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A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy.
Case Rep Nephrol Dial. 2018 Sep-Dec; 8(3):261-267.CR

Abstract

Cronkhite-Canada syndrome (CCS) is a very rare disorder with less than 500 reported cases. It is characterized by extensive gastrointestinal polyposis and ectodermal anomalies including alopecia, cutaneous hyperpigmentation, and onychodystrophy. Only 3 cases of associated kidney disease (membranous nephropathy [MN]) have been reported. A 71-year-old male with CCS was referred for further evaluation of proteinuria. The patient initially presented with abdominal discomfort, weight loss, dysgeusia, skin hyperpigmentation, alopecia, and dystrophic nails. Endoscopic evaluation showed widespread gastrointestinal nodular inflammation and polyps. Histopathology was consistent with CCS. Initial treatment was with prednisone, azathioprine, and ranitidine. He had moderate clinical improvement but developed nephrotic-range proteinuria. Renal biopsy showed MN, and cyclosporine was started. The patient had significant improvement in his CCS manifestations; however, his proteinuria and renal function worsened. Rituximab was added to his regimen of cyclosporine and azathioprine, which resulted in remission of his MN, marked improvement in his polyposis, and near resolution of his cutaneous symptoms. This case represents a unique presentation of CCS associated with MN treated with rituximab. The excellent clinical response observed for both CCS and MN advocates consideration of this treatment, especially for refractory disease.

Authors+Show Affiliations

Department of Internal Medicine, Mayo Clinic in Arizona, Scottsdale, Arizona, USA.Division of Gastroenterology, Mayo Clinic in Arizona, Scottsdale, Arizona, USA.Department of Anatomic Pathology, Mayo Clinic in Arizona, Scottsdale, Arizona, USA.Division of Nephrology and Hypertension, Mayo Clinic in Arizona, Scottsdale, Arizona, USA.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

30643792

Citation

Firth, Christine, et al. "A Case Report of Cronkhite-Canada Syndrome Complicated By Membranous Nephropathy." Case Reports in Nephrology and Dialysis, vol. 8, no. 3, 2018, pp. 261-267.
Firth C, Harris LA, Smith ML, et al. A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy. Case Rep Nephrol Dial. 2018;8(3):261-267.
Firth, C., Harris, L. A., Smith, M. L., & Thomas, L. F. (2018). A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy. Case Reports in Nephrology and Dialysis, 8(3), 261-267. https://doi.org/10.1159/000494714
Firth C, et al. A Case Report of Cronkhite-Canada Syndrome Complicated By Membranous Nephropathy. Case Rep Nephrol Dial. 2018 Sep-Dec;8(3):261-267. PubMed PMID: 30643792.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy. AU - Firth,Christine, AU - Harris,Lucinda A, AU - Smith,Maxwell L, AU - Thomas,Leslie F, Y1 - 2018/11/27/ PY - 2018/07/27/received PY - 2018/10/19/accepted PY - 2019/1/16/entrez PY - 2019/1/16/pubmed PY - 2019/1/16/medline KW - Cronkhite-Canada syndrome KW - Membranous nephropathy KW - Polyposis syndrome KW - Proteinuria KW - Rituximab SP - 261 EP - 267 JF - Case reports in nephrology and dialysis JO - Case Rep Nephrol Dial VL - 8 IS - 3 N2 - Cronkhite-Canada syndrome (CCS) is a very rare disorder with less than 500 reported cases. It is characterized by extensive gastrointestinal polyposis and ectodermal anomalies including alopecia, cutaneous hyperpigmentation, and onychodystrophy. Only 3 cases of associated kidney disease (membranous nephropathy [MN]) have been reported. A 71-year-old male with CCS was referred for further evaluation of proteinuria. The patient initially presented with abdominal discomfort, weight loss, dysgeusia, skin hyperpigmentation, alopecia, and dystrophic nails. Endoscopic evaluation showed widespread gastrointestinal nodular inflammation and polyps. Histopathology was consistent with CCS. Initial treatment was with prednisone, azathioprine, and ranitidine. He had moderate clinical improvement but developed nephrotic-range proteinuria. Renal biopsy showed MN, and cyclosporine was started. The patient had significant improvement in his CCS manifestations; however, his proteinuria and renal function worsened. Rituximab was added to his regimen of cyclosporine and azathioprine, which resulted in remission of his MN, marked improvement in his polyposis, and near resolution of his cutaneous symptoms. This case represents a unique presentation of CCS associated with MN treated with rituximab. The excellent clinical response observed for both CCS and MN advocates consideration of this treatment, especially for refractory disease. SN - 2296-9705 UR - https://www.unboundmedicine.com/medline/citation/30643792/A_Case_Report_of_Cronkhite_Canada_Syndrome_Complicated_by_Membranous_Nephropathy_ L2 - https://www.karger.com?DOI=10.1159/000494714 DB - PRIME DP - Unbound Medicine ER -
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