Article

Adult-onset Still's disease and chronic recurrent multifocal osteomyelitis: a hitherto undescribed manifestation of autoinflammation.

Department of Medicine 3, University Erlangen-Nuremberg, Krankenhausstr. 12, 91054, Erlangen, Germany.
Rheumatology International (impact factor: 1.88). 07/2011; 32(6):1827-9. DOI:10.1007/s00296-011-2020-x pp.1827-9
Source: PubMed

ABSTRACT Still's disease and chronic recurrent multifocal osteomyelitis (CRMO) are febrile rheumatic diseases of unknown etiology, which predominantly affect children but can also have their initial manifestation in adults. Both can present as intermittent, relapsing episodes and are considered potential candidates within the expanding spectrum of autoinflammatory disorders, although no genetic abnormalities have been described for either of them. Here, we describe a man with an initial manifestation of abacterial multifocal osteitis at the age of 41. During a relapsing-remitting course of his illness, he increasingly developed symptoms of adult-onset Still's disease (AOSD), and the diagnosis was established according to the Yamaguchi criteria. When treated with anakinra, not only the acute symptoms disappeared promptly, but also the osteitis went into complete remission. This is to our knowledge the first description of a simultaneous occurrence of these two manifestations of autoinflammation in adulthood.

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Keywords

adult-onset Still's disease
 
affect children
 
AOSD
 
autoinflammatory disorders
 
chronic recurrent multifocal osteomyelitis
 
CRMO
 
expanding spectrum
 
genetic abnormalities
 
initial manifestation
 
relapsing episodes
 
relapsing-remitting course
 
simultaneous occurrence
 
Still's disease
 
two manifestations
 
Yamaguchi criteria