Nephrotic syndrome caused by immune-mediated acquired LCAT deficiency.

Abstract

Lecithin-cholesterol acyltransferase (LCAT) is an enzyme involved in maintaining cholesterol homeostasis. In familial LCAT deficiency (FLD), abnormal lipid deposition causes renal injury and nephrotic syndrome, frequently progressing to ESRD. Here, we describe a 63-year-old Japanese woman with no family history of renal disease who presented with nephrotic syndrome. The laboratory data revealed an extremely low level of serum HDL and undetectable serum LCAT activity. Renal biopsy showed glomerular lipid deposition with prominent accumulation of foam cells, similar to the histologic findings of FLD. In addition, she had subepithelial electron-dense deposits compatible with membranous nephropathy, which are not typical of FLD. A mixing test and coimmunoprecipitation study demonstrated the presence of an inhibitory anti-LCAT antibody in the patient's serum. Immunohistochemistry and immunofluorescence detected LCAT along parts of the glomerular capillary walls, suggesting that LCAT was an antigen responsible for the membranous nephropathy. Treatment with steroids resulted in complete remission of the nephrotic syndrome, normalization of serum LCAT activity and HDL level, and disappearance of foam cell accumulation in renal tissue. In summary, inhibitory anti-LCAT antibody can lead to glomerular lesions similar to those observed in FLD.

• Authors: Satoshi Takahashi, Keiju Hiromura, Mayuko Tsukida, Yuko Ohishi, Hiroko Hamatani, Noriyuki Sakurai, Toru Sakairi, Hidekazu Ikeuchi, Yoriaki Kaneko, Akito Maeshima, Takashi Kuroiwa, Hideaki Yokoo, Takeo Aoki, Michio Nagata, Yoshihisa Nojima
• Journal: Journal of the American Society of Nephrology : JASN (J Am Soc Nephrol) Vol. 24 Issue 8 Pg. 1305-12 (Jul 2013) ISSN: 1533-3450 [Electronic] United States
• PMID: 23620397 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Female
• Fluorescent Antibody Technique
• Glomerulonephritis, Membranous (etiology, pathology)
• Humans
• Immunohistochemistry
• Kidney (pathology)
• Lecithin Cholesterol Acyltransferase Deficiency (complications, diagnosis, pathology)
• Middle Aged
• Nephrotic Syndrome (etiology, pathology)