IgG4 releated disease


Authors: Z. Adam 1;  M. Krejčí 1;  Z. Chovancová 2;  Z. Řehák 4,5;  R. Koukalová 3;  M. Štork 1;  A. Čermák 6;  L. Říhová 3;  L. Pour 1
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno 1;  Ústav klinické imunologie a alergologie LF MU a FN u svaté Anny Brno 2;  Laboratoř průtokové cytometrie Oddělení klinické hematologie LF MU a FN Brno 3;  Oddělení nukleární medicíny Masarykův onkologický ústav (MOU) 4;  Regionální centrum molekulární onkologie (RECAMO) Masarykův onkologický ústav 5;  Urologická klinika LF MU a FN Brno 6
Published in: Transfuze Hematol. dnes,24, 2018, No. 2, p. 78-86.
Category:

Overview

Ig-G4 related disease is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the start of this century only. Almost all organs can be involved by this disease, but mostly is manifested in gastrointestinal trakt and pankreas. In some patients it can cause retroperitoneal fibrosis and in some patients may be present as lymfadenopathy. Diagnosis involves establishing high circulating levels of IgG4 > 135 mg/dL, increased serum IgG4 to IgG ratio of > 8%, immunohistochemistry showing dense lymphoplasmacellular inflammatory infiltrate consisting of IgG4-positive plasma cells with storiform fibrosis and obliterative phlebitis, and increased IgG4 positive plasma cell. Glucocorticoids are the primary form of therapy. Rituximab is the second line of therapy in case of no responce to kortikosteroids or intolerance of corticosteroids.

Key words:

IgG4 releated disease – IgG4 subclass of immuniglobuline – corticosteroids – rituximab


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