PRIMARY SJOGREN SYNDROME

Primary Sjogren Syndrome

Primary Sjogren Syndrome

Introduction

Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disorder that occurs primarily in perimenopausal women. It is characterized by lymphocytic infiltration and destruction of exocrine glands (e.g., lacrimal and salivary glands). The spectrum of disease ranges from “sicca syndrome” (dry eyes [keratoconjunctivitis sicca] and dry mouth [xerostomia]) to systemic involvement, with manifestations including, vasculity, nephritis, arthritis, neuropathy, and interstitial lungs involvement. Primary Sjogren's syndrome has involvement of the exocrine glands with or without systemic involvement. This means that the primary form can also cause muscle and joint pain. The joint changes here are not erosive (Garcia, Nascimento, Bonfa, 1994, p.718).

Primary Sjogren Syndrome

The pathogenesis of development of the PZS lists a number of immunological processes, which are involved in a number of immune cells. Among the important role played by T lymphocytes it is especially in the local inflammatory process. In addition immune cells are important in the pathogenesis of the development of diaper dermatitis attributed to cytokines, chemotactic factors, adhesion and apoptosis (Fritzler, 1995, p.218).

HLA Alleles in Primary Sjogren Syndrome

The protein determined by HLA is protein disposed on the outside of the cells and is unique to the individual. Theimmune system uses HLAs to distinguish the cells themselves from those of non-self. Any cell showing the type of HLA that person belongs to that person (that is to say, is not attacker). Several cells with a different HLA type is "non-self" and will be identified as invaders. HLA kinds are innate some of them are related to autoimmune disease among others. Scientist believe that individuals who have definite kinds of HLA antigen seem predisposed to certain autoimmune disease, for example lupus erythematosus, the myasthenia acquired, the Sjögren's syndrome and multiple sclerosis (Barakat, Torterotot , 1992, p.89).

When there is pathogen entrance in the body cells it is known as antigen present cells APC overcome the pathogen during the procedure of phagocytosis. Protein derived from this pathogen is assimilated into small piece and laden onto HLA's antigen (especially MHC class II). Then they are presented by APC to T cell, which produce reactions to reduce the pathogen.

Via a related progression of protein (inhabitant and distant, for instance those of viruses) generated within most cells are also presented to HLA antigen (particularly MHC class I) on the cell's surface. Contaminated cell could be familiar and damaged by immune system component.

HLA acronym uses in conjunction with the letter and numbers to designate an allele at a specific locus given HLA: HLA-DQA1 and designates the A1 allele of the HLA-DQ. HLA genes are part of major histocompatibility complex. Someloci are studied, including HLA-A, HLA-B or HLA-C (belonging to the MHC class I) and HLA-DP, HLA-DQ and HLA-DR (belonging to the MHC class II) (Clark, Tomasi, 1975, p.117). Immunogenetic studies also showed a higher frequency of histocompatibility antigens HLA-B8-DR3 DRw52 c in patients with Sjogren's syndrome than the general population. Molecular analysis of HLA class II genes showed that in patients with Sjogren's syndrome is a greater ...