Clinical importance of the IgG4 related disease


Authors: Zbyněk Hrnčíř 1;  Jan Laco 2
Authors‘ workplace: Univerzita Karlova v Praze, Lékařská fakulta v Hradci Králové, II. interní klinika 1;  Univerzita Karlova v Praze, Lékařská fakulta v Hradci Králové, Fingerlandův ústav patologie 2
Published in: Čas. Lék. čes. 2011; 150: 438-441
Category: Review Articles

Overview

IgG4 related disease is a chronic immunopathological disorder, proposed as a new diagnostic entity, and characterized as follows: 1. background is in multiorgan lymphoplasmocellular infiltration associated with presence of large number of polyclonal IgG4+ plasma cells, and fibrosclerosis; 2. structural damage of the affected organs is frequently associated with pseudotumorous enlargement and/or other morphological changes; for example aortitis with aneurysmatic dilatation, sclerosing pancreatitis, retroperitoneal fibrosis ect.; 3. serum concentration of IgG4 is usually significantly elevated; 4. responsivity to glucocorticoids is a hallmark of the disease: in rare cases of glucocorticoid-resistance a response to rituximab was observed. At presence the disease related to IgG4 is considered as a rare disorder. Presented knowledge may be useful for better elucidation of its importance in clinical specialities.

Key words:
IgG4-related disease, IgG4+ plasma cells, IgG4 serum concentration, pseudotumorous enlargement, glucocorticoid-responsivity, rituximab.


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