|Classification and external resources|
Histopathologic image of focal lymphoid infiltration in the minor salivary gland associated with Sjögren's syndrome. Lip biopsy. H & E stain.
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Sjögren's syndrome (pronounced // SHOH-grinz in English) is a systemic autoimmune disease 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.
Nine out of ten Sjögren's patients are women and the average age of onset is after menopause in women, although Sjögren's occurs in all age groups in both women and men. It is estimated to affect as many as 4 million people in the United States alone, making it the second most common rheumatic disease.
Sjögren's syndrome can exist as a disorder in its own right (primary Sjögren's syndrome) or may develop years after the onset of an associated rheumatic disorder, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, primary biliary cirrhosis etc. (secondary Sjögren's syndrome). A similar syndrome, but without the arthritis symptoms, is sicca syndrome.
The disorder should not be confused with the Sjögren–Larsson syndrome, which was also denoted T. Sjögren syndrome in early studies. It shares clinical features with, and is sometimes concurrent with benign lymphoepithelial lesion or Mikulicz disease. They are, however, considered distinct entities.
Signs and symptoms
The hallmark symptom of Sjögren's syndrome is a generalized dryness, typically including xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes), part of what are known as sicca symptoms. Sicca syndrome also incorporates vaginal dryness, chronic bronchitis and lacks signs of arthritis. 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, peripheral nervous system (distal axonal sensorimotor neuropathy) and brain.
Sjögren's syndrome is associated with increased levels in cerebrospinal fluid (CSF) of IL-1RA, an interleukin 1 antagonist. This suggests that the disease begins with increased activity in the interleukin 1 system, followed by an auto-regulatory up-regulation of IL-1RA to reduce the successful binding of interleukin 1 to its receptors. It is likely that interleukin 1 is the marker for fatigue, however, increased IL-1RA is observed in the CSF and is associated with increased fatigue through cytokine induced sickness behavior. On the other hand, Sjögren's syndrome is characterized by decreased levels of IL-1ra in saliva, which could be responsible for mouth inflammation and dryness. Patients with secondary Sjögren's syndrome also often exhibit signs and symptoms of their primary rheumatic disorders, such as SLE, Rheumatoid Arthritis or Systemic Sclerosis.
Diagnosing Sjögren's syndrome is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms of Sjögren's syndrome and those of other conditions. Nevertheless, the combination of several tests can lead to a diagnosis of Sjögren's syndrome.
Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as anti-nuclear antibody (ANA) and rheumatoid factor (because SS frequently occurs secondary to rheumatoid arthritis), which are associated with autoimmune diseases. Typical Sjögren's syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions but are often present in Sjögren's.
Schirmer's test measures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler. Producing less than five millilitres of liquid is usually indicative of Sjögren's syndrome. However, lacrimal function declines with age or may be impaired by other medical conditions. An alternative test is nonstimulated whole saliva flow collection, in which the patient spits into a test tube every minute for 15 minutes. A resultant collection of less than 1.5 mL is considered a positive result. It takes longer to perform than Schirmer's test, but does not require special equipment.
A slit-lamp examination can reveal dryness on the surface of the eye. Salivary gland function can be tested by collecting saliva and determining the amount produced in a five minute period. A lip biopsy can reveal lymphocytes clustered around salivary glands, and damage to these glands due to inflammation.
Ultrasound examination of the salivary glands is the simplest confirmatory test and has the added advantage of being non-invasive with no complications. The parenchyma of the gland demonstrates multiple, small-2-6 mm hypoechoic lesions which are representations of the lymphocytic infiltrates. Often sialectasis with calculi are demonstrated if the disease is advanced. The sonographic findings have excellent symptom correlation. The other advantage of ultrasound is that complications of the disease such as extra-nodal lymphomas can often be detected as larger 1–4 cm hypoechoic intra-parenchymal masses.
There is also a radiological procedure which is a reliable and accurate test for Sjögren's syndrome. A contrast agent is injected into the parotid duct, which opens from the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth. Widespread puddling of the injected contrast scattered throughout the gland indicates Sjögren's syndrome.
The Revised Classification Criteria for Sjögren's Syndrome requires the presence of signs, symptoms, and lab findings.
Patient-reported symptoms must include both ocular symptoms, such as daily, persistent, troublesome dry eyes for more than three months, and oral symptoms, such as needing to drink water to swallow food.
Objective evidence of eye involvement relies on Schirmer's test and the Rose bengal score (or similar). Histopathology studies should show focal lymphocytic sialadenitis. Objective evidence of salivary gland involvement is tested through ultrasound examinations, the level of unstimulated whole salivary flow, a parotid sialography or salivary scintigraphy. Autoantibodies against Ro (SSA) and/or La (SSB) antigens are also expected.
SS can be excluded from people with past head and neck radiation therapy, Acquired immunodeficiency syndrome (AIDS), pre-existing lymphoma, sarcoidosis, graft-versus-host disease, and use of anticholinergic drugs (since a time shorter than four times the life of the drug).
The pathogenesis of Sjögren's Syndrome is not well understood. The number of factors involved in the progression of the disease makes it difficult to determine its exact origin and cause.
Sjögren's Syndrome was originally proposed to be a specific, self-perpetuating immune system-mediated loss of exocrine glands, specifically acinar and ductal cells. Although this explains the more obvious symptoms, i.e. the lack of salivary and lacrimal fluid, it does not explain the more widespread systemic effects that are seen in the progression of the disease.
The current theory behind the origin of the disease involves a two-hit hypothesis; the first hit is a genetic predisposition to the disease. The second hit occurs upon challenge with a virus. It is suggested that upon presentation of a virus antigen, such as Epstein-Barr virus (EBV), dysfunctional plasmacytoid dendritic cells begin to produce elevated levels of Interferon α (IFN α). This increased level may then incorrectly stimulate auto-antigen myeloid dendritic cells, which would elicit an immune response resulting in the stimulation of autoreactive T cells and the proliferation of autoantibody-producing B cells. This would ultimately result in the destruction of the tissues and glands giving rise to the symptoms associated with Sjögren's Syndrome. The genetic predisposition may involve the formation of dysfunctional plasmacytoid dendritic cells that have been theorized to play a role in the pathogenesis of Sjögren's Syndrome. Normally, a cell presenting an antigen incorrectly would be detected, and directed to undergo apoptosis thus preventing any manifestation of the disease.
There is neither a known cure for Sjögren's syndrome nor a specific treatment to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes. Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface for a longer time. Additionally, cyclosporine (Restasis) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline (Evoxac) and pilocarpine. Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed, and sometimes IVIG (intravenous immunoglobulin). Also, disease-modifying antirheumatic drugs (DMARDs) such as methotrexate may be helpful. Hydroxychloroquine (Plaquenil) is another option and is generally considered safer than methotrexate. Salagen, a man-made form of pilocarpine, can be used to help produce tears as well as saliva in the mouth and intestines. It is derived from the Jaborandi plant.  
In the advanced stage, despite the use of tear replacement drops, the eyes always feel burning, scratchy, sore. The sufferer is always aware of some discomfort; the eyes feel worse in the morning and late evening. The use of tear replacement drops becomes tedious and ineffective. At this point it may be appropriate to consider punctal plugs.
Each eye has two sites at the inner corner of each eyelid where tears drain from the eye. The upper eyelid 'puncta' drains approximately 40% of the tears away and the lower puncta drains away the remaining 60% of the tears. If there is a problem with the quantity of tears, as there is in Sjögren's disease, plugging the lower puncta can result in the tears that the patient has remaining on the eye longer.
Punctal plugs can be inserted into the lower or upper tear drainage canals of the eyes. The procedure takes only a few minutes and is painless. It can be done in the optometrist or ophthalmologist's office. Generally, collagen plugs are inserted first. These plugs will dissolve within a few days, so it gives the patient a chance to see if there is any improvement in comfort. Generally, the improvement is immediate. The patient may proceed to use of permanent plugs, although these too can be removed if necessary.
Preventive dental treatment is also necessary (and often overlooked by the patient), as the lack of saliva associated with xerostomia creates an ideal environment for the proliferation of bacteria that cause dental caries (cavities). Treatments include at-home topical fluoride application to strengthen tooth enamel and frequent teeth cleanings by a dental hygienist. Existing cavities must also be treated, as cavities that extend into the tooth can not be effectively treated through teeth cleaning alone, and are at a high risk of spreading into the pulp of the tooth, leading to the loss of vitality and need for extraction or root canal therapy. This treatment regimen is the same as that used for all xerostomia patients, such as those undergoing head and neck radiation therapy which often damages the salivary glands, as they are more susceptible to radiation than other body tissues.
Unfortunately, many patients, not realizing the need for dental treatment, do not see a dentist until most of their teeth are beyond the point of restoration. It is not uncommon for a dentist to see a xerostomia patient with severe, untreatable cavities in almost every tooth. In severe cases, the only viable treatment may be to extract all of the patient's teeth and treat with prosthetics such as dentures or implants.
Sjögren's can damage vital organs of the body with symptoms that may plateau or worsen, or go into remission as with other autoimmune diseases. Some people may experience only the mild symptoms of dry eyes and mouth, while others have symptoms of severe disease. Many patients can treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life. Some patients can develop renal involvement (autoimmune tubulointerstitial nephritis) leading to proteinuria, urinary concentrating defect and distal renal tubular acidosis.
Patients with Sjögren's syndrome have a higher rate of non-Hodgkin lymphoma compared to both patients with other autoimmune diseases and healthy people. About 5% of patients with Sjögren's syndrome will develop some form of lymphoid malignancy. Patients with severe cases are much more likely to develop lymphomas than patients with mild or moderate cases. The most common lymphomas are salivary extranodal marginal zone B cell lymphomas (MALT lymphomas in the salivary glands) and diffuse large B-cell lymphoma.
Among the complications discussed above, Sjögren's syndrome in women who become pregnant has been linked to increased incidence of neonatal lupus erythematosus with congenital heart block requiring a pacemaker. Type I cryoglobulinemia is a known complication of Sjogren's syndrome. 
The overall prevalence of Sjögren’s Syndrome is estimated to be between 0.2 and 0.65% of the total population. On the basis of prevalence data and population figures, researchers suggest that 0.4 to 3.1 million people in the United States, and 0.5 to 1.4 million people in Australia, suffer from Sjögren’s Syndrome. Few studies have been carried out on the incidence of Sjögren’s Syndrome, but it is estimated to be at 3-6 new cases per 100,000 people per year.
People of any age can be affected by Sjögren’s Syndrome, although prevalence is considerably higher among the elderly. The mean onset of symptoms has been reported to be 53 years but can range from 46 to 60 years, although experts note that up to half of all cases may be left undiagnosed. 71-74 year olds are 7 times more likely to be diagnosed with Sjögren’s Syndrome than 40-44 year olds.
Sjögren’s Syndrome occurs predominately in women, with the female to male ratio more than 9:1. In addition to being female, having a first grade relative with an autoimmune disease and previous pregnancies have been identified as epidemiological risk factors.
There is no differences in the prevalence of Sjögren’s Syndrome due to geographical distribution; it is found in all parts of the world, although regional epidemiology is not a thoroughly researched area. Differences in prevalence due to race and ethnicity are unknown.
Research on diseases such as Sjögren's syndrome focuses on increasing knowledge and understanding of the disorder, improving diagnostic techniques, and finding ways to treat, prevent, and cure the disorder. The United Kingdom Primary Sjögren's Syndrome Registry, a tissue biobank of samples taken for research, supported by the Medical Research Council, UK was established in 2010.
As an autoimmune disease, susceptibility to Sjögren's syndrome is greatly influenced by the human leukocyte antigen. DQA1*05:01, DQB1*02:01, and DRB1*03:01 alleles were identified as risk factors, while DQA1*02:01, DQA1*03:01 and DQB1*05:01 alleles were found to be protective factors for the disease. The relationship between alleles and specific race was also established. HLA-DQ2 and HLA-B8 are generally found in Caucasian patients, while HLA-DR5 is related to Greek and Israeli patients. Multiple genome-wide association scans will be carried out to identify key risk variants.
Viruses that can trigger the immune response of the syndrome include human T-lymphotropic virus type 1 (HTLV-1), Epstein-Barr virus (EBV), human immunodeficiency virus (HIV) and hepatitis C virus (HCV). The UK Primary Sjögren's Syndrome Registry supports clinical trials and genetic studies of Sjögren's syndrome and is open to patients wishing to participate in research studies and researchers studying the disease.
Some research showed that vitamin A and vitamin D are associated with this disease. Vitamin D deficiency was found to be related to neurological manifestations and the presence of lymphoma among patients, while vitamin A levels were inversely associated with extra-glandular manifestations of the disease.
In 2005, an animal model of Sjögren's syndrome was developed by immunizing mice with peptides from 60 kDa Ro-antigen. Days after immunization, the salivary flow decreased. Also, lymphocyte infiltrates and salivary dysfunction were observed which are highly reminiscent of human Sjögren's syndrome.
Saliva is a potential diagnostic tool of Sjögren's syndrome because the salivary component is changed after onset of the disease. With the new miniaturization technology, called ‘lab on a chip’, the diagnosis can be more convenient.
With regard to therapeutics, multiple monoclonal antibodies were under investigation in 2007. The most promising seemed to be the anti-CD20 rituximab and the anti-CD22 epratuzumab, while the anti-TNF-α and IFN-α seemed less effective.
- Benign lymphoepithelial lesion
- Keratoconjunctivitis sicca
- List of cutaneous conditions
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- The original text from this article was obtained from a public domain resource at NIH
- Sjögren's Syndrome Foundation
- Sjogren's Registry
- Sjogren's Syndrome at NHS Choices