title: Macrophage activation syndrome triggered by coeliac disease: a unique case report.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: University College London Great Ormond Street Institute of Child Health, London, UK. jasonpalman@nhs.net.
authors.forename: J
authors.identifier: http://orcid.org/0000-0001-6783-4235
authors.initials: J
authors.lastname: Palman
authors.affiliation: Great Ormond Street Hospital, London, UK.
authors.forename: J
authors.initials: J
authors.lastname: May
authors.affiliation: University College London Great Ormond Street Institute of Child Health, London, UK.|Great Ormond Street Hospital, London, UK.
authors.forename: C
authors.initials: C
authors.lastname: Pilkington
mesh_terms: D002446:Celiac Disease / Q000150:complications* / Q000175:diagnosis; D002648:Child; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000209:etiology; D055501:Macrophage Activation Syndrome / Q000209:etiology*
keywords: Autoimmune disease; Coeliac disease; Haemophagocytic lymphohistiocytosis; Macrophage activation syndrome