Protein-losing enteropathy associated with or without systemic autoimmune disease

Law, Siu-Tong; Man, Kwok; Li, Kin Kong

doi:10.1097/meg.0b013e32834f3ea0

Cited by 8 publications

(2 citation statements)

References 28 publications

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“…The exact mechanism of PLGE in Sjögren's syndrome remains unclear. 12 , 15 Duodenal biopsies from our patient showed architecturally normal mucosa with increased intraepithelial lymphocytes. Intestinal biopsies described in prior case reports all showed chronic inflammatory cell infiltration.…”

Section: Discussionmentioning

confidence: 56%

Protein-Losing Gastroenteropathy Associated With Sjögren's Syndrome: First Known Case Reported Outside of Asia

Gupta

Cohen

McCarthy

et al. 2015

ACG Case Reports Journal

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Protein-losing gastroenteropathy (PLGE) is a rare extraglandular manifestation of Sjögren's syndrome, reported in fewer than 10 cases. We report a 58-year-old white woman with Sjögren's syndrome, type 1 renal tubular acidosis, and PLGE, who presented with cachexia and 100-pound weight loss. The diagnosis was made based on hypoalbuminemia in the absence of significant proteinuria, low levels of fat soluble vitamins, low transferrin, and an elevated alpha-1 antitrypsin (A1AT) fecal clearance, supported by imaging and endoscopy, with biopsy showing lymphocytic infiltration. She was successfully treated with cyclophosphamide and prednisone. To our knowledge, this is the first such case outside of Asia.

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Section: Discussionmentioning

confidence: 56%

Protein-Losing Gastroenteropathy Associated With Sjögren's Syndrome: First Known Case Reported Outside of Asia

Gupta

Cohen

McCarthy

et al. 2015

ACG Case Reports Journal

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“…Lupus protein-losing enteropathy (LUPLE) represents the third most common type of GI involvement in cSLE, following lupus mesenteric vasculitis and lupus intestinal pseudo-obstruction [71,92].…”

Section: Protein-losing Enteropathymentioning

confidence: 99%

Gastrointestinal Involvement in Children with Systemic Lupus Erythematosus

et al. 2023

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Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder. When it presents before the age of 18 years (childhood-onset systemic lupus erythematosus, cSLE), the disease course tends to be more severe with a higher rate of organ involvement and requires an early diagnosis. Gastrointestinal involvement in cSLE is rare and scarcely reported in the literature. Any organ of the gastrointestinal system may be affected, either as a direct consequence of the disease, as a subsequent complication, or as an adverse drug event. Abdominal pain is the most common GI symptom, it can be diffuse or well localized, and can underline different conditions such as hepatitis, pancreatitis, appendicitis, peritonitis, or enteritis. cSLE may have an alteration of the intestinal barrier with features of protein-losing enteropathy or, in genetically predisposed patients, may develop associated autoimmune disorders such as Coeliac Disease or Autoimmune Hepatitis. The aim of this manuscript is to provide a narrative review of gastrointestinal manifestations in cSLE focused on hepatic, pancreatic, and intestinal involvement. A comprehensive literature search based on the PubMed database was performed.

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ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia

et al. 2021

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Protein-losing enteropathy associated with or without systemic autoimmune disease

Cited by 8 publications

References 28 publications

Protein-Losing Gastroenteropathy Associated With Sjögren's Syndrome: First Known Case Reported Outside of Asia

Protein-Losing Gastroenteropathy Associated With Sjögren's Syndrome: First Known Case Reported Outside of Asia

Gastrointestinal Involvement in Children with Systemic Lupus Erythematosus

ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia

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