Current Understanding of the Pathogenesis and Management of Chronic Recurrent Multifocal Osteomyelitis |
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Authors: | Polly J Ferguson Monica Sandu |
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Institution: | Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA 52240, USA. polly-ferguson@uiowa.edu |
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Abstract: | Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory disorder that primarily affects children. Its hallmark
is recurring episodes of sterile osteomyelitis. The clinical presentation is insidious onset of bone pain with or without
fever. Laboratory studies typically reveal nonspecific evidence of inflammation. Radiologic imaging and histologic appearance
resemble those of infectious osteomyelitis. There is a strong association with inflammatory disorders of the skin and intestinal
tract in affected individuals and their close relatives, suggesting a shared pathophysiology and supporting a genetic component
to disease susceptibility. Two genetic syndromes have CRMO as a prominent phenotype—Majeed syndrome and deficiency of the
interleukin-1 receptor antagonist—and suggest that interleukin-1 may be a key cytokine in disease pathogenesis. This review
briefly summarizes the main clinical and radiologic aspects of the disease and then focuses on genetics and pathophysiology
and provides an update on treatment. |
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