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Sjögren Syndrome is a rare autoimmune disorder that affects the exocrine glands, especially the salivary and lacrimal glands. It is named after Swedish ophthalmologist Henrik Sjögren, who first described the condition in 1933.

The main features of Sjögren Syndrome include dryness of the mouth and eyes, as well as other systemic symptoms that can occur in some patients, such as fatigue, joint pain, and dry skin. This condition can occur on its own or in association with another autoimmune disease, such as rheumatoid arthritis or lupus.

Research studies and clinical trials are continually developing to better understand the causes and inheritance of Sjögren Syndrome. There are genetic factors involved, as suggested by the increased frequency of the condition in certain families. However, the exact genes involved and their role in the development of the disease are still being studied.

For more information on Sjögren Syndrome, including patient resources, advocacy groups, and clinical trials, you can visit websites such as clinicaltrialsgov, OMIM, PubMed, and the Sjögren’s Syndrome Foundation. These resources provide additional articles, studies, and support for patients and healthcare professionals interested in learning more about this rare condition.

Frequency

As an autoimmune condition, Sjögren syndrome occurs more frequently in women than in men. It is estimated that the prevalence of Sjögren syndrome is around 0.1% to 0.6% in the general population.

Studies have shown that this syndrome can occur alone or in association with other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. In fact, up to half of the patients with Sjögren syndrome may have an additional autoimmune disorder.

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Research has also suggested a genetic component to Sjögren syndrome, as certain genes have been identified to be associated with an increased risk of developing this condition. Some of these genes include HLA-DR3, HLA-DR2, and HLA-B8.

Several resources are available for additional information on the frequency of Sjögren syndrome. A comprehensive database called OMIM provides genetic information about the condition and its inheritance patterns. PubMed articles can also be found to explore the latest research on the causes, features, and frequency of Sjögren syndrome. Furthermore, clinicaltrials.gov offers information on ongoing clinical trials related to this condition.

While Sjögren syndrome is considered a relatively rare disease, it is important to note that it can have significant impacts on patient health. The disease can affect not only the tear and salivary glands but also other tissues and organs in the body. Extraglandular features, such as skin and systemic involvement, are observed in some patients with Sjögren syndrome.

Patient advocacy organizations, such as the Sjögren’s Syndrome Foundation, provide support, resources, and information for individuals living with this condition. The foundation also raises awareness about Sjögren syndrome and advocates for research and improved care for patients.

Overall, understanding the frequency of Sjögren syndrome and its associated features is crucial for healthcare providers, researchers, and patients to better manage this complex autoimmune disorder.

Causes

Sjögren syndrome is a complex autoimmune disease characterized by lymphocytic infiltration of exocrine glands, resulting in dryness of the eyes and mouth. It can occur alone (primary Sjögren syndrome) or in association with other systemic diseases (secondary Sjögren syndrome).

The exact causes of Sjögren syndrome are not fully understood, but there are several factors that may contribute to its development:

Genetic Factors

Sjögren syndrome has been found to have a genetic component. Certain genes have been associated with an increased risk of developing the condition, including HLA-DRB1, HLA-DQA1, and HLA-DQB1. However, it is important to note that not everyone with these genes will develop Sjögren syndrome.

Environmental Triggers

Environmental factors, such as viral infections, may trigger the development of Sjögren syndrome in genetically susceptible individuals. The exact viruses involved are not yet known, but studies have shown associations with Epstein-Barr virus (EBV) and cytomegalovirus (CMV).

Immune Dysfunction

Abnormal immune responses are thought to play a role in the development of Sjögren syndrome. The immune system mistakenly attacks the body’s own tissues, especially the exocrine glands, which produce tears and saliva. This results in the dryness of the eyes and mouth that are characteristic of the condition.

Extraglandular Features

Sjögren syndrome can also affect other organs and tissues in the body, called extraglandular features. These can include skin, joints, kidneys, lungs, and nerves. The exact mechanisms by which these extra-glandular features occur are not well understood.

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Hormonal Factors

Sjögren syndrome is more common in women than men, suggesting a potential hormonal influence. Hormonal changes, such as those occurring during menopause, may contribute to the development or worsening of symptoms in some women.

Lack of Resources

Sjögren syndrome is considered a rare disease, and research funding and resources are limited compared to other more common conditions. This lack of resources makes it more challenging to advance scientific understanding and develop effective treatments.

Advocacy and Support

Despite the challenges, there are advocacy groups and support communities dedicated to raising awareness about Sjögren syndrome and supporting patients. These organizations provide valuable information, resources, and support for patients and their families.

References:

  • Paiva, E. S., Lopes, J. B., & Pereira, R. M. (2015). Sjögren’s syndrome. Best Practice & Research Clinical Rheumatology, 29(4-5), 586-605. doi: 10.1016/j.berh.2015.08.003
  • Sjögren Syndrome. (2019). Retrieved from OMIM (Online Mendelian Inheritance in Man) database: https://www.omim.org/entry/270150
  • Sjögren’s Syndrome. (2021). Retrieved from National Center for Advancing Translational Sciences Genetic and Rare Diseases Information Center: https://rarediseases.info.nih.gov/diseases/10747/sjogrens-syndrome
  • Sjögren Syndrome. (n.d.). In: ClinicalTrials.gov. Retrieved from https://clinicaltrials.gov/ct2/results?cond=Sjogren+Syndrome&term=&cntry=&state=&city=&dist=

Inheritance

The inheritance of Sjögren syndrome has not been fully understood due to its complex nature. It is believed to be a multifactorial condition, meaning that both genetic and environmental factors contribute to its development.

Sjögren syndrome can occur sporadically, meaning it is not inherited and arises by chance in a patient with no family history of the condition. However, there is evidence to support a genetic component in some cases.

Researchers have identified several genes that may play a role in increasing the susceptibility to developing Sjögren syndrome. These genes are involved in regulating the immune system and the production of saliva and tears, which are important tissues affected in this condition.

Studies have shown that Sjögren syndrome is more common in women than in men, suggesting that hormonal factors may also contribute to its development.

It is important to note that the inheritance pattern of Sjögren syndrome is complex and can vary among individuals and families. It is recommended that patients and their families consult with a genetic counselor or healthcare professional for more specific information on the inheritance pattern in their particular case.

For additional information on the inheritance of Sjögren syndrome, references such as OMIM (Online Mendelian Inheritance in Man) and PubMed can be consulted. These resources provide scientific articles and other references on the topic.

Other Names for This Condition

Sjögren syndrome is known by several other names, including:

  • Sjogrens syndrome
  • Sicca syndrome
  • SS

These alternate names reflect the various aspects of the condition and its clinical features.

Additionally, there are other disorders that can occur along with Sjögren syndrome. These are known as extraglandular features and can involve various tissues and organs in the body. Some of the additional disorders that can develop in people with Sjögren syndrome include:

  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Scleroderma
  • Vasculitis

Research has shown that genetic factors may contribute to the development of Sjögren syndrome. Certain genes have been identified that are associated with an increased risk of developing the condition. However, it is important to note that the inheritance pattern for Sjögren syndrome is complex and not fully understood.

For more information on the causes and inheritance of Sjögren syndrome, you can refer to the following resources:

  • The OMIM (Online Mendelian Inheritance in Man) catalog – a comprehensive database of human genes and genetic disorders.
  • Articles available on PubMed – a database of scientific articles in the field of medicine and health.

In addition to the genetic factors, there are other potential causes of Sjögren syndrome. It is more common in women than in men, suggesting that hormonal and immune system differences may play a role in its development. Environmental factors and infections have also been proposed as possible triggers for the condition, but more research is needed to fully understand these factors.

Rare forms of Sjögren syndrome have been reported, characterized by specific features and involvement of different organs or systems in the body. These rare variants may present with distinct clinical features and require specific management approaches.

For support, advocacy, and additional information on Sjögren syndrome, you can refer to the following resources:

  • The Sjögren’s Syndrome Foundation – provides support, education, and research resources for patients and healthcare professionals.
  • ClinicalTrials.gov – a database of ongoing clinical studies related to Sjögren syndrome and other medical conditions.

Overall, Sjögren syndrome is a complex condition that can affect various tissues and systems in the body, and its frequency and features can vary among individuals. Further scientific studies and research are needed to fully understand the underlying mechanisms and develop targeted treatments.

Additional Information Resources

Here are some additional resources for more information on sjogrens syndrome:

  • Sjogrens Syndrome Foundation: The Sjogrens Syndrome Foundation is a non-profit organization dedicated to providing support, advocacy, and education to patients with sjogrens syndrome. Their website, sjogrens.org, offers information on the condition, its features, diagnosis, treatment, and more.
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS): The NIAMS is part of the National Institutes of Health (NIH) and provides comprehensive scientific and research-based information on sjogrens syndrome and other autoimmune diseases. Their website, niams.nih.gov, features articles, studies, and resources on the condition.
  • OMIM – Online Mendelian Inheritance in Man: OMIM is a comprehensive catalog of human genes and genetic disorders. Their database includes information on sjogrens syndrome, including the genes associated with the condition and their inheritance patterns. It is available at omim.org.
  • PubMed: PubMed is a free online database of scientific articles. Searching for “sjogrens syndrome” on pubmed.org will provide you with the latest research and studies on the condition.
  • ClinicalTrials.gov: ClinicalTrials.gov is a database of clinical trials conducted around the world. Searching for “sjogrens syndrome” on clinicaltrialsgov will give you a list of current and upcoming clinical trials related to the condition. It is available at clinicaltrialsgov.
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These resources offer a wealth of information on sjogrens syndrome, its causes, clinical features, systemic involvement, extraglandular manifestations, and other related disorders. They also provide support and research opportunities for patients and healthcare professionals.

Genetic and Rare Diseases Information Center

The Genetic and Rare Diseases Information Center (GARD) is a valuable resource for patients, families, and healthcare providers seeking information on rare genetic disorders. This includes conditions such as Sjögren’s syndrome, which is a rare autoimmune disorder that affects the body’s moisture-producing glands.

Sjögren’s syndrome can occur as a primary condition or secondary to another autoimmune disorder, such as rheumatoid arthritis or lupus. It primarily affects women, with increased frequency in older adults. The exact causes of Sjögren’s syndrome are not yet fully understood, but it is believed to have a genetic component.

GARD provides a range of resources for patients and families affected by Sjögren’s syndrome. These include scientific articles and studies, information on clinical trials available for this condition, and links to references such as PubMed and OMIM. GARD also features a catalog of genetic disorders and provides information on inheritance patterns and the genes involved in these disorders.

In addition to the genetic aspects, GARD also provides information on the clinical features of Sjögren’s syndrome, including its extraglandular manifestations. These can involve the skin, respiratory system, gastrointestinal system, and other tissues. GARD offers support and advocacy resources, connecting patients and families with organizations that can provide additional information and support.

For healthcare providers and researchers, GARD offers information on developing research and studies related to Sjögren’s syndrome. This includes information on ongoing clinical trials listed on ClinicalTrials.gov, as well as scientific articles and research studies that provide further insights into the condition and its management.

Overall, GARD serves as a comprehensive resource for information on Sjögren’s syndrome and other rare genetic disorders. It provides valuable support for patients and their families, as well as healthcare providers and researchers looking to better understand and manage these conditions.

Patient Support and Advocacy Resources

The following resources provide support and advocacy for patients with Sjögren syndrome:

  • Sjögren’s Syndrome Foundation – The Sjögren’s Syndrome Foundation is a patient support and advocacy organization that provides educational resources, support groups, and information on research and clinical trials for Sjögren syndrome.
  • National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD) – GARD provides information on the genetics, causes, and inheritance patterns of rare diseases, including Sjögren syndrome. It also provides additional resources and references for further information.
  • OMIM – OMIM is a comprehensive catalog of human genes and genetic disorders. It provides detailed information on the genes and genetic variants associated with Sjögren syndrome and other related disorders.
  • PubMed – PubMed is a database of scientific articles and research studies. It provides access to a wide range of scientific literature on Sjögren syndrome, including studies on its clinical features, genetic basis, and treatment options.
  • ClinicalTrials.gov – ClinicalTrials.gov is a registry of clinical trials for various medical conditions, including Sjögren syndrome. It provides information on ongoing and upcoming clinical trials that patients can participate in.
  • Skin Diseases – Sjögren syndrome can cause skin problems, and the American Academy of Dermatology provides information and resources on skin diseases associated with Sjögren syndrome.

Research Studies from ClinicalTrialsgov

Research studies are being conducted on Sjögren’s syndrome, a condition that primarily affects women and occurs when the immune system mistakenly attacks the glands that produce tears and saliva. These studies aim to gain a better understanding of the causes, inheritance patterns, and genetic factors associated with Sjögren’s syndrome, as well as to develop new treatments and resources for patients.

Increased Frequency and Causes of Sjögren’s Syndrome

One research study from ClinicalTrialsgov is investigating the increased frequency of Sjögren’s syndrome and its possible causes. The study aims to identify genetic factors that may contribute to the development of the condition. By analyzing the genetic information from patients with Sjögren’s syndrome, researchers hope to uncover the underlying causes and potentially develop targeted therapies.

Extraglandular Manifestations of Sjögren’s Syndrome

Another research study focuses on the extraglandular manifestations of Sjögren’s syndrome, referring to symptoms and complications that occur outside the glands. By studying different tissues and organs affected by Sjögren’s syndrome, researchers aim to gain insight into the systemic nature of the disease and develop better treatment strategies for patients with related disorders.

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Advocacy and Support Resources for Sjögren’s Syndrome

In addition to clinical research, studies are also being conducted to develop advocacy and support resources for patients with Sjögren’s syndrome. These resources aim to provide information, educational materials, and emotional support for patients and their families. By collaborating with advocacy organizations and patients, researchers hope to improve the quality of life for individuals living with this rare autoimmune condition.

Additional Information and References

For additional information on research studies related to Sjögren’s syndrome, interested individuals can visit ClinicalTrials.gov, a comprehensive catalog of ongoing clinical trials. PubMed, another scientific database, may also have articles available on the topic. Omim, a genetic database, contains information on genes associated with Sjögren’s syndrome and other related diseases.

Catalog of Genes and Diseases from OMIM

Sjögren’s syndrome is a complex autoimmune disease that primarily affects the exocrine glands, causing dryness of the eyes and mouth. It is characterized by lymphocytic infiltration of the affected tissues and increased levels of autoantibodies. In addition to the involvement of the exocrine glands, Sjögren’s syndrome can also affect other tissues, leading to systemic manifestations.

The exact cause of Sjögren’s syndrome remains unknown, but it is believed to have a combination of genetic and environmental factors. Research has identified several genes that may be associated with the development of Sjögren’s syndrome. These genes play a role in the immune system, regulating the production and function of certain immune cells. Rare genetic mutations in these genes can lead to an increased risk of developing the condition.

OMIM (Online Mendelian Inheritance in Man) is a comprehensive database that catalogs genes and diseases. It provides information on the inheritance patterns, clinical features, and genetic basis of various diseases, including Sjögren’s syndrome. OMIM is a valuable resource for researchers, clinicians, and patients.

OMIM Resources on Sjögren’s Syndrome:

  • ClinicalTrials.gov: This resource provides information on ongoing clinical trials related to Sjögren’s syndrome. By participating in these trials, patients can contribute to the advancement of research and access new treatment options.
  • Scientific Articles: OMIM includes references to scientific articles that discuss the genetic basis, clinical features, and management of Sjögren’s syndrome. These articles provide valuable information for researchers and clinicians.
  • Genetic Information: OMIM provides detailed genetic information on genes that are associated with Sjögren’s syndrome. This information helps in understanding the underlying mechanisms of the disease and may lead to the development of targeted therapies.
  • Support and Advocacy Organizations: OMIM lists support and advocacy organizations that provide resources, educational materials, and support for individuals affected by Sjögren’s syndrome.
  • Additional Information: OMIM provides additional information on rare causes of Sjögren’s syndrome, extraglandular manifestations, and other related disorders. This information helps in a comprehensive understanding of the condition.

OMIM is an invaluable tool for researchers, clinicians, and patients seeking information about Sjögren’s syndrome. By accessing the extensive catalog of genes and diseases, individuals can stay informed about the latest research, genetic discoveries, and treatment options.

Scientific Articles on PubMed

  • A study on the genes associated with Sjögren syndrome.
  • Analysis of the systemic features of Sjögren syndrome.
  • Information on the rare occurrence of Sjögren syndrome.
  • Advocacy and support resources available for Sjögren syndrome patients.
  • Developing clinical trials for Sjögren syndrome through clinicaltrialsgov.
  • Uncovering the involvement of other tissues in Sjögren syndrome.
  • The relationship between Sjögren syndrome and other diseases.
  • Inheritance patterns of Sjögren syndrome.
  • Insights from studies on Sjögren syndrome available on PubMed.
  • An overview of the genetic basis of Sjögren syndrome.
  • Scientific articles on rare variants of Sjögren syndrome.
  • The role of infections in the development of Sjögren syndrome.
  • Understanding the inheritance of Sjögren syndrome from OMIM resources.
  • Another name for Sjögren syndrome: Sicca syndrome.
  • Sjögren syndrome and its impact on the skin.
  • Causes of Sjögren syndrome.
  • The prevalence of Sjögren syndrome in women.
  • A catalog of references on Sjögren syndrome.
  • Genetic factors contributing to the development of Sjögren syndrome.
  • Available support system for Sjögren syndrome patients.
  • Studies on Sjögren syndrome listed on PubMed.
  • OMIM resources for understanding the genetic basis of Sjögren syndrome.
  • Research articles on the rare extraglandular features of Sjögren syndrome.
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    References

    • Mavragani CP, et al. The genetics of Sjögren’s syndrome: an update. Clin Exp Rheumatol. 2019;37 Suppl 118(3):97-103. PubMed PMID: 31498049.
    • Sjögren’s Syndrome Foundation. About Sjögren’s. Available from: https://www.sjogrens.org. Accessed November 17, 2021.
    • Sjögren’s Syndrome Foundation. Sjögren’s Syndrome Resources. Available from: https://www.sjogrens.org/resources. Accessed November 17, 2021.
    • Brito-Zerón P, et al. Systemic involvement in primary Sjögren’s syndrome evaluated by the EULAR-SS Disease Activity Index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford). 2014;53(2):321-31. PubMed PMID: 24163227.
    • Sjögren’s Syndrome Foundation. Clinical Trials in Sjögren’s. Available from: https://www.sjogrens.org/research/clinical-trials. Accessed November 17, 2021.
    • Paiva ES, et al. Skin disorders in primary Sjögren syndrome: a case series. Indian J Dermatol. 2018;63(3):249-251. PubMed PMID: 29861530.
    • Omim. Sjögren-Larsson Syndrome. Available from: https://omim.org/entry/270200. Accessed November 17, 2021.
    • Sjögren’s Syndrome Foundation. Sjögren’s FAQs. Available from: https://www.sjogrens.org/about-sjogrens. Accessed November 17, 2021.
    • Buchanan WW, et al. Frequency of extraglandular sites of involvement in primary Sjögren’s syndrome. Scand J Rheumatol Suppl. 1986;61:26-30. PubMed PMID: 3750193.

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