*3.1.2 General treatment*

Immunosuppressive drugs such as azathioprine, methotrexate, leflunomide, mycophenolic acid, cyclosporine A (topical, rare oral), and cyclophosphamide effects are used to inhibit general symptoms and organ involvement [26, 27].

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 other severe neurologic manifestations of this disease.

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 life-threatening cases of nervous system involvement and vasculitis.

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

### **3.2 Treatment of primary biliary cholangitis**

#### *3.2.1 Treatment for itching*

Antihistamines, e.g., loratadine or diphenhydramine, are used. Cholestyramine 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 the central nervous system [29].

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

#### *3.3.1 General treatment*

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.

#### *3.3.2 Changes in the style of life*

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.

## **4. Conclusions**

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

**9**

**Author details**

Maria Maślińska

Clinic, Warsaw, Poland

provided the original work is properly cited.

\*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,

National Institute of Geriatrics, Rheumatology and Rehabilitation, Early Arthritis

*Introductory Chapter: Autoimmune Epithelitis - Discussion about Sjögren's Syndrome…*

processes. The impact of environmental, genetic, and epigenetic factors, leading to the epithelial cell damage/apoptosis, may cause a breakdown of epithelium hemostasis and the development of autoimmune diseases, Sjögren's syndrome being its prominent representative. For years pSS was associated with autoimmune epithelial inflammation and referred to as the "autoimmune epithelitis." However, the spectrum of diseases related to the epithelial autoimmunity is wider including, e.g., primary biliary cholangitis. The damaged epithelium is a source of autoantigens, and a persistent immune cell stimulation may lead to the lymphomas associated with the mucosa. Adoption of a wider perspective, combining the clinical experience and scientific knowledge, in an approach to the problem of epithelitis enables making the connection between emerging symptoms and autoimmune diseases, leading to the earlier diagnosis and introduction of proper treatment. Thus the reduction in an activity of the immune process and inhibition of further damage to the epithelium

*DOI: http://dx.doi.org/10.5772/intechopen.86258*

and of loss of its protective properties can be achieved.

*Introductory Chapter: Autoimmune Epithelitis - Discussion about Sjögren's Syndrome… DOI: http://dx.doi.org/10.5772/intechopen.86258*

processes. The impact of environmental, genetic, and epigenetic factors, leading to the epithelial cell damage/apoptosis, may cause a breakdown of epithelium hemostasis and the development of autoimmune diseases, Sjögren's syndrome being its prominent representative. For years pSS was associated with autoimmune epithelial inflammation and referred to as the "autoimmune epithelitis." However, the spectrum of diseases related to the epithelial autoimmunity is wider including, e.g., primary biliary cholangitis. The damaged epithelium is a source of autoantigens, and a persistent immune cell stimulation may lead to the lymphomas associated with the mucosa. Adoption of a wider perspective, combining the clinical experience and scientific knowledge, in an approach to the problem of epithelitis enables making the connection between emerging symptoms and autoimmune diseases, leading to the earlier diagnosis and introduction of proper treatment. Thus the reduction in an activity of the immune process and inhibition of further damage to the epithelium and of loss of its protective properties can be achieved.
