**Conflict of interest**

*Chronic Autoimmune Epithelitis - Sjogren's Syndrome and Other Autoimmune Diseases...*

70% as compared to the group treated with glucocorticoid monotherapy.

confirmation of indications by vascular surgeons.

disease or not responding to basic treatment [92].

CD19+ on B lymphocytes, were not published.

*10 months of treatment (C, D) with prednison 0.6 mg/kg/day.*

In case vascular location and lesions in the aorta, there are no explicit guidelines concerning treatment. Similarly as in other forms of IgG4-RD, steroids are used, most commonly prednisone at a dose of 0.6 mg/kg daily. This dose is gradually reduced to the maintenance dose of 5 mg/kg daily. There are no guidelines concerning therapy duration. Steroid administration does not protect against development and progression of aneurysms in patients in whom vascular wall widening was found initially [9, 80]. In this group of patients, surgery is performed in case of

Due to participation of T and B lymphocytes in the pathomechanism of the disease, the first biological drug which turned out to be effective in the treatment of IgG4-RD was rituximab (RTX). Typically this drug is used intravenously at a dose of 1 g every 15 days, up to a dose of 2 g [91]. In a prospective open-label clinical trial, RTX was effective in 97% of patients with IgG4-RD after 6 months of therapy despite no glucocorticoids used [80]. Also, the efficacy of RTX in patients with involvement of cerebrospinal meninges was reported on [92]. Currently RTX is recommended by experts for use as a second-line therapy in patients with recurrent

So far, the data from a clinical trial with XmAB5871, i.e., a reversible inhibitor of

The patients with IgG4-RD are shown in **Figure 6**, before and after treatment.

*Typical Mikulicz disease (IgG4-related disease) with lacrimal enlargement at the diagnosis (A, B) and after* 

recurrences. In 46% of patients with IgG4-RD who have their glucocorticoid doses decreased, the disease recurs [89]. As in other rheumatic diseases, the disease modifying antirheumatic drugs are tried in such cases. The adjunctive therapy has so far applied most drugs used in rheumatology, including methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide. However, the results and their efficacy are different. Adjunctive treatment with disease modifying antirheumatic drugs is currently based mainly on the experience of attending physicians and is experimental. At present, there are no well-designed clinical trials summarizing this problem. Moreover, the disease modifying antirheumatic drugs were always used as adjunctive treatment to glucocorticoid therapy and not as first-line therapy, therefore their efficacy is even more difficult to evaluate. In 2017, a summary of observations in patients treated with glucocorticoids vs. glucocorticoids and cyclophosphamide orally was published [90]. It turned out that a combination of disease modifying antirheumatic drugs decreased the risk of disease recurrence by

**84**

**Figure 6.**

None declare.
