A relapsing case of pulmonary–renal syndrome after a sequential rise in MPO–ANCA and anti-GBM antibodies

  • Akiko Hoshino
  • Toru SakairiEmail author
  • Ken Kayakabe
  • Masahito Baba
  • Masayasu Ando
  • Hayato Kimura
  • Rena Motohashi
  • Yoshihisa Nojima
  • Keiju Hiromura
Case Report


A 69-year-old woman who presented with severe renal dysfunction and diffuse alveolar hemorrhage was diagnosed with pulmonary–renal syndrome (PRS) based on the coexistence of serum myeloperoxidase (MPO)–antineutrophil cytoplasmic antibodies (ANCA) and anti-glomerular basement membrane (GBM) antibodies (Ab). Hemodialysis was started; plasma exchange and intravenous methylprednisolone pulse therapy were administered followed by oral prednisolone administration. Pulmonary hemorrhage decreased; however, renal dysfunction persisted. On maintenance hemodialysis and prednisolone therapy, MPO–ANCA and anti-GBM Ab became negative at 4 and 10 months, respectively; thereafter, they became positive again at 18 and 35 months, respectively. At 36 months, there was relapse of pulmonary hemorrhage. Plasma exchange and intravenous methylprednisolone pulse therapy were administered; pulmonary hemorrhage ceased, and both antibodies became negative. It is known that PRS cases that are double positive for ANCA and anti-GBM Ab occasionally relapse after remission, and, even though they are double positive at initial diagnosis, most relapses occur with reappearance or re-elevation of ANCA but with absence of anti-GBM-Ab. Therefore, this was a rare relapsing case that presented with double-positive serology. Further, our observation that the reappearance of ANCA preceded that of anti-GBM-Ab suggests that ANCA contribute to the reproduction of anti-GBM Ab.


Anti-GBM antibody MPO–ANCA Pulmonary–renal syndrome Rapidly progressive glomerulonephritis Relapse 



This case report was presented in the 18th international vasculitis and ANCA Workshop (Tokyo, Japan, 2017).

Compliance with ethical standards

Conflict of interest

Authors declare no conflict of interest.

Informed consent

Informed consent was obtained from the patient for publication of this case report.


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Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  1. 1.Division of Rheumatology and NephrologyMaebashi Red Cross HospitalMaebashiJapan
  2. 2.Department of Nephrology and RheumatologyGunma University Graduate School of MedicineMaebashiJapan

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