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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = {{PAGENAME}} | | | Name = {{PAGENAME}} | |
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| ICDO = | | | ICDO = | |
| OMIM = 270150 | | | OMIM = 270150 | |
| MedlinePlus = 000456 | | | MedlinePlus = | |
| eMedicineSubj = med |
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| eMedicineTopic = 2136 |
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| eMedicine_mult = {{eMedicine2|emerg|537}} {{eMedicine2|derm|846}} {{eMedicine2|ped|2811}} {{eMedicine2|oph|477}} {{eMedicine2|oph|695}} |
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| MeshID = D012859 | | | MeshID = D012859 | |
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| {{Search infobox}} | | {{Sjögren's syndrome}} |
| {{CMG}} | | {{CMG}} {{AE}} {{F.K}} |
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| '''Associate Editor-In-Chief:''' {{CZ}} | | {{SK}} Sicca syndrome; Sjogren syndrome; Sjögren syndrome; |
| | ==[[Sjögren's syndrome overview|Overview]]== |
| | ==[[Sjögren's syndrome historical perspective|Historical Perspective]]== |
| | ==[[Sjögren's syndrome classification|Classification]]== |
| | ==[[Sjögren's syndrome pathophysiology|Pathophysiology]]== |
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| {{Editor Join}}
| | ==[[Sjögren's syndrome causes|Causes]]== |
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| ==Overview== | | ==[[Sjögren's syndrome differential diagnosis|Differentiating Sjögren's syndrome from other Diseases]]== |
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| '''Sjögren's syndrome''' is an [[autoimmunity|autoimmune disorder]] in which [[immune cell]]s attack and destroy the [[exocrine gland]]s that produce [[tears]] and [[saliva]]. It is named after Swedish [[ophthalmology|ophthalmologist]] Henrik Sjögren (1899-1986), who first described it. Sjögren's syndrome is also associated with [[rheumatic]] disorders such as [[rheumatoid arthritis]], and it is [[rheumatoid factor]] positive in 90 percent of cases. The hallmark symptoms of the disorder are [[dry mouth]] and dry eyes (part of what are known as sicca symptoms). In addition, Sjögren's syndrome may cause [[skin]], [[nose]], and vaginal dryness, and may affect other [[organ (anatomy)|organ]]s of the body, including the [[kidney]]s, [[blood vessel]]s, [[lung]]s, [[liver]], [[pancreas]], and [[brain]]. Nine out of ten Sjögren's patients are women and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone making it the second most common autoimmune rheumatic disease.
| | ==[[Sjögren's syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
| | | ==[[Sjögren's syndrome risk factors|Risk Factors]]== |
| The primary form, often called sicca syndrome, involves both [[Keratoconjunctivitis sicca]] and [[Xerostomia]]. The secondary form includes, in addition, the presence of a connective tissue disease, usually [[rheumatoid arthritis]].
| | ==[[Sjögren's syndrome screening|Screening]]== |
| | ==[[Sjögren's syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Diagnosis== | | ==Diagnosis== |
| Diagnosing Sjögren’s syndrome is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms from Sjögren's syndrome and those caused by other conditions. Nevertheless, the combination of several tests can lead to a diagnosis of Sjögren's syndrome.
| | [[Sjögren's syndrome diagnostic study of choice|Diagnostic Study of Choice]] | [[Sjögren's syndrome history and symptoms|History and Symptoms]] | [[Sjögren's syndrome physical examination|Physical Examination]] | [[Sjögren's syndrome laboratory findings|Laboratory Findings]] | [[Sjögren's syndrome x ray|X Ray]] | [[Sjögren's syndrome CT|CT]] | [[Sjögren's syndrome MRI|MRI]] | [[Sjögren's syndrome other imaging findings|Other Imaging Findings]] | [[Sjögren's syndrome other diagnostic studies|Other Diagnostic Studies]] |
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| Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as [[anti-nuclear antibody]] (ANA) and [[rheumatoid factor]], which are associated with autoimmune diseases. Typical Sjögren syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions but are often present in Sjögren's (Franceschini & Cavazzana I 2005).
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| The [[Schirmer's test|Schirmer test]] measures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler. A [[slit lamp|slit-lamp examination]] is done to look for dryness on the surface of the eye. Salivary gland function can be tested by collecting saliva and determining the amount produced. A lip [[biopsy]] can reveal [[lymphocyte]]s clustered around salivary glands, and damage to these glands due to [[inflammation]].
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| A radiological procedure can also be used as a reliable and accurate way of diagnosing Sjögren's syndrome. A contrast is injected into Stensen's Duct (i.e., parotid duct, Steno's Duct), which is a duct opening from the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth. Widespread puddling of the injected contrast scattered throughout the gland indicates Sjögren's syndrome.
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| ===Classification Criteria===
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| Revised Classification Criteria for Sjogren's Syndrome<ref>Ann Rheum Dis 2002; 61: 54-558</ref>
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| A. '''Ocular symptoms''': a positive response to at least one of the following questions:
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| :1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?
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| :2. Do you have a recurrent sensation of sand or gravel in the eyes?
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| :3. Do you use tear substitutes more than 3 times a day?
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| B. '''Oral symptoms''': a positive response to at least one of the following questions:
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| :1. Have you had a daily feeling of dry mouth for more than 3 months?
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| :2. Have you had recurrently or persistently swollen salivary glands as an adult?
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| :3. Do you frequently drink liquids to aid in swallowing dry food?
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| C. '''Ocular signs'''- that is, objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:
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| :1. Schirmer's test, performed without anaesthesia ( ≦5 mm in 5 minutes )
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| :2. Rose bengal score or other ocular dye score ( ≧4 according to van Bijsterveld's scoring system )
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| D. '''Histopathology''': In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score ≧1, defined as a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm2 of glandular tissue.
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| E. '''Salivary gland involvement''': objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests:
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| :1. Unstimulated whole salivary flow ( ≦ 1.5 ml in 15 minutes )
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| :2. Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary,or destructive pattern ), without evidence of obstruction in the major ducts.
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| :3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer
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| F. '''Autoantibodies''': presence in the serum of the following autoantibodies:
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| :1. antibodies to Ro(SSA) or La(SSB) antigens, or both
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| ===Revised rules for classification===
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| ====For primary SS====
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| In patients without any potentially associated disease, primary SS may be defined as follows:
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| :a. The presence of any 4 of the 6 items is indicative of primary SS, as long as either item 4 (Histopathology) or 6 (Serology) is positive.
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| :b. The presence of any 3 of the 4 objective criteria items (that is, items 3, 4, 5, 6)
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| :c. The classification tree procedure represents a valid alternative method for classification, although it should be more properly used in clinical-epidemiological survey.
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| ====For secondary SS====
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| In patients with a potentially associated disease (for instance, another well defined connective tissue disease), the presence of item 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS.
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| ===Exclusive criteria===
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| :Past head and neck [[radiation therapy]]
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| :[[Hepatitis C]] infection
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| :[[Acquired immunodeficiency syndrome]] ([[AIDS]])
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| :Pre-existing [[lymphoma]]
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| :[[Sarcoidosis]]
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| :[[Graft-versus-host disease]]
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| :Use of [[anticholinergic]] drugs (since a time shorter than 4-fold life of the drug)
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| ==Treatment== | | ==Treatment== |
| There is neither a known cure for Sjögren's syndrome nor a specific treatment to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes (some patients with more severe problems use goggles to increase local humidity or have [[Lacrimal punctum|punctal]] plugs inserted to help retain tears on the occular surface for a longer time). Additionally, [[Cyclosporin]] (Restasis) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as [[cevimeline]] and [[pilocarpine]]. [[Nonsteroidal anti-inflammatory drug]]s may be used to treat musculoskeletal symptoms. For individuals with severe complications, [[corticosteroid]]s or immunosuppressive drugs may be prescribed. Also, [[disease-modifying antirheumatic drug]]s (DMARDs) such as [[methotrexate]] may be helpful.
| | [[Sjögren's syndrome medical therapy|Medical Therapy]] | [[Sjögren's syndrome surgery|Surgery]] | [[Sjögren's syndrome primary prevention|Primary Prevention]] | [[Sjögren's syndrome secondary prevention|Secondary Prevention]]| [[Sjögren's syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Sjögren's syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| ==Prognosis==
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| Sjögren's can damage vital organs of the body with symptoms that may plateau, worsen, or go into [[remission (medicine)|remission]]. Some people may experience only the mild symptoms of dry eyes and mouth, while others go through cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically. Others are forced to cope with [[blurred vision]], constant eye discomfort, recurrent mouth [[infection]]s, swollen [[parotid gland]]s, [[hoarseness]], and difficulty in swallowing and eating. Debilitating [[fatigue (physical)|fatigue]] and [[joint pain]] can seriously impair quality of life. Some patients can develop renal involvement (autoimmune tubulointerstitial nephritis) leading to [[proteinuria]], urinary concentrating defect and distal [[renal tubular acidosis]]. | |
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| ==Epidemiology==
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| Sjogren'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 Sjogren's syndrome than men.
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| ==Research==
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| The goals of research on diseases such as Sjögren's syndrome focus on increasing knowledge and understanding of the disorder, improving diagnostic techniques, and finding ways to treat, prevent, and cure the disorder.
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| Many scientists are working on developing an animal model of Sjögren's syndrome. Dr Hal Scofield's lab at Oklahoma Medical Research Foundation have developed an animal model by immunizing mice with 60 kD Ro peptide. Days after immunization, salivary flow was decreased and lymphocyte infiltrates as well as salivary dysfunction was observed which are highly reminiscent of human Sjögren's syndrome.
| | ==Case Studies== |
| | :[[Sjögren's syndrome case study one|Case #1]] |
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| ==See also== | | ==Related Chapters== |
| * [[Benign lymphoepithelial lesion]] | | * [[Benign lymphoepithelial lesion]] |
| * [[Keratoconjunctivitis sicca]] | | * [[Keratoconjunctivitis sicca]] |
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| * [[Sjögren-Larsson syndrome]] | | * [[Sjögren-Larsson syndrome]] |
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| ==References==
| | [[Category:Medicine]] |
| {{reflist}}
| | [[Category:Immunology]] |
| * Franceschini F, Cavazzana I. Anti-Ro/SSA and La/SSB antibodies. ''Autoimmunity'' 2005;38:55-63. PMID 15804706.
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| *Sjögren H. ''Zur Kenntnis der keratoconjunctivitis sicca''. Doctoral thesis, 1933.
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| *Scofield RH, Asfa S, Obeso D, Jonsson R, Kurien BT. Immunization with short peptides from the 60-kDa Ro antigen recapitulates the serological and pathological findings as well as the salivary gland dysfunction of Sjogren's syndrome.J Immunol. 2005 Dec 15;175(12):8409-14.
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| *Kurien BT, Asfa S, Li C, Dorri Y, Jonsson R, Scofield RH. Induction of oral tolerance in experimental Sjogren's syndrome autoimmunity. Scand J Immunol. 2005 May;61(5):418-25.
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| ==External links==
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| * The original text from this article was obtained from a [[public domain]] resource at: [http://www.ninds.nih.gov/health_and_medical/disorders/sjogrens_doc.htm www.ninds.nih.gov/health_and_medical/disorders/sjogrens_doc]
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| * [http://www.sjogrens.netfirms.com Sjogrens Sarasota]
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| * [http://www.sjogrens.org Sjögren's Syndrome Foundation]
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| * [http://www-scf.usc.edu/~arianjam/sjs/ Role of MHC Class II Molecules in the Development of Sjögren’s Syndrome in the NOD Mouse]
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| * [http://www.mayoclinic.com/invoke.cfm?id=DS00147 Sjögren's Syndrome article by Mayo Clinic staff]
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| * [http://www.sjogrenssyndrome.info/ Sjögren's Syndrome Information]
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| * {{cite news | first= | last= | coauthors= | title=Green tea may help prevent autoimmune diseases | date=April 18 2007 | publisher=Medical College of Georgia | url =https://my.mcg.edu/portal/page/portal/News/archive/2007/Green%20tea%20may%20help%20prevent%20autoimmune%20diseases | work = | pages = | accessdate = 2007-04-20 | language = }}
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| {{Diseases of the musculoskeletal system and connective tissue}}
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| {{DEFAULTSORT:Sjogren's syndrome}}
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| {{SIB}}
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| [[Category:Autoimmune diseases]] | |
| [[Category:Rheumatology]] | | [[Category:Rheumatology]] |
| [[Category:Syndromes]] | | [[Category:Up-To-Date]] |
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| [[de:Sjögren-Syndrom]]
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| [[et:Sjögreni sündroom]]
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| [[es:Síndrome de Sjögren]]
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| [[fr:Syndrome de Goujerot-Sjögren]]
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| [[it:Sindrome di Sjögren]]
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| [[nl:Syndroom van Sjögren]]
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| [[ja:シェーグレン症候群]]
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| [[no:Sjögrens syndrom]]
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| [[pt:Síndrome de Sjögren]]
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