Myeloperoxidase-antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis in autosomal dominant polycystic kidney disease

BMC Nephrology

Kenichi Ohashi¹  Kenmei Takaichi²  Naoki Sawa²  Masayuki Yamanouchi²  Eiko Hasegawa²  Rikako Hiramatsu²  Tatsuya Suwabe²  Noriko Hayami²  Junichi Hoshino²  Yoshifumi Ubara²  Keiichi Sumida² 

[1]Department of Pathology, Toranomon Hospital, Tokyo, Japan

[2]Nephrology Center, Toranomon Hospital, Tokyo, Japan

关键词: Autosomal dominant polycystic kidney disease;    Crescentic glomerulonephritis;    Antineutrophil cytoplasmic antibody;    Myeloperoxidase;   

Others  :  1082945
DOI  :  10.1186/1471-2369-14-94

 received in 2012-11-03, accepted in 2013-04-17,  发布年份 2013

【 摘 要 】

Background

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder that is characterized by the development of cysts in the kidneys and other organs. Urinary protein excretion is usually less than 1 g/day, and ADPKD is rarely associated with nephrotic syndrome or rapidly progressive glomerulonephritis (RPGN). To date, myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CrGN) has not been reported in a patient with ADPKD.

Case presentations

We report two cases of MPO-ANCA positive ADPKD. A 60-year-old Japanese woman (case 1) and a 54-year-old Japanese woman (case 2) presented with RPGN featuring severe proteinuria and microscopic hematuria. In both patients percutaneous needle biopsy of the kidney revealed MPO-ANCA-associated CrGN with a paucity of glomerular immunoglobulin staining. Each patient received intravenous methylprednisolone for 3 days, followed by oral prednisolone. Case 1 showed gradual improvement and has not progressed to end-stage renal disease (ESRD), but case 2 developed ESRD requiring hemodialysis within one month despite treatment.

Conclusion

These are the first two reported cases of MPO-ANCA-associated CrGN in patients with ADPKD. Our experience suggests that serial measurement of the ANCA titer and renal biopsy should be considered for accurate diagnosis and appropriate treatment of ADPKD patients who present with proteinuria, hematuria, and rapid decline of renal function.

【 授权许可】

   
2013 Sumida et al.; licensee BioMed Central Ltd.

【 图 表 】

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