J 2022

Léčba nemocnění asociovaného s imunoglobulinem IgG4

ADAM, Zdeněk, Milan DASTYCH, Aleš ČERMÁK, Martina DOUBKOVÁ, Šárka SKORKOVSKÁ et. al.

Basic information

Original name

Léčba nemocnění asociovaného s imunoglobulinem IgG4

Name (in English)

Therapy of immunoglonuline IgG4 related disease (IgG4-RD)

Authors

ADAM, Zdeněk (203 Czech Republic, guarantor, belonging to the institution), Milan DASTYCH (203 Czech Republic, belonging to the institution), Aleš ČERMÁK (203 Czech Republic, belonging to the institution), Martina DOUBKOVÁ (203 Czech Republic, belonging to the institution), Šárka SKORKOVSKÁ (203 Czech Republic, belonging to the institution), Luděk POUR (203 Czech Republic, belonging to the institution), Zdeněk ŘEHÁK (203 Czech Republic), Renata KOUKALOVÁ (203 Czech Republic), Zuzana ADAMOVÁ (203 Czech Republic), Martin ŠTORK (203 Czech Republic, belonging to the institution), Marta KREJČÍ (203 Czech Republic, belonging to the institution) and Ivanna BOICHUK (203 Czech Republic, belonging to the institution)

Edition

Vnitřní lékařství, Praha, Česká lékařská společnost J. E. Purkyně, 2022, 0042-773X

Other information

Language

Czech

Type of outcome

Článek v odborném periodiku

Field of Study

30203 Respiratory systems

Country of publisher

Czech Republic

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

RIV identification code

RIV/00216224:14110/22:00128161

Organization unit

Faculty of Medicine

Keywords (in Czech)

Abetacept; Dupilimab; Glucocorticoids; IgG4-related disease; Immunosuppressive agents; Rituximab; Sirolimus; Takrolimus

Keywords in English

Abetacept; Dupilimab; Glucocorticoids; IgG4-related disease; Immunosuppressive agents; Rituximab; Sirolimus; Takrolimus

Tags

Tags

Reviewed
Změněno: 8/2/2023 14:33, Mgr. Tereza Miškechová

Abstract

V originále

Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects. In case of combination glucocorticoids and immunosuppressive agents lower doses of glucocorticoids are needed, the response rate is higher and therapy is better tolerated. Rituximab is drug, that is possible use as monotherapy or in combination with glucocorticoids and immunosuppressive drugs. Only one study compared two immunosuporessive drugs, mycophenolate mofetil and cyclophosphamide. The response rated was similar but remissions were longer after glucocorticoids with cyclophosphamide then glucocorticoids with mycofenolat mofetil. No other comparative study of combination of various imunossupressive drugs with glucocorticoids was published. Rituximab has high number (90%) of response rate in monotherapy, but can be used in combination with glucocorticoids and immunosuppressives. Rituximab is now preferred and recommended for maintenance therapy administered in 6-month interval. In case of advanced disease, we prefer therefore combination of rituximab, cyclofosphamide and dexamethasone for initial therapy followed by maintenance with rituximab in 6 months interval. There are two new drugs under investigation abatacept and dupilimab with promising results. Although we have very intensive therapies for good results of therapy early diagnosis before irreversible fibrotic changes in IgG4-RD involved organs is still needed. © 2022, SOLEN s.r.o.. All rights reserved.

In English

Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects. In case of combination glucocorticoids and immunosuppressive agents lower doses of glucocorticoids are needed, the response rate is higher and therapy is better tolerated. Rituximab is drug, that is possible use as monotherapy or in combination with glucocorticoids and immunosuppressive drugs. Only one study compared two immunosuporessive drugs, mycophenolate mofetil and cyclophosphamide. The response rated was similar but remissions were longer after glucocorticoids with cyclophosphamide then glucocorticoids with mycofenolat mofetil. No other comparative study of combination of various imunossupressive drugs with glucocorticoids was published. Rituximab has high number (90%) of response rate in monotherapy, but can be used in combination with glucocorticoids and immunosuppressives. Rituximab is now preferred and recommended for maintenance therapy administered in 6-month interval. In case of advanced disease, we prefer therefore combination of rituximab, cyclofosphamide and dexamethasone for initial therapy followed by maintenance with rituximab in 6 months interval. There are two new drugs under investigation abatacept and dupilimab with promising results. Although we have very intensive therapies for good results of therapy early diagnosis before irreversible fibrotic changes in IgG4-RD involved organs is still needed. © 2022, SOLEN s.r.o.. All rights reserved.