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Protein losing enteropathy as the initial manifestation of childhood systemic lupus erythematosus
J F Molina1, R F Brown, A Gedalia
1Division of Rheumatology, Children's Hospital, New Orleans, Louisiana, USA.
Insights
Systemic lupus erythematosus (SLE) can affect the gastrointestinal system, causing protein-losing enteropathy (PLE). Children presenting with PLE should be evaluated for SLE, as it is a rare but possible cause of their symptoms.
Area of Science:
- Rheumatology
- Pediatrics
- Gastroenterology
Background:
- Systemic lupus erythematosus (SLE) is a complex autoimmune disease affecting multiple organ systems.
- Gastrointestinal (GI) involvement in SLE is uncommon, making diagnosis challenging.
- Protein-losing enteropathy (PLE) is a condition characterized by excessive protein loss through the GI tract.
Observation:
- A case report details a 13-year-old female presenting with abdominal pain, diarrhea, edema, and hypoalbuminemia.
- Diagnostic evaluation revealed protein-losing enteropathy (PLE) confirmed by elevated fecal alpha 1-antitrypsin clearance.
- The patient subsequently developed clinical and serological criteria diagnostic of SLE.
Findings:
- This case adds to the limited number of reported pediatric cases of SLE-associated PLE (3 out of 22 total).
- The findings highlight the potential for PLE as an initial presentation of SLE in children.
- Early recognition of PLE in SLE patients is crucial for appropriate management.
Implications:
- Pediatric patients with unexplained PLE should be screened for SLE.
- Physicians treating SLE patients should consider PLE as a differential diagnosis for unexplained edema and hypoalbuminemia.
- Further research is needed to understand the pathogenesis and optimize treatment strategies for lupus-associated PLE in children.
Abstract:
Systemic lupus erythematosus (SLE) is a multisystem organ disease, and involvement of the gastrointestinal system is relatively rare. We describe a 13-year-old girl who presented initially with abdominal pain, diarrhea, edema, and hypoalbuminemia. She was diagnosed with protein losing enteropathy (PLE) based on the significant increase of alpha 1-antitrypsin clearance in the stool. Two weeks after admission she developed clinical and serological findings that fulfilled the ACR criteria for SLE. Over 22 cases of lupus associated PLE have now been reported, but only 3 in children. Children with PLE should be evaluated for SLE. In addition, PLE should be suspected as a possible cause of unexplained edema and/or hypoalbuminemia in SLE.