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Atypical inflammatory myopathy associated with Crohn's disease
1Department of Neurology, Friedrich Alexander University of Erlangen-Nürnberg, Germany.
Introduction:
We report on a 37-year-old woman with quiescent Crohn's disease who developed steroid responsive myositis. Although there are some case reports about inflammatory myopathy in Crohn's disease, the myopathological characteristics have still to be detailed.
Material And Methods:
Open muscle biopsy was performed on the left medial gastrocnemius muscle. For light-microscopical investigation conventional and immunohistochemical detection procedures were employed.
Results:
Focal necrotic changes together with peri- and endomysial inflammatory infiltrates were found predominantly consisting of CD67- and CD68-positive cells accompanied by CD8-and CD4-positive ones. Especially at sites of otherwise normal muscle area CD8-positive cells were seen, 100. Furthermore, severe inflammatory infiltration of connective tissue septa and the occurrence of granulocytes are striking features of the inflammatory myopathy in this condition.
Conclusion:
We report a probably T cell-mediated inflammatory myopathy associated with preceding Crohn's disease. The myositis is characterized by features which are unknown in disorders constituting the polymyositis and dermatomyositis syndromes. Thus, Crohn's disease associated "focal necrotizing neutrophilic myositis" probably has to be regarded as a specific myositis entity.