Instruct: Given a question, retrieve documents that can help answer the question.
Query: When was sjögren syndrome first identified?
The disease was described in 1933 by Henrik Sjögren, after whom it is named; however, a number of earlier descriptions of people with the symptoms exist.[3] Between 0.2% and 1.2% of the population are affected, with half having the primary form and half the secondary form.[7] Females are affected about ten times as often as males and it commonly begins in middle age;[2][3] however, anyone can be affected.[2] Among those without other autoimmune disorders, life expectancy is unchanged.[6]
With Charles Lucien de Beurmann (1851-1923), he conducted extensive research of fungal diseases that included pioneer studies of sporotrichosis. In 1925 he described three separate cases of atrophy of the salivary glands associated with dryness of the eyes, mouth and vagina. Several years later, Swedish ophthalmologist Henrik Sjögren (1899-1986) wrote a detailed and comprehensive report of the disease in ""Zur Kenntnis der keratoconjunctivitis sicca"" (To the knowledge of keratoconjunctivitis sicca). Today this autoimmune disease is known as Sjögren's syndrome, however it is sometimes referred to as "Gougerot-Sjögren syndrome".
Marinesco–Sjögren syndrome (MSS), sometimes spelled Marinescu–Sjögren syndrome, is a rare autosomal recessive disorder.
Most cases of benign lymphoepithelial lesions appear in conjunction with Sjögren's syndrome. When Sjögren's syndrome is present, the swelling is usually bilateral. Otherwise, the affected glands are usually only on one side of the body.
In 2014, the Sjögren's Syndrome Foundation announced a five-year goal to cut the time to diagnoses in half.[62]
It should not be confused with Sjögren's syndrome, which is a distinct condition named after a different person (Henrik Sjögren).
While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacteria.[4] It can occur independently of other health problems (primary Sjögren syndrome) or as a result of another connective tissue disorder (secondary Sjögren syndrome).[3] The inflammation that results progressively damages the glands.[7] Diagnosis is by biopsy of moisture-producing glands and blood tests looking for specific antibodies.[2] On biopsy there are typically lymphocytes within the glands.[2]
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).[59] The UK Primary Sjögren's Syndrome Registry supports clinical trials and genetic studies of SS and is open to people wishing to participate in research studies and researchers studying the disease.[56]
Sjögren–Larsson syndrome is named after him (along with Tage Larsson) as well as Marinesco–Sjögren syndrome.
He was also involved in the characterization of juvenile neuronal ceroid lipofuscinosis.
"Sjögren's syndrome:"
Chronic inflammation of the salivary glands may also be an autoimmune disease known as Sjögren's syndrome. The disease most commonly appears in people aged 40–60 years, but it may affect small children. In Sjögren syndrome, the prevalence of parotitis in women versus men is approximately 9:1. The involved parotid gland is enlarged and tender at times. The cause is unknown. The syndrome is often characterized by excessive dryness in the eyes, mouth, nose, vagina, and skin.
In ophthalmology, clobetasone butyrate 0.1% eye drops have been shown to be safe and effective in the treatment of dry eyes in Sjögren's Syndrome. Sjögren's Syndrome is an autoimmune disorder that affects the moisture producing glands of the body causing many symptoms including dry eyes. When compared to other corticosteroid eye drops; clobetasone butyrate showed only minimal rises in intraocular pressure. Increased pressure within the eye can lead to glaucoma.
Xerostomia may be caused by autoimmune conditions which damage saliva-producing cells. Sjögren's syndrome is one such disease, and it is associated with symptoms including fatigue, myalgia and arthralgia. The disease is characterised by inflammatory changes in the moisture-producing glands throughout the body, leading to reduced secretions from glands that produce saliva, tears and other secretions throughout the body. Primary Sjögren's syndrome is the combination of dry eyes and xerostomia. Secondary Sjögren's syndrome is identical to primary form but with the addition of a combination of other connective tissue disorders such as systemic lupus erythematosus or rheumatoid arthritis.
SS is usually classified as either 'primary' or 'secondary'. Primary Sjögren syndrome occurs by itself and secondary Sjögren syndrome occurs when another connective tissue disease is present.
Anti-Ro and anti-La antibodies, also known as SS-A and SS-B, respectively, are commonly found in primary Sjögren's syndrome, an autoimmune disorder that affects the exocrine glands. The presence of both antibodies is found in 30–60% of Sjögren's syndrome, anti-Ro antibodies alone are found in 50–70% of Sjögren's syndrome and 30% of SLE with cutaneous involvement, and anti-La antibodies are rarely found in isolation. Anti-La antibodies are also found in SLE; however, Sjögren's syndrome is normally also present. Anti-Ro antibodies are also found less frequently in other disorders including autoimmune liver diseases, coeliac disease, autoimmune rheumatic diseases, cardiac neonatal lupus erythematosus and polymyositis. During pregnancy, anti-Ro antibodies can cross the placenta and cause neonatal lupus in babies. In Sjögren's syndrome, anti-Ro and anti-La antibodies correlate with early onset, increased disease duration, parotid gland enlargement, disease outside the glands and infiltration of glands by lymphocytes. Anti-Ro antibodies are specific to components of the Ro-RNP complex, comprising 45kDa, 52kDa, 54kDa and 60kDa proteins and RNA. The 60kDa DNA/RNA binding protein and 52kDa T-cell regulatory protein are the best characterised antigens of anti-Ro antibodies. Collectively, these proteins are part of a ribonucleoprotein (RNP) complex that associate with the hyRNAs, hY1-hY5. The La antigen is a 48kDa transcription termination factor of RNA polymerase III, which associates with the Ro-RNP complex.
He should not be confused with Henrik Sjögren, after whom Sjögren's syndrome is named.
Henrik Samuel Conrad Sjögren (, ; ; 23 July 1899, Köping – 17 September 1986, Lund) was a Swedish ophthalmologist best known for describing the eponymous condition Sjögren's syndrome. Sjögren received his medical degree in Stockholm 1927 and in 1933 published a doctoral thesis at Karolinska Institutet titled "On knowledge of keratoconjunctivitis" that eventually served as the basis of identifying and naming of Sjögren's syndrome. He had one child born in 1934 named Gunvor. 
After extensive research and data collection, Sjögren published an essential paper in 1951, describing 80 patients with keratoconjunctivitis sicca, 50 of whom also had arthritis.[55] His subsequent follow-up conference trips pertaining to his paper led to an international interest in SS.[55] The term keratoconjunctivitis sicca was coined by Sjögren himself and began to be identified as SS in literature,[55] although it can now have more general usage.
It is associated with a deficiency of the enzyme fatty aldehyde dehydrogenase (ALDH3A2) located on the short arm of chromosome 17 (17p11.2). At least 11 distinct mutations have been identified.
Lactoferrin levels in tear fluid have been shown to decrease in dry eye diseases such as Sjögren's syndrome. A rapid, portable test utilizing microfluidic technology has been developed to enable measurement of lactoferrin levels in human tear fluid at the point-of-care with the aim of improving diagnosis of Sjögren's syndrome and other forms of dry eye disease.
Sjögren syndrome type B antigen (SS-B) also known as Lupus La protein is a protein that in humans is encoded by the "SSB" gene.
Henrik Sjögren should not be confused with his contemporary, Torsten Sjögren, after whom Sjögren–Larsson syndrome and Marinesco–Sjögren syndrome are named.
Nine out of ten SS patients are women.[14][49] 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.[51] Differences in prevalence due to race and ethnicity are unknown.
It is named for Gheorghe Marinescu and Torsten Sjögren.
Differential diagnosis includes Congenital Cataracts Facial Dysmorphism Neuropathy (CCFDN), Marinesco–Sjögren like syndrome with chylomicronemia, carbohydrate deficient glycoprotein syndromes, Lowe syndrome, and mitochondrial disease.
Anti-SSA autoantibodies (Anti-Sjögren’s-syndrome-related antigen A, also called anti-Ro, or the combination anti-SSA/Ro or anti Ro/SSA autoantibodies) are anti-nuclear autoantibodies that are associated with many autoimmune diseases, such as systemic lupus erythematosus (SLE), SS/SLE overlap syndrome, subacute cutaneous lupus erythematosus (SCLE), neonatal lupus and primary biliary cirrhosis. Also, they are often present in Sjögren's syndrome.
Presence of Anti-SSA/Ro in pregnant women with SLE is associated with an increased risk of neonatal lupus erythematosus, a disease process identified by Hooman Azad, which can present with congenital heart block in the fetus.