Sjogren syndrome is a chronic inflammatory disorder of the body’s exocrine glands in which white blood cells attack the glands and infiltrate them. It is primarily a disease of old age and mainly affects women. The destruction of exocrine glands leads to “sicca” symptoms: dry mouth, dry eyes, and dry skin with parotid gland enlargement. Extra-glandular manifestations also develop as the disease progresses.
The cause of Sjogren syndrome is not very well understood. It can occur on its own (primary disease) or in conjunction with other autoimmune disorders. In both forms, an auto-immune mechanism against exocrine glands develops and destroys these glands causing symptoms of dryness. As the disease progresses, virtually all the organs can get involved. There is some genetic predisposition for this auto-immune mechanism to occur in individuals that destroy their exocrine cells.
Sjogren syndrome can occur in isolation, also called primary Sjogren syndrome or secondary Sjogren syndrome, which occurs with other rheumatic diseases such as systemic lupus erythematosus, rheumatoid arthritis, and scleroderma. In these conditions, the clinical signs overlap, and often, it is difficult to determine if Sjogren syndrome occurred first, leading to these conditions or vice versa. Viral infections such as HIV. Epstein-Barr virus, herpes, etc., act as environmental triggers.
Sjogren syndrome is one of the most common rheumatic diseases after SLE. It occurs worldwide with around 1-2 million cases yearly. It occurs most commonly in the middle age of life during the fourth and fifth decade. The female to male ratio is 9 to 1. Risk factors for Sjogren syndrome include:
Clinical manifestations of Sjogren syndrome include:
Complications of Sjogren syndrome include:
Sjogren syndrome is diagnosed based upon clinical signs. The diagnostic criteria are met with the presence of any three of the following four objectives:
Serologic abnormalities can be detected by:
Differential diagnoses of Sjogren syndrome include:
Currently, no cure for Sjogren syndrome exists, and only symptomatic relief can be provided to manage it.
Patients with primary Sjogren syndrome who do not develop any associated disorder such as SLE or rheumatoid arthritis have a good prognosis. These patients can have a normal life expectancy. Patients with infiltration of exocrine glands with lymphocytic cells can have considerable morbidity. They are at increased risk of developing non-Hodgkin lymphoma. During pregnancy, the complications can worsen that can cause fetal wastage and other disorders.
The exact mechanism by which Sjogren syndrome is caused is unknown, so there are no definitive ways of preventing it. However, once it is diagnosed, patient education is essential for self-care for symptoms such as drying of mouth, skin, eyes, vagina, etc.
Our clinical experts continually monitor the health and medical content posted on CURA4U, and we update our blogs and articles when new information becomes available. Last reviewed by Dr.Saad Zia on May 08, 2023.