätsklinikum Heidelberg
  • , Sophia RossAffiliated withKinder- und Jugendklinik der Friedrich-Alexander-Universität Erlangen-Nürnberg
  • , Jörg DötschAffiliated withKinder- und Jugendklinik, Universität Köln
  • , Oliver AmonAffiliated withKlinik für Kinder- und Jugendmedizin, Universitätsklinikum Tübingen
    • , Henry FehrenbachAffiliated withKlinik für Kinderheilkunde und Jugendmedizin, Klinikum Memmingen
    • , Christian SteuberAffiliated withKlinikum Links der Weser
    • , Antje BeissertAffiliated withUniversitäts-Kinderklinik Würzburg
    • , Josef HagerAffiliated withDepartment of Pediatric Surgery of the Department of Visceral, Transplant and Thoracic Surgery, Innsbruck Medical University
    • , Gottfried WechselbergerAffiliated withDepartment of Plastic and Reconstructive Surgery, Innsbruck Medical University
    • , Lutz T. WeberAffiliated withDr. von Haunersches Kinderspital, Klinikum der Universität München
    • , Lothar Bernd ZimmerhacklAffiliated withDepartment of Pediatrics I, Innsbruck Medical University


Current treatment regimens for childhood lupus nephritis (LN) are associated with significant side-effects and toxicity in vulnerable phases of growth and development. The paucity of biomarkers particularly in childhood impedes the appropriate clinical management and the development of new therapeutics. We analyzed markers of immune system (BAFF, RANTES), complement (Bb, C1q, C3d-CIC, C5a) and endothelial cell activation (sVCAM-1) in children with LN (n = 22, mean age 14.8 ± 4.7 years), nephrotic syndrome (n = 13) and age-matched healthy controls (n = 20) to define parameters that correlate with LN activity. Complement fragments of the alternative (Bb, p = 0.0004) classical (C3d-CIC, p < 0.0001) and common pathway (C5a, p < 0.0001) and the levels of BAFF (p < 0.0001), RANTES (p = 0.0002) and sVCAM-1 (p = 0.0004) were significantly higher in active compared to inactive LN. Activation of complement was associated with the occurrence of anti-C1q antibodies and reduced complement C1q. Complement-activation fragments highly correlated with the markers for immune system and endothelial cell activation. The ensemble of these parameters may be of great value in identifying early flares or remissions of childhood LN, and moreover may prove useful in the assessment of new treatments and in determining the optimization of their use.


Anti-C1q antibodies B cell activating factor C1q Complement activation products Lupus nephritis Soluble VCAM-1 RANTES