“It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Sarah J. van der Lely, Jeffrey Boorsma, Marc Hilhorst, Jesper Kers, Joris Roelofs, Lily Jakulj, Marieke van Onna

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE).Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.
Original languageEnglish
Pages (from-to)495-501
Number of pages7
JournalLupus
Volume30
Issue number3
Early online date2021
DOIs
Publication statusPublished - Mar 2021

Keywords

  • Lupus nephritis
  • mesangiocapillary glomerulonephritis
  • nephrotic syndrome
  • paraneoplastic
  • premature diagnostic closure

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