ADAM, Zdeněk, Milan DASTYCH, Aleš ČERMÁK, Martina DOUBKOVÁ, Šárka SKORKOVSKÁ, Luděk POUR, Zdeněk ŘEHÁK, Renata KOUKALOVÁ, Zuzana ADAMOVÁ, Martin ŠTORK, Marta KREJČÍ and Ivanna BOICHUK. Léčba nemocnění asociovaného s imunoglobulinem IgG4 (Therapy of immunoglonuline IgG4 related disease (IgG4-RD)). Vnitřní lékařství. Praha: Česká lékařská společnost J. E. Purkyně, 2022, vol. 68, No 6, p. "E15"-"E22", 8 pp. ISSN 0042-773X. Available from: https://dx.doi.org/10.36290/vnl.2022.086.
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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
Original language Czech
Type of outcome Article in a journal
Field of Study 30203 Respiratory systems
Country of publisher Czech Republic
Confidentiality degree is not subject to a state or trade secret
WWW URL
RIV identification code RIV/00216224:14110/22:00128161
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.36290/vnl.2022.086
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
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Tags Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 8/2/2023 14:33.
Abstract
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.
Abstract (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.
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