Sjögren syndrome
Sjögren's syndrome | |
---|---|
Other names | Sjögren's syndrome, sicca syndrome |
Tissue biopsy, blood tests[2] | |
Differential diagnosis | Medication side effect, anxiety, sarcoidosis, amyloidosis[5] |
Treatment | Artificial tears, medications to reduce inflammation, surgery[4] |
Prognosis | Normal life expectancy[6] |
Frequency | ~0.7%[7] |
Sjögren syndrome or Sjögren's syndrome (SjS, SS) is a
Symptoms
Overview and importance to patients
In a 2021 poll of Sjogren's patients, a majority of respondents stated that eight Sjogren's symptoms had a major or moderate impact on their life: fatigue (79%); dry eyes (75%); dry mouth (73%); joint pain (65%); trouble sleeping (64%); eye discomfort (60%); muscle pain (56%); and brain fog (54%).[9][10][11]
Symptoms
Primary symptoms are dryness (
The hallmark symptom of Sjögren syndrome is
In some people with SS, skin dryness may be the result of
Sjögren's syndrome can damage
Complications
Among the complications discussed above, women with anti-Ro/SS-A and anti-La/SS-B antibodies who become pregnant have an increased rate of
Sjögren's syndrome can affect such organs as the liver, pancreas, kidneys, lungs, and central nervous system.[20]
Associated conditions
Sjögren's syndrome is associated with a number of other medical conditions, many of which are
Sjogren's is the second most common cause of dysautonomia.[25][26][27][28]
Causes
While the exact cause is unclear, it is believed to involve a combination of
The cause of Sjögren's syndrome is unknown, but it may be the influence of a combination of genetic, environmental, and other factors, as is the case with many other autoimmune disorders.[29] Around 20 autoantibodies could be involved.[30]
Genetics
The observation of high rates of autoimmune disorders in families with a history of Sjögren's syndrome is linked with a genetic predisposition to the syndrome.[31] Studies on the polymorphisms of human leukocyte antigen (HLA)-DR and HLA-DQ gene regions in Sjögren's patients show differential susceptibility to the syndrome as the result of different types of the resulting autoantibody production.[31]
Hormones
Since Sjögren's syndrome is associated with a high prevalence in women,
Microchimerism
Environment
Pathogenesis
The
Sjögren's syndrome was originally proposed as a specific,
In the presence of a susceptible
Sjögren's syndrome is associated with increased levels in
Genetic predisposition
The
Patients of different ethnic origin carry different HLA-susceptibility
Beyond genetics,
Environmental triggers
Environmental factors, such as glandular
Inflammation
Epithelial cells in Sjögren's syndrome lesions are active participants in the induction and perpetuation of the inflammatory process. Environmental and hormonal factors, in concert with an appropriate genetic background, are believed to trigger Sjögren's syndrome, which
Programmed cell death
Dysregulation of
Hormonal factors
Diagnosis
While Sjögren's syndrome[50] is one of the most common auto-immune diseases, it has no specific and non-invasive diagnostic tests.
Diagnosing Sjögren's syndrome (SS) is complicated by the range of symptoms that a patient may manifest, and the similarity between symptoms of Sjögren's syndrome and those of other conditions. Also, patients with SS symptoms approach different
Tests
The combination of several tests, which can be done in a series, can eventually diagnose Sjögren's syndrome.[33][51]
Blood tests
Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as
Rose bengal test
The
Schirmer test
Use of Schirmer strips to test oral dryness are possible.[54][55]
Saliva flow tests
Symptoms of dry mouth and dryness in the oral cavity are caused by the reduced production of saliva from the salivary glands (parotid gland, submandibular gland, and sublingual gland). In unstimulated whole saliva flow collection, the person spits into a test tube every minute for approximately 15 minutes. A resultant collection of less than 1.5 ml (0.053 imp fl oz; 0.051 US fl oz) is considered a positive result.[56] [51] In a stimulated saliva flow test the person sucks on a sugar free sweet, whilst collecting saliva. An unstimulated salivary flow rate of 0.1 to 0.2 ml/min and a stimulated flow rate of 0.7 ml/min or less is considered to be abnormally low flow rates indicative of salivary gland hypofunction.[57]
Unstimulated saliva production reduces by 40 to 70% between the age of 20 and 80 years, but stimulated saliva production is not affected.[58]
Lip biopsy
A lip/salivary gland biopsy takes a tissue sample that can reveal lymphocytes clustered around salivary glands, and damage to these glands from inflammation. This test involves removing a sample of tissue from a person's inner lip/salivary gland and examining it under a microscope. On such biopsies, the single most important test result in the diagnosis of the oral component of Sjögren syndrome is likely the focus score, which is the number of mononuclear cell infiltrates containing at least 50 inflammatory cells in a 4 mm2 glandular section.[59] The Chisholm-Mason grades are also widely used for salivary gland biopsies (see table).[60]
Ultrasound
Salivary gland ultrasonography is not invasive, and may help reduce unnecessary biopsies in anti-SSA-negative patients. [61][62][63][64]
Other tests
A radiological procedure is available as a reliable and accurate test for Sjögren's syndrome, in the form of a
For Sjögren's syndrome, sudomotor function through electrochemical skin conductance may help in the diagnosis process.[65][66]
Autoimmune comorbidity
People with Sjögren's may also have other autoimmune conditions.[67][68][69]
Exclusions
Sjögren's syndrome may be excluded in people with past head and neck
Prevention
No prevention mechanism exists for Sjögren's syndrome (SS) because of its complexity as an autoimmune disorder.
However
Diet is strongly associated with the inflammation seen in many autoimmune related diseases, including SS. An experimental study concluded that SS patients often show high sensitivity to gluten that directly relates to inflammation.[70]
Moderate exercise is also helpful in SS patients, mainly reducing the effect of lung inflammation.[71][citation needed]
Treatment
Overview
Treatment is directed at managing the person's
Treatments
Neither a cure nor a specific treatment for Sjögren's syndrome is known to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive.[72][73]
Eye care
Moisture replacement therapies such as
Additionally,
Vaginal dryness
In women with Sjögren's syndrome,
Musculoskeletal
Systemic
For systemic symptoms, including fatigue, joint pain, myositis and
Dental care
Fatigue
A small study showed possible efficacy of vagus nerve stimulation for Sjogren's fatigue reduction.[78]
Prognosis
Non-Hodgkin lymphoma
Results from a number of studies indicate that, compared to other autoimmune diseases, Sjögren's syndrome is associated with a notably high incidence of
Lymphomagenesis in primary Sjögren's syndrome patients is considered as a multistep process, with the first step being chronic stimulation of autoimmune B cells, especially B cells that produce
Other organs
Apart from the notably higher incidence of malignant NHL, Sjögren's patients show only modest or clinically insignificant deterioration in specific organ-related function.
Burden of illness
Sjögren's syndrome is associated with a high burden of illness,
Mortality
Published studies on the survival of Sjögren's syndrome patients have been limited in varied respects, perhaps owing to the relatively small sample sizes, and the fact that secondary Sjögren's syndrome is associated with other autoimmune diseases. A 2010 study found a slight increase in mortality rates of Sjögren's patients in comparison with the remainder of the population.[31] A 2016 study found that primary Sjögren's was not associated with an increase in all-cause mortality as compared with the general population, but that a subset of patients with extraglandular involvement, vasculitis, hypocomplementaemia and cryoglobulinaemia may be at increased risk of mortality.[90] A 2021 metaanalysis showed a 46% increase in mortality, with significantly greater mortality risk in patients with older age, male gender, vasculitis, interstitial lung disease, low complements, positive anti-La/SSB and cryoglobulinaemia.[91]
Among those without other autoimmune disorders, life expectancy is unchanged.[6]
Epidemiology
Sjögren's syndrome (SS) is the third-most common rheumatic autoimmune disorder, behind rheumatoid arthritis and
There are no geographical differences in the rates of SS.[92] Sjögren's syndrome has been reported in all areas of the world, although regional rates have not been well studied.[92][93]
Depending on the criteria for determining prevalence, studies estimate the prevalence of SS at between 500,000 and two million people in the United States. Broader studies of SS prevalence range widely, with some reports of up to a prevalence of 3% of the population.[16] A few studies have reported that the incidence of the syndrome varies between three and six per 100,000 per year.[16][94] Between 0.2 and 1.2% of the population is affected, with half having the primary form and half the secondary form.[7] It is around 10 times more common in women than in men.[3] Though the disease commonly begins in middle age, people of any age can be affected.[2][3]
Nine out of 10 SS patients are women.[29][93] In addition to prevalence in women, having a first-degree relative with an autoimmune disease and previous pregnancies have been identified as epidemiological risk factors.[95] Despite the lower risk for men, primary SS in men tends to represent a more severe form of the disease.[96] The role of race and ethnicity in the prevalence of the disease is unknown.[citation needed]
Although Sjögren's syndrome occurs in all age groups, the average age of onset is between ages 40 and 60, although as many as half of all cases may be left undiagnosed or unreported.[29][16][97][98] The prevalence of SS generally increases with age.[16]
Sjögren's syndrome is reported in 30-50% of people with rheumatoid arthritis and in 10-25% with systemic lupus erythematosus.[29]
History
Overview
The disease was described in 1933 by Henrik Sjögren, after whom it is named, but a number of earlier descriptions of people with the symptoms exist.[3]
History
In 1930,
After extensive research and data collection, Sjögren published an essential paper in 1951, describing 80 patients with
Research
Research into multifactorial autoimmune diseases such as SS focuses on expanding the knowledge surrounding the disorder, improving diagnostic tools and finding ways to prevent, manage and cure the disorder. The
As with other autoimmune diseases, susceptibility to Sjögren's syndrome is greatly influenced by the human leukocyte antigen.
Some research has shown that a paucity of vitamin A and vitamin D are associated with the disease.[104] Vitamin D deficiency was found to be related to neurological manifestations and the presence of lymphoma among patients, but vitamin A levels were inversely associated with extraglandular manifestations of the disease.[104]
Saliva is a potential diagnostic tool for Sjögren's syndrome because the salivary component is changed after onset of the disease.
With regard to therapeutics, multiple
In 2014, the Sjögren's Syndrome Foundation announced a five-year goal to halve the disease's average time to diagnosis.[109]
Notable cases
- Shannon Boxx (U.S. Olympic soccer player) has both Sjögren's syndrome and lupus.[110]
- Carrie Ann Inaba (singer-actress) is the national awareness ambassador and spokesperson for the Sjögren's Syndrome Foundation.[111]
- fatigue for years.[112]
- Stephen McPhail (professional soccer player for Ireland, Leeds and Cardiff City) was diagnosed with lymphoma and Sjögren's syndrome at age 29.[113]
- Postural Orthostatic Tachycardia Syndrome[114]
- Slađana Milošević (Serbian singer), died after struggling with Sjögren's syndrome. [115]
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Further reading
- Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, Granata G, Ng WF, Fisher BA, Bombardieri M, Astorri E, Empson B, Larkin G, Crampton B, Bowman SJ, British Society for Rheumatology Standards, Guideline and Audit Working Group (1 October 2017). "The British Society for Rheumatology guideline for the management of adults with primary Sjögren's Syndrome". Rheumatology. 56 (10): e24–e48. PMID 28957550.
External links
- Sjögren syndrome at NHS Choices
- Sjögren syndrome – US National Institute of Arthritis and Musculoskeletal and Skin Diseases
- US Sjögren's Foundation