Immunological features of patients affected by Barraquer-Simons syndrome.

Fernando CorvilloGiovanni CeccariniPilar NozalSilvia MagnoCaterina PelosiniSofía GarridoAlberto López-LeraManuela MoraruCarlos VilchesSilvia FornaciariSabrina GabbrielliniFerruccio SantiniDavid Araújo-VilarMargarita López-Trascasa

Published in: Orphanet journal of rare diseases (2020)

Our results confirmed the association between BSS, autoimmunity and C3 hypocomplementemia. Moreover, the finding of autoantibodies targeting complement system proteins points to complement dysregulation as a central pathological event in the development of BSS.

Keyphrases

end stage renal disease
ejection fraction
newly diagnosed
systemic lupus erythematosus
prognostic factors
case report
patient reported outcomes