Sjögren's syndrome
| Sjögren syndrome | |
|---|---|
| Synonyms | Sjögren syndrome |
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| Image with a microscope of focal lymphoid infiltration in the minor salivary gland associated with Sjögren's syndrome, H & E stain | |
| Pronunciation | English /ˈʃoʊɡrᵻn/ or /ˈʃɜːrɡrɛn/ Swedish [ˈɧøːɡreːn] |
| Classification and external resources | |
| Specialty | Rheumatology |
| ICD-10 | M35.0 |
| ICD-9-CM | 710.2 |
| OMIM | 270150 |
| DiseasesDB | 12155 |
| MedlinePlus | 000456 |
| eMedicine | med/2136 emerg/537 derm/846 ped/2811 oph/477 oph/695 |
| Patient UK | Sjögren's syndrome |
| MeSH | D012859 |
Sjögren's syndrome (SjS, SS) is a long-term autoimmune disease in which the moisture-producing glands of the body are affected. This results primarily in the development of a dry mouth and . Other symptoms can include dry skin, a chronic cough, vaginal dryness, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. Those affected are at an increased risk (5%) of lymphoma.
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. It can occur independently of other health problems (primary Sjögren's syndrome) or as a result of another connective tissue disorder (secondary Sjögren's syndrome). The inflammation that results progressively damages the glands. Diagnosis is by biopsy of moisture-producing glands and blood tests looking for specific antibodies. On biopsy there is typically lymphocyte within the glands.
Treatment is directed at the person's symptoms. For dry eyes artificial tears, medications to reduce inflammation, or surgery to shut the tear ducts, may be tried. For a dry mouth, chewing gum, sipping water, or a saliva substitute may be used. In those with joint or muscle pain, ibuprofen may be used. Medications that can cause dryness may also be stopped such as antihistamines.
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. Between 0.2% and 1.2% of the population are affected, with half having the primary form and half the secondary form. Females are affected about ten times as often as males and it commonly begins in middle age; however, anyone can be affected. Among those without other autoimmune disorders, life expectancy is unchanged.
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