Lynn M. Petruzzi and Frederick B. Vivino
AORN Journal, Denver, Colorado (March, 2003)

Sjögren's Syndrome (SS), also known as Sicca Syndrome, is a chronic autoimmune disorder in which lymphocytes invade and destroy the exocrine glands, particularly the salivary and lacrimal glands, resulting in decreased saliva and tear production.

Dryness also may affect the skin, sinuses, upper airway, gastrointestinal tract, and vaginal tissues. Systemic manifestations of SS include rash, Raynaud's Phenomenon, fatigue, and nerve and muscle pain.

Sjögren's Syndrome is the second most-common autoimmune rheumatic disease, surpassed only by rheumatoid arthritis, and occurs even more frequently than systemic lupus erythematosus.

Estimates of the prevalence of SS range from 500,000 to four million people, 90% of whom are women. The typical patient is a Caucasian perimenopausal woman in her forties.

Sjögren's Syndrome frequently is unrecognized and untreated. The average time from onset of symptoms to diagnosis is at least three and one-half years.

The person often seeks the help of multiple physicians and specialists, only to repeatedly receive an incorrect diagnosis or no diagnosis at all. The symptoms continue or worsen, leading to anger and frustration.

Delays in diagnosis can be explained by the insidious development of symptoms during a number of years, the lack of universally accepted diagnostic criteria, and the tendency of patients and medical personnel to trivialize the initial symptoms of the disorder.

ETIOLOGY AND PATHOGENESIS

The trigger that initiates the autoimmune events of SS remains unknown.

A variety of factors potentially may influence the development of the disease.

Most research indicates that SS and other autoimmune diseases result from the interaction of specific, susceptible genes and environmental agents that fool the immune system into attacking a target organ. In SS, the exocrine or moisture producing glands are attacked.

No single gene causes SS. However, the human leukocyte antigen 5 HLADQZ,DR3 occurs most frequently in Caucasian patients with SS.

Apoptosis (i.e., programmed cell death) of T lymphocytes and salivary acinar epithelial cells is a gene-regulated process that functions abnormally in patients with SS and appears to contribute to glandular destruction.

Indirect evidence suggests that viruses may play a role as the environmental agent. Hormonal changes also may serve as an agent that influences the disease.

An injury to the exocrine gland may initiate SS. As T lymphocytes invade the tissue, cytokines (i.e., inflammatory messengers) are released locally, which perpetuates the immune inflammatory response.

The cytokines may escape into the bloodstream, stimulating other parts of the body to make proteins that result in an increased erythrocyte sedimentation rate (ESR) and increased production of Creactive protein (CRP). T lymphocytes also stimulate B cells, causing antibody formation (e.g., immunoglobulin G [IgG], immunoglobulin A [IgA], immunoglobulin M [IgM], antinuclear antibody [ANA], Sjögren's Syndrome antigen A [SSA], Sjögren's Syndrome antigen B [SSB]).

Eventually the normal glandular tissue is replaced by fibrosed and fatty tissue. The autonomic nerves that send signals to the moisture producing glands also may be damaged.