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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2016

Open Access 01-12-2016 | Case Report

Macrophage activation syndrome triggered by coeliac disease: a unique case report

Authors: J. Palman, J. May, C. Pilkington

Published in: Pediatric Rheumatology | Issue 1/2016

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Abstract

Background

Macrophage activation syndrome is described as a “clinical syndrome of hyperinflammation resulting in an uncontrolled and ineffective immune response” in the context of an autoinflammatory or rheumatic disease. Current associations of macrophage activation syndrome with autoimmune disease most notably include a host of rheumatological conditions and inflammatory bowel disease. Epidemiological studies have shown that macrophage activation syndrome is precipitated by autoimmune disease more commonly than previously thought. Diagnosing the precipitating factor is essential for effective treatment and prognosis.

Case presentation

We report a case of a six year old girl with coeliac disease diagnosed after two episodes of secondary haemophagocytic lymphohistiocytosis. Her condition only responded to treatment once the patient was placed on a gluten free diet. Further immunological testing confirmed anti-transglutaminase and anti-endomysial antibodies, however histological biopsy was deemed inappropriate due to the severity of her condition. She has remained stable with no further episodes of macrophage activation syndrome since commencing a gluten free diet.

Conclusion

This case report is the first literature that links macrophage activation syndrome to coeliac disease and highlights the challenge of diagnosing coeliac disease with unusual features such as associated prolonged fever. Clinicians should have a low threshold for screening children with other autoimmune diseases for coeliac disease.
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Metadata
Title
Macrophage activation syndrome triggered by coeliac disease: a unique case report
Authors
J. Palman
J. May
C. Pilkington
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2016
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-016-0128-y

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