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1563459 
Journal Article 
Extra-articular manifestations of rheumatoid arthritis: An update 
Prete, M; Racanelli, V; Digiglio, L; Vacca, A; Dammacco, F; Perosa, F 
2011 
Yes 
Autoimmunity Reviews
ISSN: 1568-9972
EISSN: 1873-0183 
11 
2 
123-131 
21939785 
WOS:000299065600007 
Rheumatoid arthritis (RA) is an immune-mediated disease
involving chronic low-grade inflammation that may progressively lead to joint destruction,
deformity, disability and even death. Despite its predominant osteoarticular and periarticular
manifestations, RA is a systemic disease often associated with cutaneous and organ-specific
extra-articular manifestations (EAM). Despite the fact that EAM have bee.a studied in aumerous RA
cohorts, there is no uniformity in their definition or classification. This paper reviews current
knowledge about EAM in terms of frequency, clinical aspects and current therapeutic approaches.
In an initial attempt at a classification, we separated EAM from RA co-morbidities and from
general, constitutional manifestations of systemic inflammation. Moreover, we distinguished EAM
into cutaneous and visceral forms, both severe and not severe. In aggregated data from 12 large
RA cohorts, patients with EAM, especially the severe forms, were found to have greater co-
morbidity and mortality than patients without EAM. Une.erstanding the complexity of EAM and their
management remains a challenge for clinicians, especially since the effectiveness of drug therapy
on EAM has not been systematically evaluated in randomized clinical trials. (C) 2011 Elsevier
B.V. All rights reserved. 
Rheumatoid arthritis; Extra-articular manifestation; Co-morbidities; Classification; Incidence/rate; Vasculitides; Amyloidosis; Felty's syndrome