**Author details**

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

other severe neurologic manifestations of this disease.

**3.2 Treatment of primary biliary cholangitis**

**3.3 Treatment for dry eyes and mouth as in pSS**

*3.2.1 Treatment for itching*

*3.3.1 General treatment*

the central nervous system [29].

*3.3.2 Changes in the style of life*

**4. Conclusions**

Among biological drugs, rituximab (anti-CD20 monoclonal antibody) has been showing positive results in current clinical trials and is used to inhibit certain aspects of the disease. Rituximab improves saliva flow rate and lacrimal gland function (discussed), diminishes fatigue and malaise, and is recommended in case of cryoglobulinemia or vasculitis-related peripheral nervous system involvement or

Glucocorticosteroids (GCS) are used in immunosuppressive therapy combined with other immunosuppressive drugs. Pulses of GCS are used in the case of the intensification of organ changes, vasculitis, and nervous system involvement.

Intravenous immunoglobulin administration and plasma exchanges are used in

Antihistamines, e.g., loratadine or diphenhydramine, are used. Cholestyramine

Ursodeoxycholic acid (UDCA) is the main medication used in biliary cholangitis. A complementary therapy with obeticholic acid was introduced in 2016, as second-line treatment. If the UDCA treatment is ineffective, the use of fibrates (e.g., bezafibrate) in combination therapy (UDCA plus fibrate) is also considered; ongoing clinical trials have yielded encouraging results [30]. Of the immunosuppressants, the use of methotrexate (MTX), as a drug which may affect pruritus score, serum level of alkaline phosphatase, or IgM level, is discussed [31], although there were observations that MTX could increase mortality in this group of patients [32]. There were clinical trials with rituximab [33] and with ustekinumab [34], but at the present time, they have not produced positive results to the expected extent. The liver transplant aims at prolonging the patient's life, but it is reported that up to 29% of patients develop a relapse of the disease in the transplanted organ [35].

Therefore, the use of UDCA after transplantation is still recommended.

PBC is a chronic autoimmune liver disease in which a lifestyle is particularly important. Reducing the intake of foods with high sodium content, avoiding alcohol, as well as being careful with new medications or dietary supplements are extremely important. Physical exercise is recommended to reduce risk of bone loss and muscle weakness.

The epithelium is an important element of the human body due to its protective, secretory, and transporting functions. It is also the target for the immunological

as the addition to beverages and foods may be used. Rifampicin, an antibiotic which may act as a medicine against itching, may also be administered [28]. Opioid antagonists containing naloxone or naltrexone inhibit pruritus by their effects on

**Other:** Vitamin D supplementation and aerobic exercises are recommended.

life-threatening cases of nervous system involvement and vasculitis.

**8**

Maria Maślińska National Institute of Geriatrics, Rheumatology and Rehabilitation, Early Arthritis Clinic, Warsaw, Poland

\*Address all correspondence to: maslinskam@gmail.com

© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
