Sjogren Syndrome

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MedlinePlus Genetics: ⁴² Sjögren syndrome is a disorder whose main features are dry eyes and a dry mouth. The condition typically develops gradually beginning in middle adulthood but can occur at any age.Sjögren syndrome is classified as an autoimmune disorder, one of a large group of conditions that occur when the immune system attacks the body's own tissues and organs. In Sjögren syndrome, the immune system primarily attacks the glands that produce tears (the lacrimal glands) and saliva (the salivary glands), impairing the glands' ability to secrete these fluids.Dry eyes may lead to itching, burning, a feeling of sand in the eyes, blurry vision, or intolerance of bright or fluorescent lighting. A dry mouth can feel chalky or full of cotton, and affected individuals may have difficulty speaking, tasting food, or swallowing. Because saliva helps protect the teeth and the tissues of the oral cavity, people with Sjögren syndrome are at increased risk of tooth decay and infections in the mouth.In most people with Sjögren syndrome, dry eyes and dry mouth are the primary features of the disorder, and general health and life expectancy are largely unaffected. However, in some cases the immune system also attacks and damages other organs and tissues. This complication is known as extraglandular involvement. Affected individuals may develop inflammation in connective tissues, which provide strength and flexibility to structures throughout the body. Disorders involving connective tissue inflammation are sometimes called rheumatic conditions. In Sjögren syndrome, extraglandular involvement may result in painful inflammation of the joints and muscles; dry, itchy skin and skin rashes; chronic cough; a hoarse voice; kidney and liver problems; numbness or tingling in the hands and feet; and, in women, vaginal dryness. Prolonged and extreme tiredness (fatigue) severe enough to affect activities of daily living may also occur in this disorder. A small number of people with Sjögren syndrome develop lymphoma, a blood-related cancer that causes tumor formation in the lymph nodes but can spread to other organs.Some individuals who are first diagnosed with another rheumatic disorder, such as rheumatoid arthritis or systemic lupus erythematosus, later develop the dry eyes and dry mouth characteristic of Sjögren syndrome. Other autoimmune disorders can also develop after the onset of Sjögren syndrome. In all, about half of all individuals with Sjögren syndrome also have another autoimmune disorder.

MalaCards based summary: Sjogren Syndrome, also known as sjogren's syndrome, is related to mononeuritis multiplex and aplasia of lacrimal and salivary glands, and has symptoms including dryness of eye, eye manifestations and back pain. An important gene associated with Sjogren Syndrome is IFNG-AS1 (IFNG Antisense RNA 1), and among its related pathways/superpathways are Apoptosis and Autophagy and NF-kappaB Signaling. The drugs Fluorometholone and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include eye, salivary gland and skin, and related phenotypes are keratoconjunctivitis sicca and xerostomia

MedlinePlus: ⁴¹ Sjogren's syndrome is an autoimmune disease. This means that your immune system attacks parts of your own body by mistake. In Sjogren's syndrome, it attacks the glands that make tears and saliva. This causes a dry mouth and dry eyes. You may have dryness in other places that need moisture, such as your nose, throat, and skin. Sjogren's can also affect other parts of the body, including your joints, lungs, kidneys, blood vessels, digestive organs, and nerves. Most people with Sjogren's syndrome are women. It usually starts after age 40. It is sometimes linked to other diseases such as rheumatoid arthritis and lupus. To make a diagnosis, doctors may use a medical history, physical exam, certain eye and mouth tests, blood tests, and biopsies. Treatment focuses on relieving symptoms. It can differ for each person; it depends on what parts of the body are affected. It may include artificial tears for dye eyes and sucking on sugar-free candy or drinking water often for a dry mouth. Medicines may help with severe symptoms. NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

NINDS: ⁵² Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid arthritis. The hallmark symptoms of the disorder are dry mouth and dry eyes. In addition, Sjogren'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. Sjögren's syndrome affects 1-4 million people in the United States. Most people are more than 40 years old at the time of diagnosis. Women are 9 times more likely to have Sjögren's syndrome than men.

Orphanet: ⁵⁸ A rare systemic autoimmune disease characterized by exocrine gland dysfunction, resulting predominately in keratoconjunctivitis sicca and xerostomia, but also affecting exocrine glands of the skin, as well as respiratory, urogenital, and digestive tract. Extraglandular manifestations include arthritis, interstitial lung disease, renal disease, and peripheral neuropathy. The disease is accompanied by a substantially increased risk to develop B-cell non-Hodgkin lymphoma, especially MALT (mucosa-associated lymphoid tissue) lymphoma.

GARD: ¹⁹ Sjogren syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjogren syndrome is also associated with rheumatic disorders such as rheumatoid arthritis or systemic lupus erythematosus. The hallmark symptoms of Sjogren syndrome are dry mouth and dry eyes. In addition, Sjogren syndrome may cause skin, nose, and vaginal dryness. It also may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain.

OMIM®: ⁵⁷ Sjogren syndrome is an autoimmune disease that mainly affects the exocrine glands. It is clinically characterized by keratoconjunctivitis sicca and xerostomia (Goransson et al., 2006). See 200400 for association of Sjogren syndrome with achalasia in sisters. (270150) (Updated 24-Oct-2022)

Disease Ontology: ¹¹ An autoimmune disease that involves attack of immune cells which destroy the exocrine glands that produce tears and saliva.

Wikipedia: ⁷⁵ Sjögren syndrome or Sjögren's syndrome (SjS, SS) is a long-term autoimmune disease that affects the... more...

Clinical features from OMIM®:

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