Sjögren’s syndrome versus IgG4-related diseases – classification difficulties and treatment progress

• Main Authors: Anna Nowakowska-Płaza, Jacek Falkowski
• Format: Article
• Language: English
• Published: Termedia Publishing House 2014-09-01
• Series: Rheumatology
• Subjects: B lymphocytes; epithelitis; IgG4-related diseases; rituximab
• Online Access: http://www.termedia.pl/Sjogren-s-syndrome-versus-IgG4-related-diseases-classification-difficulties-and-treatment-progress,18,23336,1,1.html

Sjögren’s syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytic infiltration in exocrine glands mainly salivary and lacrimal which affects impairment of their functions. Some patients develop extraglandular symptoms such as chronic fatigue, arthralgia, or lung, renal, central or peripheral nervous system involvement. Recent decades have brought understanding of some pathogenetic mechanisms and offered new therapeutic options by depleting B cells. Furthermore, the American College of Rheumatology proposed a new set of classification criteria based on objective symptoms. IgG4-related diseases are new nosological entities. The clinical course similarities of SS to Mikulicz’s disease (a subtype of IgG4-related disease) result in diagnostic difficulties. Typical conditions of them are: an increased IgG4 level and infiltrations of parenchymal organs by plasmatic cells. This review summarizes classification difficulties, pathogenesis and treatment strategies of SS and IgG4-related diseases.