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

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

Absence of Expression of c-sis and Transforming Growth Factor-β mRNA in Malignant Fibrous Histiocytoma

Total RNA was extracted from five malignant fibrous histiocytomas and two benign fibrohistiocytic lesions and assayed for mRNA expressions for transforming growth factor beta (TGF-β) and c-sis by Northern blot analysis. Production of both of these has been associated with cells of monocyte-macrophage lineage, and these factors have been shown to be important in physiologic mesenchymal cell proliferation. No mRNA expression of either TGF-β or c-sis was identified in any of the fibrohistiocytic tumor samples. The lack of expression of TGF-β and c-sis may be consistent with a nonhistiocytic origin of malignant fibrous histiocytoma, or may reflect transformation- associated loss of the normal molecular mechanisms of mesenchymal proliferation. The absence of c-sis mRNA expression can be reconciled with the prior immunohisto chemical demonstration of platelet-derived growth factor in tumor cells of malignant fibrous histiocytoma. Int J Surg Pathol(2):117-122, 1993Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/67413/2/10.1177_106689699300100205.pd