A relapsing case of pulmonary-renal syndrome after a sequential rise in MPO-ANCA and anti-GBM antibodies. View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-04-03

AUTHORS

Akiko Hoshino, Toru Sakairi, Ken Kayakabe, Masahito Baba, Masayasu Ando, Hayato Kimura, Rena Motohashi, Yoshihisa Nojima, Keiju Hiromura

ABSTRACT

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. More... »

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s13730-019-00397-1

DOI

http://dx.doi.org/10.1007/s13730-019-00397-1

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1113182544

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30945174


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