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| | <div class="editorbox"> '''Original Editor '''- [[User:Cassie Shay|Cassie Shay]] [[Pathophysiology of Complex Patient Problems|from Bellarmine University's Pathophysiology of Complex Patient Problems project.]] |
| <div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div> <div class="editorbox"> | | '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} |
| '''Original Editors '''- [http://www.physio-pedia.com/index.php5?title=User:Cassie_Shay Cassie Shay] [[Pathophysiology of Complex Patient Problems|from Bellarmine University's Pathophysiology of Complex Patient Problems project.]] | |
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| '''Lead Editors''' - Your name will be added here if you are a lead editor on this page. [[Physiopedia:Editors|Read more.]] | |
| </div> | | </div> |
| == Definition/Description == | | == Introduction == |
| | | [[File:Sjogrens Syndrome.jpeg|thumb| Signs and symptoms: SS]] |
| Sjogren's syndrome was first described by physician Henrik Sjogren in the early nineteen hundreds to explain the signs and symptoms of a group of women exhibiting chronic arthritis along with extremely dry eyes and dry mouth<ref name="American College of Rheumatology">American College of Rheumatology. Sjogren's Syndrome. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp (accessed 17 March 2011).</ref>. It is now understood that Sjogren's syndrome is an autoimmune connective tissue disease in which the body's own immune system attacks moisture producing glands, causing inflammation in addition to reduction in both the quality and quantity of the glands' secretions<ref name="American College of Rheumatology" />. As observed by Dr. Sjogren, the glands responsible for producing saliva and tears, the salivary and lachrymal glands respectively, are the organs most notably impacted by the disease<sup>2</sup>. However, Sjogren's syndrome is a systemic disorder in which many organs may be affected, including kidneys, gastrointestinal tract, blood vessels, respiratory tracts, liver, pancreas, and central nervous system<sup>3</sup>. Additionally, it is considered a rheumatic disease, like rheumatoid arthritis or lupus, and like these disease Sjogren's syndrome can cause inflammation in joints, muscles, skin, and other organs<sup>2</sup>.
| | Sjögren's syndrome (SS) is a [[Chronic Disease|chronic]] systemic [[Autoimmune Disorders|autoimmune disease]] characterized by [[Lymphocytes|lymphocytic]] infiltration of exocrine [[Hormones|glands]]. It is a relatively common condition that mainly affects the eyes and salivary glands, but can affect different parts of the body.<ref name=":0">Patel R, Shahane A. The epidemiology of Sjögren's syndrome. Clin Epidemiol. 2014 Jul 30;6:247-55. doi: 10.2147/CLEP.S47399. PMID: 25114590; PMCID: PMC4122257.Available: https://pubmed.ncbi.nlm.nih.gov/25114590/<nowiki/>(accessed 6/3/2022)</ref><ref name="NIAMS">National Institute of Arthritis and Musculoskeletal and Skin Diseases. Sjogren's Syndrome. http://ww.niams.niih.gov/Health_Info/Sjogrens_Syndrome/default.asp (accessed 17 March 2011).</ref>. |
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| {{#ev:youtube|-PIcNX5SJ0s|400|right}}
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| == Prevalence ==
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| Sjogren's syndrome is the second most prevalent autoimmune rheumatic disease. In 2008, it was estimated that 1.3 million Americans were affected<sup>2,3</sup>. Statistics from rheumotology clinics indicate that Sjogren's syndrome affects 0.5 - 1% of the general population, and approximately the same number of people have been diagnosed with Sjogren's syndrome and [http://www.physio-pedia.com/index.php5?title=Systemic_Lupus_Erythematosus Systemic Lupus Erythematosus] <sup>4</sup>.
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| Although, it can affect individuals of all genders and ages, including children, symptoms usually occur in women between the ages of 45 and 55 years old<sup>1</sup>. In fact, it is estimated that nine times as many women are affected when compared to men<sup>3</sup>. Among individuals with [http://www.physio-pedia.com/index.php5?title=Rheumatoid_Arthritis rheumatoid arthritis] or systemic sclerosis, thirty percent have histological evidence of Sjogren's syndrome<sup>3</sup>.
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| == Characteristics/Clinical Presentation ==
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| Patient's with Sjogren's syndrome can present with a number of complaints. However, the most common symptoms of this autoimmunue disease are dry mouth and dry eyes. These complaint may be considered mild in comparison to the symptoms commonly associated with other autoimmune diseases, such as rheumatoid arthritis. However, the dry eyes and dry mouth associated with Sjogren's syndrome can be quite severe. In SS, the extreme dry eyes is called keratoconjuctivitis sicca (KCS)<ref name="Monika">Szturmowicz M, Wilinska E, Paczek A, Wawrzynska L, Opoka L, Gatarek J, Langfort R, Rowinska-Zakrzewska E, Torbicki A. Primary Sjogren's syndrome with two extraglandular sites involvement- case report. Pneumonol. Alergol. Pol. 2010;78:445-450. https://docs.google.com/viewer?url=http%3A%2F%2Fwww.pneumonologia.viamedica.pl%2Fdarmowy_pdf.phtml%3Findeks%3D55%26indeks_art%3D644 (accessed 17 March 2011)</ref>, and individuals may describe it as a dry, burning, or gritty feeling, as though they have sand in their eyes<ref name="American College of Rheumatology" /><ref name="Pathology" />. The excessive dryness in the mouth, also known as xerostomia<ref name="Monika" />, may make it difficult to speak, swallow,chew, or it may make foods taste differently<ref name="Pathology" />.
| | There is no cure, but the condition can be managed with medications and products such as artificial tears and saliva as well as mouth washes and lozenges.<ref name=":1">Better Health Sjogren's Syndrome Available: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/sjogrens-syndrome#self-management-of-sjogren%E2%80%99s-syndrome<nowiki/>(accessed 6.3.2022)</ref> |
| | == Epidemiology == |
| | SS is the second most prevalent autoimmune rheumatic disease. In 2008, it was estimated that 1.3 million Americans were affected<ref name="NIAMS" /><ref name="Pathology">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. Denver, CO: Saunders, 2009.</ref> It can present as an entity by itself, primary Sjögren's syndrome (pSS), or in addition to another autoimmune disease, secondary Sjögren's syndrome (sSS). pSS has a strong female propensity and is more prevalent in Caucasian women, with the mean age of onset usually in the 4th to 5th decade.<ref name=":0" /> |
| | == Etiology == |
| | It is not known what causes SS. Genes may play a role, combined with an outside trigger eg bacteria or virus.It’s also more likely to affect people who have conditions such as rheumatoid arthritis, scleroderma and lupus<ref name=":1" />. |
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| While these are the hallmark signs of Sjogren's syndrome, 40 to 60 percent of individuals with primary SS will develop extraglandular problems<ref name="Monika" />.A patient may present with a number of other complaints including joint and muscle pain, fatigue, skin rashes or extremely dry skin, weakness, numbness, digestive problems, and Raynauld's phenomenon<ref name="American College of Rheumatology" /><ref name="NIAMS" />. Females diagnosed with Sjogren's may also experience vaginal dryness and pain with intercourse<ref name="NIAMS" />.<br>
| | == Clinical Presentation == |
| | This 90 second video takes a look at this syndrome. {{#ev:youtube|-PIcNX5SJ0s|400|right}}The more common symptoms of Sjogren’s syndrome include: |
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| == Associated Co-morbidities == | | * dry eyes (irritation, feeling gritty or itchy, burning) |
| | * dry mouth (or difficulty chewing or swallowing). |
| | [[Image:Sjogrenssymptoms.png|300px|alt=|thumb|Symptoms: Sjogren’s ]]Sjogren’s syndrome is a systemic condition, which means it can affect the entire body, other symptoms can include: |
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| Approximately half of patients who have been diagnosed with Sjogren's Syndrome have also been diagnosed with rheumatoid arthritis, lupus, or some other connective tissue disorder<ref name="American College of Rheumatology" />. Fewer people may also experience Sjogren's in association with lymphoma<ref name="American College of Rheumatology" />. As more research is developed on the topic, more and more co-morbities are being associated with SS. Recent studies suggest that clinical depression is also common among individuals diagnosed with this disease<ref name="NIAMS" />.
| | * swelling and tenderness of the glands around your face, neck, armpits and groin |
| | * tiredness (fatigue) |
| | * dry skin or rashes |
| | * joint pain and general achiness |
| | * dryness of the nose, ear and throat |
| | * vaginal dryness |
| | * bowel irritation. |
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| Secondary Sjogren's is a form of the syndrome which develops after the presentation of a primary disease, usually another autoimmune connective tissue disease<ref name="NIAMS">National Institute of Arthritis and Musculoskeletal and Skin Diseases. Sjogren's Syndrome. http://ww.niams.niih.gov/Health_Info/Sjogrens_Syndrome/default.asp (accessed 17 March 2011).</ref>. Common primary diseases associated with Sjogren's Syndrome are listed in the table below.
| | Complications can include: |
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| {| cellspacing="1" cellpadding="1" border="1" style="width: 613px; height: 129px;"
| | * increased risk of dental decay |
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| | * increased risk of developing thrush infections in the mouth |
| | Disease
| | * vision problems |
| | Description
| | * inflammation of internal organs (such as the kidneys, lungs or liver) |
| |-
| | * problems with the circulatory and nervous system.<ref name=":1" /> |
| | [http://www.ninds.nih.gov/disorders/polymyositis/polymyositis.htm Polymyositis]
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| | Inflammation of muscles that cause pain, weaking, and difficulty moving. If the skin is involved too, it is called [http://www.physio-pedia.com/index.php5?title=Dermatomyositis dermatomyositis].
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| | [http://www.physio-pedia.com/index.php5?title=Rheumatoid_Arthritis Rheumatoid Arthritis]
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| | Severe inflammation of the joints caused by the bodies own immune system attacking the synovium located throughout the body.
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| | [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/ Scleroderma]
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| | Accumulation of too much collagen, resulting in thick, tight skin, and possible damage to joints, muscles, and internal organs.
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| | [http://www.physio-pedia.com/index.php5?title=Systemic_Lupus_Erythematosus Systemic Lupus Erythematosus]
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| | An autoimmune disease in which the patient may experience joint pain, muscle pain, fatigue, weakness, skin rashes, and possibily even heart, lung, kidney, and nervous system issues.
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| | [http://www.physio-pedia.com/index.php5?title=Non_Hodgkin_Lymphoma Lymphoma]
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| | Although rare, there is a small percentage of individuals with Sjogren's whom also have lymphoma, which is cancer of the lymph system. This can affect salivary glands, lymph nodes, the gastrointestinal tract, and lungs.
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| |}
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| == Medications == | | == Associated Co-morbidities == |
| | [[File:Cytokine profile in Sjogren’s syndrome.png|thumb|Cytokine profile found in SS ]] |
| | Secondary Sjogren's is a form of the syndrome which develops after the presentation of a primary disease, usually another autoimmune connective tissue disease<ref name="NIAMS" />. Common primary diseases associated with Sjogren's Syndrome include: [http://www.ninds.nih.gov/disorders/polymyositis/polymyositis.htm Polymyositis]; [http://www.physio-pedia.com/index.php5?title=Rheumatoid_Arthritis Rheumatoid Arthritis]; [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/ Scleroderma;] [http://www.physio-pedia.com/index.php5?title=Systemic_Lupus_Erythematosus Systemic Lupus Erythematosus]; [http://www.physio-pedia.com/index.php5?title=Non_Hodgkin_Lymphoma Lymphoma].<ref name="American College of Rheumatology">American College of Rheumatology. Sjogren's Syndrome. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp (accessed 17 March 2011).</ref> |
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| There is no cure for Sjogren's syndrome, therefore its symptoms are managed through medications, both prescription and over the counter. A variety of medications may be used to treat each of the associated symptoms.<br>
| | Image R: The [[Cytokines|cytokine]] profile found in Sjogren’s syndrome (SS) is imbalanced with the overexpression of numerous pro-inflammatory cytokines (on the left) vs low or undetectable levels of anti-inflammatory cytokines (on the right in image). |
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| {| cellspacing="1" cellpadding="1" border="1" style="width: 705px; height: 140px;"
| | == Treatment == |
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| | There is no cure for Sjogren's syndrome, therefore its symptoms are managed through medications, both prescription and over the counter. A variety of medications may be used to treat each of the associated symptoms. Treatment may include: |
| | '''Drug category '''<br>
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| | style="vertical-align: top;" | '''Signs/ Symptoms Treated'''
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| | '''Examples of Brand Names'''
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| | Nonsteroidal anti-inflammatory drugs<br>
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| | style="vertical-align: top;" | Joint and Muscle Pain
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| | [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000598/ Ibuprofen ]<br>
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| | Corticosteriods<br>
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| | style="vertical-align: top;" | Inflammation of lungs, kidneys, blood vessels, or nervous system
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| | [http://www.nlm.nih.gov/medlineplus/druginfo/meds/a601102.html Prednisone]<br>
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| | Immune-modifying drugs<br>
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| | style="vertical-align: top;" | Overactivity of the immune system
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| | Plaquenil ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000171/ hydroxychloroquine]), Rheumatrex ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000547/ methotrexate]), Cytoxan ([http://www.nlm.nih.gov/medlineplus/druginfo/meds/a682080.html cyclophosphamide])<br>
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| | Artificial Tears<br>
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| | valign="middle" align="left" style="vertical-align: top;" rowspan="4" | Dry Eyes
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| | Clear Eyes, Visine Tears, etc.<br>
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| | style="vertical-align: top;" | Ointments (for night use)<br>
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| | style="vertical-align: top;" | GenTeal, Systane<br>
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| | style="vertical-align: top;" | Hydroxypropyl methylcellulose<br>
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| | style="vertical-align: top;" | Ocucoat<br>
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| | style="vertical-align: top;" | Topical steroids<br>
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| | style="vertical-align: top;" | Restasis ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000253/ cylcosporin A])<br>
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| | style="vertical-align: top;" | Saliva substitue<br>
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| | style="vertical-align: top;" rowspan="2" | Dry Mouth
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| | style="vertical-align: top;" | Oralube<br>
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| | style="vertical-align: top;" | Cholinergic Agonists<br>
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| | style="vertical-align: top;" | Salagen ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000465/ pilocarpine]), Evoxac ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000452/ cevimeline])<br>
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| |}
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| This table was created using information from the National Institute of Arthritis and Musculoskeletal and Skin Diseases<ref name="NIAMS" /> and PubMed Health<ref name="Drugs">PubMed Health. Drugs and Supplements. http://www.ncbi.nlm.nih.gov/pubmedhealth/s/drugs_and_supplements/a/ (accessed 6 April 2011).</ref>, see references below.
| | * artificial tears and lubricating ointments for the eyes |
| | * artificial saliva |
| | * mouth rinses and lozenges |
| | * nasal sprays |
| | * vaginal lubricants |
| | * moisturising lotion for the skin |
| | * non-steroidal anti-inflammatory drugs (NSAIDs) – these medications (for example naproxen and ibuprofen) help control inflammation and provide temporary pain relief |
| | * corticosteroid medications – may be used as a temporary treatment for joint pain |
| | * immunosuppressive medications (for example hydroxychloroquine and methotrexate) – may be used to help control your overactive immune system<ref name=":1" />. |
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| == Diagnostic Tests/Lab Tests/Lab Values <br> == | | == Diagnostic Tests == |
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| Diagnosis of this disease is based on a medical history, a physical exam, and results from some clinical and laboratory tests<ref name="American College of Rheumatology" /><ref name="NIAMS" /><ref name="Pathology" />. Due to the nature of disease in that the symptoms are similar to other diseases and they appear gradually over time, it may take years for Sjogren's to be properly diagnosed<ref name="NIAMS" />. Nonetheless, diagnostic testing may include the following:[[Image:Lip Biopsy.jpg|thumb|right|300px|Histopathologic image of a lip biopsy demonstrating lymphoid infiltration in a minor salivary gland. H&E stain]]
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| *The [http://www.nlm.nih.gov/medlineplus/ency/article/003501.htm Schirmer's test] assesses the function of the lacrimal glands by measuring the amount of tears they are producing. This test involves putting a strip of paper under the lower eyelid, waiting five minutes, then measuring how much moisture was absorbed by the paper. An individual who tests positive for Sjogren's syndrome will typically produce too few tears to get more than 8 millimeters of the paper wet<ref name="NIAMS" />.
| | Diagnosis of this disease is based on medical history, a physical exam, and results from some clinical and laboratory tests<ref name="American College of Rheumatology" /><ref name="NIAMS" /><ref name="Pathology" />. Due to the nature of the disease in that the symptoms are similar to other diseases and they appear gradually over time, it may take years for Sjogren's to be properly diagnosed<ref name="NIAMS" />. Nonetheless, diagnostic testing may include the following: |
| *During a [http://www.nlm.nih.gov/medlineplus/ency/article/003880.htm slit lamp examination] an ophthalmologist magnifies and examines the eye for severe dryness and inflammation. This test may be performed in conjunction with a vital dye applied to the eyes to determining the extent to which the eyes may have been damaged secondary to the dryness<ref name="NIAMS" />.
| | * Schirmer’s test – special blotting paper held to the eye is used to assess the degree of tear production |
| *A mouth examination may be performed to determine if signs of mouth dryness are present. These signs may include, sticky oral mucosa, tooth decay and cavities in certain locations, thick saliva, and redness inside the oral cavity. The physician may also be able to note inflamed or under-producing salivary glands during this examination<ref name="NIAMS" />.
| | * eye examination – including the use of special dyes |
| *A [http://www.hopkinssjogrens.org/disease-information/diagnosis-sjogrens-syndrome/labial-gland-lip-biopsy/ lip biopsy] is a method used to determine if the dry mouth experienced by the patient is a symptom of Sjogren's syndrome. During this test, minor salivary glands are removed from the bottom lip and placed under a microscope. If the glands contain a certain arrangement of white blood cells, the test is positive for the salivary component of Sjogren's syndrome<ref name="NIAMS" />. | | * biopsy – a small piece of salivary gland tissue is removed (usually from the lip) and examined under a microscope |
| | * blood tests – may be used to check the levels of particular immune system cells in the blood, and to check for any problems with your kidneys or liver<ref name=":1" />. |
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| Because dry eyes and dry mouth are common symptoms and side affects of a number of diseases and treatments, including radiation to the head and neck, the physician will commonly perform a battery of additional tests to confirm no other causes. These tests may include:
| | == Physical Therapy Management == |
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| *Blood tests may be performed to determine the presence of antibodies and immunological substances often found in individuals positive for Sjogren's. Antibodies commonly present in affected individuals include immunoglobulins, antithyroid antibodies, rheumatoid factors, antinuclear antibodies (ANA), and Sjogren's antibodies.<br>
| | Individuals with Sjogren's syndrome have reduced physical capacity and tend to fatigue very easily. While research on the effects of exercise on individuals diagnosed with Sjogren's is limited, the available studies suggest these patients benefit from moderate to high-intensity levels of exercise<ref name="Pathology" />. This type of activity has a positive influence on aerobic capacity, fatigue, physical function, and mood. |
| *Chest X-Rays are a method used to examine for inflammation of the lungs, which may be present in Sjogren's syndrome.
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| *A urine analysis may also take place to determine if the kidneys are functioning properly<ref name="NIAMS" />.
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| Between 1989 and 1996, the classification criteria for Sjögren’s syndrome (SS) were established by the European Study Group on Classification for Sjogren's syndrome<ref name="Criteria">Vitali C, Bombardieri S, Jonsson R, Moutsopoulos H, Alexander E, Carsons S, Daniels T, Fox P, Fox R, Kassan S, Pilemer S, Tala N, Weisman M, and the European Study Group on Classification Criteria for Sjogren's Syndrome. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1754137/pdf/v061p00554.pdf (accessed 6 April 2011)</ref>. These criteria and rules for diagnosing SS were then revised in 2002 by the American-European Consensus Group. Many of these criteria for classification involve test explained above. The below tables display the classification critera for Sjogren's syndrome as proposed by the American-European Consensus Group:<br>
| | Further research is required to understand the effects exercise may have on individuals with varying severities of this disease, and to determine the long-term effects exercise may have on their symptoms<ref name="Pathology" />. However, some of the musculoskeletal presentations of SS are muscle and joint pain, along with arthritis. Thererfore, a patient may be treated by a physical therapist to relieve these symptoms<ref name="Medline">Medline Plus. Sjogren Syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/000456.htm (accessed 6 April 2011)</ref>. |
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| | == Lifestyle Changes == |
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| | '''Revised international classification criteria for Sjogren’s syndrome'''<ref name="Criteria" /><br>
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| | style="vertical-align: top;" | I. Ocular symptoms: a positive response to at least one of the following questions:<br> 1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?<br> 2. Do you have a recurrent sensation of sand or gravel in the eyes?<br> 3. Do you use tear substitutes more than 3 times a day?<br>
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| | II. Oral symptoms: a positive response to at least one of the following questions:<br> 1. Have you had a daily feeling of dry mouth for more than 3 months?<br> 2. Have you had recurrently or persistently swollen salivary glands as an adult?<br> 3. Do you frequently drink liquids to aid in swallowing dry food?<br>
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| | III. Ocular signs—objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:<br> 1. Schirmer's test, performed without anesthesia (less than or equal to 5 mm in 5 minutes)<br> 2. Rose bengal score or other ocular dye score (greater than 4 according to the van Bijsterveld's scoring system)<br>
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| | IV. Histopathology: In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score greater than or equal to 1, defined as a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm<sup>2</sup> of glandular tissue.<br>
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| | V. Salivary gland involvement: objective evidence of salivary gland involvement defined by a positive result for at least one of the following diagnostic tests:<br> 1. Unstimulated whole salivary flow (<1.5 ml in 15 minutes)<br> 2. Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts19<br> 3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer20<br>
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| | VI. Autoantibodies: presence in the serum of the following autoantibodies:<br> 1. Antibodies to Ro(SSA) or La(SSB) antigens, or both<br>
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| |}
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| ''(The above table was adapted from Table 2 in Vitali C, et al. ''[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1754137/pdf/v061p00554.pdf ''Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group'']''. Ann Rheum Dis 2002;61:554-558. See references below.'')<br>
| | There are also several things a patient can do at home in order to manage his or her symptoms. These may include<ref name="NIAMS" />: |
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| | *Over the counter eye drops |
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| | *Chewing gum or sucking on hard candy to increase oral secretions |
| | '''Revised rules for classification'''<ref name="Criteria" /><br>
| | *Drinking water to relieve a dry mouth |
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| | *Using lip balm to moisten the lips |
| | ''For primary SS''<br>In patients without any potentially associated disease, primary SS may be defined as follows:<br> a. The presence of any 4 of the 6 items is indicative of primary SS, as long as either item IV (Histopathology) or VI (Serology) is positive<br> b. The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI)<br> c. The classification tree procedure represents a valid alternative method for classification, although it should be more properly used in clinical-epidemiological survey<br>
| | *Practice thorough oral hygiene to avoid oral infection |
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| | *Heavy moisturizing creams or ointment for dry skin |
| | ''For secondary SS''<br>In patients with a potentially associated disease (for instance, another well defined connective tissue disease), the presence of item I or item II plus any 2 from among items III, IV, and V may be considered as indicative of secondary SS<br>
| | *Humidifier to hydrate the skin and moisten the air for breathing<ref name=":1" /> |
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| | ''Exclusion Criteria'':<br>Past head and neck radiation treatment<br>Hepatitis C infection<br>Acquired immunodeficiency disease (AIDS)<br>Pre-existing lymphoma<br>Sarcoidosis<br>Graft versus host disease<br>Use of anticholinergic drugs (since a time shorter than 4-fold the half life of the drug)<br>
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| |}
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| ''(The above table was adapted from Table 3 in Vitali C, et al. ''[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1754137/pdf/v061p00554.pdf ''Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group'']''. Ann Rheum Dis 2002;61:554-558. See references below.'')<br>
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| == Etiology/Causes ==
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| The primary symptoms of Sjogren's syndrome, including the dry [[Image:Acinar cells.JPG|thumb|right|300px|Digital camera shot of human acinar cells through a microscope.]]eyes and dry mouths, are a result of the destruction of the exocrine (lacrimal and salivary) glands by focal T-lymphocytic infiltrates<ref name="Pathology">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. Denver, CO: Saunders, 2009.</ref>. T-cells and B-cells are components of the immune system and usually work together to detroy foreign structures in the body, such as viruses and bacterias. However, in autoimmune diseases, such as Sjogren's syndrome, these cells interfere with the function and structure of organs within the body. In the case of Sjogren's, these cells interfere with glandular function<ref name="Pathology" />.<br>
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| Regulation of the salivary glands by the nervous system is impaired, and the secretory acinar apparatus displays structural abnormalities<ref name="Pathology" />. The acinus is the terminal end of the exocrine gland where the secretions are produced. The membrane of this structure lacks the laminin alpha-1 chain, which may explain this organ's inability to induce differentiation of the stem cells into acinar cells<ref name="Pathology" />.
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| Evidence in the pattern of cases suggest that the cause of Sjogren's syndrome has a genetic component<ref name="Pathology" />. Recent research has found that the same gene variant commonly present in individuals with other connective tissue diseases, such as RA and lupus is also associated with Sjogren's syndrome<ref name="NIAMS" />. The discovery of the STAT4 gene variant in association with autoimmune diseases could lead to a number of cutting edge treatments<ref name="NIAMS" />, including the possiblitly of gene replacement therapy to combat these incurable conditions.
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| As with other autoimmune diseases, scientist believe that Sjogren's may be triggered by an infection in individuals with a genetic predisposition<ref name="NIAMS" />. This infection jump starts the the immune system. However instead of calming down when the infection is eraticated, the immune system continues to remain on high alert, and starts to destroy the body's own cells.
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| == Systemic Involvement ==
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| Individuals with Sjogren's syndrome are prone to a number of problems such as inflammation and infection throughout the body. These issues may include:
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| [[Image:Sjogrenssymptoms.png|right|300px]]
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| '''Pulmonary Problems'''
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| *bronchitis | |
| *tracheobronchitis
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| *lymphocytic interstitial pneumonitis
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| *pleurisy
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| '''Kidney Problems'''
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| *interstitial nephritis
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| *glomerulonephritis (rarely)
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| *renal tubular acidosis
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| *hypokalemia
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| '''Nervous System Problems'''
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| *peripheral neuropathy
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| *cranial neuropathy
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| '''Liver Problems'''
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| *hepatitis
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| *cirrhosis
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| '''Thyroid Problems'''
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| *Grave's disease
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| *Hashimoto's thyroiditis
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| '''Vascular Problems:'''
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| *vasculitis
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| *Raynaud's phenomenon
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| == Medical Management (current best evidence) ==
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| Common medical practice revolves around medication to treat Sjogren's syndrome. Specifically, physicians aim to control the dry eyes using substitue topical agents and manage extraglandular symptoms with glucocorticoids and immunosuppressive drugs<ref name="Treatment">Ramos-Casals M, Tzioufas A, Stone J, Siso A, Bosch X. Treatment of primary sjogren syndrome: a systemic review. JAMA 2010;304:452-460. http://jama.ama-assn.org/content/304/4/452.long (accessed 17 March 2011).</ref>. Many of these pharmaceutical therapies can be seen in the [http://www.physio-pedia.com/index.php5?title=Sjogren%27s_Syndrome#Medications Medications] section of this page.<br>
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| Current evidence does support frequently using preservative free tear substitutes to treat dry eyes during the day and using the ocular lubricating ointment at night<ref name="Treatment" />. The ointments are not suggested for day time use secondary to the application producing blurred and cloudy vision. The treatment recommended to a patient may depend upon the severity of the patient symptoms. To treat moderate to severe dry eyes associated with Sjogren's syndrome, controlled trials support the application of topical 0.05% [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000253/ cyclosporine] two times a day<ref name="Treatment" />. Topical NSAIDs or glucocorticosteroids may be required for the patients suffering from severe refractory ocular dryness<ref name="Treatment" />. In extreme cases of severe dry eyes, a patient my elect to undergo a punctual occlusion. This is a surgical percedual attempts to keep the natural tears over the surface of the eye by closing the tear ducts that drain said tears from the eye<ref name="NIAMS" />.<br>
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| For patients experiencing mild to moderate dry mouth, studies support the use of saliva replacement products and/ or sugar free gum<ref name="Treatment" />. Along with the use of such products, it is suggested that individuals practice thorough oral hygiene, while avoiding smoking and alcohol consumption<ref name="Treatment" /><ref name="NIAMS" />. Oral [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000465/ pilocarpine] and [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000452/ cevimeline] are the preferred treatments for patients who have maintained residual salivary gland function, however these two drugs have not yet been tested in a comparison study to determine which is more effective<ref name="Treatment" />. Recommended daily doses are as follows:<br>
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| *Pilocarpine: 5 mg every 6 hours
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| *Cevimeline: 30 mg every 8 hours
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| If a patient presents with contraindications for these therapies or is not tolerant of these medications, it has been found that N-aceylcysteine is an acceptable alternative<ref name="Treatment" />.
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| The studies supporting the use of glucocorticoids and immunosuppressants have been small providing limited evidence for the prescription of such therapies. However, [http://www.nlm.nih.gov/medlineplus/druginfo/meds/a607038.html Rituximab] has been shown to improve some extraglandular symptoms, including vasculitis, neuropathy, [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001515/ glomerulonephritis], and arthritis<ref name="Treatment" />.
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| == Physical Therapy Management (current best evidence) ==
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| Individuals with Sjogren's syndrome have reduced physical capacity and tend to fatigue very easily. While research on the effects of exercise on individuals diagnosed with Sjogren's is limited, the available studies suggest these patients benefit from moderateto high-intensity levels of exercise<ref name="Pathology" />. This type of activity has a positive influence on aerobic capacity, fatigue, physical function, and mood.<br>
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| == Alternative/Holistic Management (current best evidence) ==
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| Researchers have come to notice a decreased incidence of Sjogren's syndrome in some Asian countries, such as Japan and China. Therefore, green tea polyphenols (GTPs) are being studied as possible components of treatment<ref name="Pathology" />. GTPs have anti-inflammatory and antiapoptotic properties, making it a promising alternative to the medications currently used to treat this autoimmune disease<ref name="Pathology" />. Animal studies demonstate that GTPs may protect the body against autoimmune attacks specifically to the skin and salivary glands<ref name="Pathology" />.<br>
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| == Differential Diagnosis ==
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| There are several diseases that present with symptoms similar to Sjogren's syndrome, including the dry eyes and dry mouth<sup>3</sup><sup></sup>. Some differential diagnoses include:<br>
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| *[http://www.physio-pedia.com/index.php5?title=Systemic_Lupus_Erythematosus Systemic Lupus Erythematosus]<br>
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| *[http://www.mayoclinic.com/health/vasculitis/DS00513 Vasculitis]
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| *[http://www.nlm.nih.gov/medlineplus/thyroiddiseases.html Thyroid disease]
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| *[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/ Scleroderma]
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| *Medication side-effects
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| <br>
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| == Case Reports/ Case Studies<br> ==
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| Szturmowicz M, Wilinska E, Paczek A, et al. [http://docs.google.com/viewer?url=http%3A%2F%2Fwww.pneumonologia.viamedica.pl%2Fdarmowy_pdf.phtml%3Findeks%3D55%26indeks_art%3D644 Primary Sjogren's syndrome with two extraglandular sites involvement-case report.] ''Pneumonol. Alergol. Pol''. 2010:78;445-450.
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| == Resources <br> ==
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| [http://www.niams.nih.gov/Health_Info/Sjogrens_Syndrome/default.asp National Institute of Arthritis and Musculoskeletal and Skin Disease]<br>
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| [http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp American College of Rheumatology]
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| [http://www.sjogrens.org/ Sjogren's Syndrome Foundation]
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| [http://www.nei.nih.gov National Eye Institute]
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| [http://www.aao.org American Academy of Ophthalmology]
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| [http://www.aarda.org American Autoimmune-Related Diseases Associated, Inc. (AARDA)]
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| [http://www.arthritis.org Arthritis Foundation]
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| [http://www.rarediseases.org National Organization for Rare Disorders (NORD)]
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| == Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed]) <br> ==
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| <rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1jeAVjyuKpXiEUVu0BHJQBuRC5NMeqm6NxzqGzoerHIyvWUYT0|charset=UTF-8|short|max=15</rss>
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| == References == | | == References == |
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| see [[Adding References|adding references tutorial]].
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| <references /> | | <references /> |
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| [[Category:Bellarmine_Student_Project]] | | [[Category:Bellarmine_Student_Project]] |
| | [[Category:Conditions]] |
| | [[Category:Autoimmune Disorders]] |
| | [[Category:Genetic Disorders]] |
| | [[Category:Rheumatology]] |
