Document Detail

IgG4-related disease.
MedLine Citation:
PMID:  23459998     Owner:  NLM     Status:  Publisher    
IgG4-related disease is a systemic fibroinflammatory syndrome of unknown etiology characterized by local inflammatory swelling and tumefactive lesions in one or several organs. It unifies several diseases previously thought to be unrelated. Recently, diagnostic criteria for the disease have been formulated and were complemented by an international consensus on histopathological assessment. In general, the disease activity can be rapidly controlled by high doses of prednisolone (0.6 mg/kg body weight); however, relapses, either local or in other regions, are frequent during tapering of the steroid dose. Commonly used steroid-sparing agents are only partially effective. Persistent local inflammatory activity may result in permanent organ damage. In refractory cases rituximab treatment has been used with good success. In the long-term care of affected patients a probable increased risk of malignancies (e.g. solid tumors and lymphoma) requires attention.
J Loock; B Manger
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-3-6
Journal Detail:
Title:  Zeitschrift fur Rheumatologie     Volume:  -     ISSN:  1435-1250     ISO Abbreviation:  Z Rheumatol     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-3-5     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0414162     Medline TA:  Z Rheumatol     Country:  -    
Other Details:
Languages:  GER     Pagination:  -     Citation Subset:  -    
Klinik für Rheumatologie und klinische Immunologie, Schön Klinikum Hamburg Eilbek, Dehnhaide 120, 22081, Hamburg, Deutschland,
Vernacular Title:
IgG4-assoziierte Erkrankung.
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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