A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy

Christine Firth, Lucinda A. Harris, Maxwell L. Smith, Leslie Francis Thomas

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)261-267
Number of pages7
JournalCase Reports in Nephrology and Dialysis
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Intestinal Polyposis
Membranous Glomerulonephritis
Proteinuria
Hyperpigmentation
Alopecia
Azathioprine
Skin
Cyclosporine
Dysgeusia
Kidney
Ranitidine
Kidney Diseases
Nails
Prednisone
Polyps
Weight Loss
Inflammation
Biopsy
Therapeutics

Keywords

  • Cronkhite-Canada syndrome
  • Membranous nephropathy
  • Polyposis syndrome
  • Proteinuria
  • Rituximab

ASJC Scopus subject areas

  • Nephrology

Cite this

A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy. / Firth, Christine; Harris, Lucinda A.; Smith, Maxwell L.; Thomas, Leslie Francis.

In: Case Reports in Nephrology and Dialysis, 01.01.2018, p. 261-267.

Research output: Contribution to journalArticle

@article{765e6f1ae2a147d5bd222f4d9965eae8,
title = "A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy",
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.",
keywords = "Cronkhite-Canada syndrome, Membranous nephropathy, Polyposis syndrome, Proteinuria, Rituximab",
author = "Christine Firth and Harris, {Lucinda A.} and Smith, {Maxwell L.} and Thomas, {Leslie Francis}",
year = "2018",
month = "1",
day = "1",
doi = "10.1159/000494714",
language = "English (US)",
pages = "261--267",
journal = "Case Reports in Nephrology and Dialysis",
issn = "2296-9705",
publisher = "S. Karger AG",

}

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 Francis

PY - 2018/1/1

Y1 - 2018/1/1

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.

AB - 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.

KW - Cronkhite-Canada syndrome

KW - Membranous nephropathy

KW - Polyposis syndrome

KW - Proteinuria

KW - Rituximab

UR - http://www.scopus.com/inward/record.url?scp=85057586720&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85057586720&partnerID=8YFLogxK

U2 - 10.1159/000494714

DO - 10.1159/000494714

M3 - Article

AN - SCOPUS:85057586720

SP - 261

EP - 267

JO - Case Reports in Nephrology and Dialysis

JF - Case Reports in Nephrology and Dialysis

SN - 2296-9705

ER -