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

Palman, J; May, J; Pilkington, C; (2016) Macrophage activation syndrome triggered by coeliac disease: a unique case report. Pediatric Rheumatology , 14 , Article 66. 10.1186/s12969-016-0128-y. Green open access

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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.

Type: Article
Title: Macrophage activation syndrome triggered by coeliac disease: a unique case report
Open access status: An open access version is available from UCL Discovery
DOI: 10.1186/s12969-016-0128-y
Publisher version: http://dx.doi.org/10.1186/s12969-016-0128-y
Language: English
Additional information: This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Keywords: Macrophage activation syndrome, Haemophagocytic lymphohistiocytosis, Coeliac disease, Autoimmune disease
UCL classification: UCL
URI: https://discovery-pp.ucl.ac.uk/id/eprint/1546301
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