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

被引：5

作者：

Sumida, Keiichi ^{[1
]}

Ubara, Yoshifumi ^{[1
]}

Hoshino, Junichi ^{[1
]}

Hayami, Noriko ^{[1
]}

Suwabe, Tatsuya ^{[1
]}

Hiramatsu, Rikako ^{[1
]}

Hasegawa, Eiko ^{[1
]}

Yamanouchi, Masayuki ^{[1
]}

Sawa, Naoki ^{[1
]}

Takaichi, Kenmei ^{[1
]}

Ohashi, Kenichi ^{[2
]}

机构：

[1] Toranomon Gen Hosp, Nephrol Ctr, Tokyo, Japan

[2] Toranomon Gen Hosp, Dept Pathol, Tokyo, Japan

来源：

BMC NEPHROLOGY | 2013年 / 14卷

关键词：

Myeloperoxidase; Antineutrophil cytoplasmic antibody; Crescentic glomerulonephritis; Autosomal dominant polycystic kidney disease; NEPHROTIC SYNDROME; NECROTIZING GLOMERULONEPHRITIS; RENAL-FAILURE; NEPHROPATHY;

D O I：

10.1186/1471-2369-14-94

中图分类号：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

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.

引用

页数：7

共 25 条

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