Case report: anti-glomerular basement membrane antibody disease with normal renal function

BMC Nephrology

Yoshiyuki Kuroyanagi¹  Katuaki Kasahara¹  Yoshimitu Goto¹  China Nagano¹ 

[1] Japanese Red Cross Nagoya Daini Hospital, 2-9 Myoken-cho, Showa-ku, Nagoya City 466-8650, Aichi Prefecture, Japan

关键词: case report;    School urine screening program;    Normal renal function;    Pediatrics;    Anti-GBM;   

Others  :  1231058
DOI  :  10.1186/s12882-015-0179-1

 received in 2015-06-15, accepted in 2015-10-29,  发布年份 2015

【 摘 要 】

Background

Anti-glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis caused by autoantibodies against the α3-chain of type IV collagen in the GBM.

Case presentation

An 8-year-old girl with hematuria and proteinuria due to anti-GBM nephritis was diagnosed with hematuria and proteinuria during a school urine screening program. Her blood pressure and serum creatinine levels were normal. Her hematuria and proteinuria persisted for several months. Since a spot urine protein to creatinine ratio was around 7 g/g Cre, a percutaneous renal biopsy was performed. Immnofluorescent staining demonstrated a linear pattern for immunoglobulin G along the entire GBM. Chest computed tomography was normal. Anti-GBM antibody assays were reported as slightly raised; thus, the diagnosis was anti-GBM disease with normal renal function. Treatment included plasma exchange, intravenous high-dose methylprednisolone, and cyclophosphamide as a mainstay medication. The treatment was rapidly effective with an immediate decrease in anti-GBM titers and proteinuria.

Conclusions

Cases of anti-GBM disease with normal renal function in children are rare. Treatment in children has not been established; therefore, clinicians need to carefully select an effective treatment because the prognosis is poor.

【 授权许可】

   
2015 Nagano et al.

【 图 表 】

Fig. 1.

【 参考文献 】

• [1]Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture's disease (anti-GBM). J Autoimmun. 2014; 48–49:108-112.
• [2]Bayat A, Kamperis K, Herlin T. Characteristics and outcome of Goodpasture's disease in children. Clin Rheumatol. 2012; 31:1745-1751.
• [3]Silvariño R, Noboa O, Cervera R. Anti-glomerular basement membrane antibodies. Isr Med Assoc J. 2014; 16:727-732.
• [4]Salama AD, Levy JB, Lightstone L, Pusey CD. Goodpasture's disease. Lancet. 2001; 358:917-920.
• [5]Cui Z, Zhao MH, Singh AK, Wang HY. Antiglomerular basement membrane disease with normal renal function. Kidney Int. 2007; 72:1403-1408.
• [6]Ang C, Savige J, Dawborn J, Miach P, Heale W, Clarke B et al.. Anti-glomerular basement membrane (GBM)-antibody-mediated disease with normal renal function. Nephrol Dial Transplant. 1998; 13:935-939.
• [7]Cui Z, Wang HY, Zhao MH. Natural autoantibodies against glomerular basement membrane exist in normal human sera. Kidney Int. 2006; 69:894-899.
• [8]Williamson SR, Phillips CL, Andreoli SP, Nailescu C. A 25-year experience with pediatric anti-glomerular basement membrane disease. Pediatr Nephrol. 2011; 26:85-91.
• [9]Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Soc Nephrol. 1999; 10:2446-2453.
• [10]Kumazaki S, Umeda Y, Sato K, Mishima H, Ishihara T, Uzawa T. [Double filtration plasmapheresis in case of Goodpasture's syndrome]. Nihon Kyobu Shikkan Gakkai Zasshi. 1990;28:628–633. [Article in Japanese]
• [11]Hajime N, Michiko A, Atsunori K, Tatsuo K, Yuko N, Naoki O et al.. A case report of efficiency of double filtration plasmapheresis in treatment of Goodpasture's syndrome. Ther Apher Dial. 2009; 13:373-377.