Sjögren's syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It is named after Swedish ophthalmologist Henrik Sjögren (1899-1986), who first described it. Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid arthritis, and it is rheumatoid factor positive in 90 percent of cases. The hallmark symptoms of the disorder are dry mouth and dry eyes (part of what are known as sicca symptoms). In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, and brain. Nine out of ten Sjögren's patients are women and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone making it the second most common autoimmune rheumatic disease.
The primary form, often called sicca syndrome, involves both Keratoconjunctivitis sicca and Xerostomia. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis.
Historical Perspective
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome.
Classification
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to clinical findings.
Pathophysiology
Causes
Common causes of Sjögren's syndrome include viral infection such as Epstein-Barr virus (EBV), Coxsackie virus, Hepatitis C virus, Cytomegalovirus (CMV), Human herpesvirus 6 (HHV-6), Retroviruses and genetic factors.
Differentiating Sjögren's syndrome overview from Other Diseases
Epidemiology and Demographics
The incidence of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The prevalence of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.
Risk Factors
Common risk factors in the development of Sjögren's syndrome include family history of autoimmune diseases, serological markers such as low complement levels and cryoglobulinaemia and parotid gland enlargement.
Screening
There is insufficient evidence to recommend routine screening for Sjögren's syndrome.
Natural History, Complications, and Prognosis
Natural History
The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as ocular and oral dryness.
Complications
Common complications of Sjögren's syndrome include blurred vision and corneal damage, optic neuritis and lymphoma.
Prognosis
Prognosis is generally good and presence of low complement level is associated with a particularly poor prognosis among patients with Sjögren's syndrome.
Diagnosis
Diagnostic Criteria
History and Symptoms
The most common symptoms of Sjögren's syndrome include ocular and oral symptoms. Patients with Sjögren's syndrome may have a positive history of rheumatoid arthritis (RA), systemic lupus erythematous (SLE) and non-Hodgkin B-cell lymphoma.
Physical Examination
Physical examination of patients with is usually remarkable for dryness of all mucous membranes such as mouth, eyes, lips, anal and rectal.
Laboratory Findings
Laboratory findings consistent with the diagnosis of Sjögren's syndrome include Elevated erythrocyte sedimentation rate (ESR), cytopenia, presence of anti-SSA/Ro, anti-SSB/La.
Imaging Findings
Findings on an ultrasound suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous salivary glands, parenchymal inhomogeneity in the submandibular glands and focal or diffuse hypoechoic or anechoic foci in glands. Parotid gland CT scan may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse fat tissue deposition and diffuse punctate calcification.
Other Diagnostic Studies
The most commonly used tests for dry eyes of Sjögren's syndrome include Schirmer test, ocular surface staining and tear break-up time.
Treatment
Medical Therapy
Pharmacologic medical therapies for dry eye, dry mouth, and other sicca symptom of Sjögren's syndrome are Pilocarpine, Cevimeline and artifical tears.