Vnitr Lek 2022, 68(6):E15-E22 | DOI: 10.36290/vnl.2022.086

Therapy of immunoglonuline IgG4 related disease (IgG4-RD)

Zdeněk Adam¹, Milan Dastych², Aleš Čermák³, Martina Doubková⁴, Šárka Skorkovská⁵, Luděk Pour¹, Zdeněk Řehák⁶, Renata Koukalová⁶, Zuzana Adamová⁷, Martin Štork¹, Marta Krejčí¹, Ivanna Boichuk¹, Zdeněk Král¹

¹ Interní hematologická a onkologická klinika LF MU a FN BRNO

² Interní gastroenterologická klinika LF MU a FN Brno

³ Urologická klinika LF MU a FN Brno

⁴ Klinika nemocí plicních a tuberkulózy LF MU a FN Brno

⁵ Oční klinika LF MU a FN BRNO

⁶ Oddělení nukleární medicíny, Masarykův onkologický ústav Brno

⁷ Oddělení chirurgie nemocnice Frýdek Místek a Chirurgické oddělení Vsetínské nemocnice

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.

Keywords: IgG4-related disease; glucocorticoids; immunosuppressive agents; rituximab, sirolimus, takrolimus, dupilimab, abetacept.

Accepted: September 23, 2022; Published: October 3, 2022  Show citation