Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Sunday, April 02, 2006

Protein-losing enteropathy in systemic lupus erythematosus associated with intestinal lymphangiectasia

Edworthy SM, Fritzler MJ, Kelly JK, McHattie JD, Shaffer EA.

Department of Medicine, University of Calgary, Alberta, Canada.

This case report concerns a young woman with systemic lupus erythematosus who presented with a protein-losing enteropathy. Intestinal biopsy revealed lymphangiectasia. Mesenteric lymph nodes showed paracortical lymphoid depletion and multiple small sinusoids. Elevated cholesterol levels and normal lymphocyte counts characterized the peripheral blood samples. Lymph nodes, though enlarged, showed no evidence of obstructive pathology. No secondary cause of lymphangiectasia could be identified. The patient exhibited antinuclear antibodies and antibodies to dsDNA at the onset, and then, 5 yr later, the classic features of systemic lupus erythematosus (SLE). The lymphocytopenia and hypolipidemia that characterizes lymphangiectasia is not a feature of the SLE cases reported to date. Furthermore, the mechanism for the dilated lymphatics and villous edema is more likely immunological than mechanical disruption of lymphatics.

Publication Types:
Case ReportsPMID: 2220736 [PubMed - indexed for MEDLINE]

* * * * *

Protein-losing enteropathy associated with systemic lupus erythematosus: response to cyclophosphamide.

Werner de Castro GR, Appenzeller S, Bertolo MB, Costallat LT.

Rheumatology Unit, Department of Internal Medicine, Faculty of Medical Sciences, State University of Campinas, Campinas, Brazil.

Protein-losing enteropathy is a rare manifestation of systemic lupus erythematosus (SLE) leading to hypoalbuminemia and anasarca. We report the case of a woman with SLE who presented chronic hypoalbuminemia diagnosed as protein-losing enteropathy associated with SLE. She was refractory to prednisone and azathioprine administration but showed good response to cyclophosphamide. The diagnosis and management of hypoalbuminemia in lupus-associated enteropathy are discussed.

PMID: 15249982 [PubMed - indexed for MEDLINE]

* * * * *

A Systemic Lupus Erythematosus Patient with Protein Losing Enteropathy


Jee Min Park1, Sun Young Ahn1, Jae Il Shin1, Mi Jin Yun2, and Jae Seung Lee1


The Institute of Kidney Disease, 1Department of Pediatrics and 2Department of Nuclear Medicine, Yonsei University College of Medicine, Seoul, Korea.


Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and 99mTc-human serum albumin scintigraphy.


24-hour stool alpha-1 antitrypsin clearance, 99mTc-human serum albumin scintigraphy

Corresponding Author info

Dr. Jae Seung Lee, Department of Pediatrics, Division of Nephrology, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemun-gu, Seoul 120-752, Korea. Tel: 82-2-361-5517, Fax: 82-2-393-9118, E-mail: jsyonse@

Full Text PDF Yonsei Medical Journal

* * * * *

Related Article - Systemic Lupus Erythematosus

Systemic Lupus Erythematosus - eMedicine