Sjogren's Syndrome: Difference between revisions

No edit summary
m (Added a category.)
 
(70 intermediate revisions by 7 users not shown)
Line 1: Line 1:
 
<div class="editorbox"> '''Original Editor '''- [[User:Cassie Shay|Cassie Shay]] [[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;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}}}} &nbsp;  
'''Original Editors '''- [http://www.physio-pedia.com/index.php5?title=User:Cassie_Shay Cassie Shay] [[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  
 
'''Lead Editors''' - Your name will be added here if you are a lead editor on this page.&nbsp; [[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>.
 
&nbsp;{{#ev:youtube|-PIcNX5SJ0s|400|right}}
 
== Prevalence  ==
 
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>.
 
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>.
 
== Characteristics/Clinical Presentation  ==
 
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. Individuals with this condition may describe their symptoms as they relate to their eyes as an irritation, burning, or gritty feeling, as though they have sand in their eyes<sup>1,3</sup>. The dryness in their mouth may make it difficult to speak, swallow,chew, or it may make foods taste differently<sup>3</sup>.
 
While these are the hallmark signs of Sjogren's syndrome, 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<sup>1,2</sup>. Females diagnosed with Sjogren's may also experience vaginal dryness and pain with intercourse<sup>2</sup>.<br>
 
== Associated Co-morbidities  ==
 
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 experience Sjogren's in association with lymphoma<ref name="American College of Rheumatology" />.&nbsp; 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.
 
{| cellspacing="1" cellpadding="1" border="1" style="width: 613px; height: 129px;"
|-
| Disease  
| Description
|-
| [http://www.ninds.nih.gov/disorders/polymyositis/polymyositis.htm Polymyositis]
| 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].
|-
| [http://www.physio-pedia.com/index.php5?title=Rheumatoid_Arthritis Rheumatoid Arthritis]  
| Severe inflammation of the joints caused by the bodies own immune system attacking the synovium located throughout the body.
|-
| [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/ Scleroderma]  
| Accumulation of too much collagen, resulting in thick, tight skin, and possible damage to joints, muscles, and internal organs.
|-
| [http://www.physio-pedia.com/index.php5?title=Systemic_Lupus_Erythematosus Systemic Lupus Erythematosus]  
| 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.
|-
| [http://www.physio-pedia.com/index.php5?title=Non_Hodgkin_Lymphoma Lymphoma]
| 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.
|}
 
== Medications  ==
 
There is no cure for Sjogren's syndrome, therefore its symptoms are managed through medications, both prescription and over the counter. A variety of&nbsp; medications may be used to treat each of the associated symptoms.<br>
 
{| cellspacing="1" cellpadding="1" border="1" style="width: 705px; height: 140px;"
|-
| '''Drug category '''<br>  
| style="vertical-align: top;" | '''Signs/ Symptoms Treated'''
| '''Examples of Brand Names'''
|-
| Nonsteroidal anti-inflammatory drugs<br>
| style="vertical-align: top;" | Joint and Muscle Pain
| [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000598/ Ibuprofen ]<br>
|-
| Corticosteriods<br>
| style="vertical-align: top;" | Inflammation of lungs, kidneys, blood vessels, or nervous system
| [http://www.nlm.nih.gov/medlineplus/druginfo/meds/a601102.html Prednisone]<br>
|-
| Immune-modifying drugs<br>
| style="vertical-align: top;" | Overactivity of the immune system
| Plaquenil ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000171/ hydroxychloroquine]),&nbsp;Rheumatrex ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000547/ methotrexate]), Cytoxan ([http://www.nlm.nih.gov/medlineplus/druginfo/meds/a682080.html cyclophosphamide])<br>
|-
| Artificial Tears<br>
| valign="middle" align="left" style="vertical-align: top;" rowspan="4" | Dry Eyes
| Clear Eyes, Visine Tears, etc.<br>
|-
| style="vertical-align: top;" | Ointments (for night use)<br>
| style="vertical-align: top;" | GenTeal, Systane<br>
|-
| style="vertical-align: top;" | Hydroxypropyl methylcellulose<br>
| style="vertical-align: top;" | Ocucoat<br>
|-
| style="vertical-align: top;" | Topical steroids<br>
| style="vertical-align: top;" | Restasis ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000253/ cylcosporin A])<br>
|-
| style="vertical-align: top;" | Saliva substitue<br>
| style="vertical-align: top;" rowspan="2" | Dry Mouth
| style="vertical-align: top;" | Oralube<br>
|-
| style="vertical-align: top;" | Cholinergic Agonists<br>
| 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>
|}
 
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.


== Diagnostic Tests/Lab Tests/Lab Values&nbsp;<br> ==
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" />.


Diagnosis of this disease is based on a medical history, a physical exam, and results from some clinical and laboratory tests<sup>1,2,3</sup>. 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<sup>2</sup>. Nonetheless, diagnostic testing may include the following:<br>
== Clinical Presentation ==
This 90 second video takes a look at this syndrome.&nbsp;{{#ev:youtube|-PIcNX5SJ0s|400|right}}The more common symptoms of Sjogren’s syndrome include:  


*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 pro[[Image:Lip Biopsy.jpg|thumb|right|300px|Histopathologic image of a lip biopsy demonstrating lymphoid infiltration in a minor salivary gland. H&E stain]]duce too few tears to get more than 8 millimeters of the paper wet<sup>2</sup>.
* dry eyes (irritation, feeling gritty or itchy, burning)
*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<sup>2</sup>.
* dry mouth (or difficulty chewing or swallowing).
*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<sup>2</sup>.&nbsp;
[[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:
*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.&nbsp;


== Etiology/Causes&nbsp;  ==
* 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.


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>
Complications can include:  


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" />.
* increased risk of dental decay
* increased risk of developing thrush infections in the mouth
* vision problems
* inflammation of internal organs (such as the kidneys, lungs or liver)
* problems with the circulatory and nervous system.<ref name=":1" />


Evidence in the pattern of cases suggest that the cause of Sjogren's syndrome has a genetic component. However, research has not been able to identify specific gene or set of genes responsible for the symptoms<ref name="Pathology" />.
== 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>


== Systemic Involvement  ==
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).


Individuals with Sjogren's syndrome are prone to a number of problems such as inflammation and infection throughout the body. These issues may include:  
== Treatment ==
There is no cure for Sjogren's syndrome, therefore its symptoms are managed through medications, both prescription and over the counter. A variety of&nbsp; medications may be used to treat each of the associated symptoms. Treatment may include:  


[[Image:Sjogrenssymptoms.png|right|300px]]
* 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" />.


'''Pulmonary Problems'''
== Diagnostic Tests ==


*bronchitis
*tracheobronchitis
*lymphocytic interstitial pneumonitis
*pleurisy


'''Kidney Problems'''
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:
* Schirmer’s test – special blotting paper held to the eye is used to assess the degree of tear production
* eye examination – including the use of special dyes
* 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" />.


*interstitial nephritis
== Physical Therapy Management ==
*glomerulonephritis (rarely)
*renal tubular acidosis
*hypokalemia


'''Nervous System Problems'''
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.


*peripheral neuropathy
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>.
*cranial neuropathy


'''Liver Problems'''
== Lifestyle Changes  ==


*hepatitis
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" />:
*cirrhosis


'''Thyroid Problems'''
*Over the counter eye drops
 
*Chewing gum or sucking on hard candy to increase oral secretions
*Grave's disease
*Drinking water to relieve a dry mouth  
*Hashimoto's thyroiditis
*Using lip balm to moisten the lips
 
*Practice thorough oral hygiene to avoid oral infection
'''Vascular Problems:'''
*Heavy moisturizing creams or ointment for dry skin
 
*Humidifier to hydrate the skin and moisten the air for breathing<ref name=":1" />
*vasculitis
*Raynaud's phenomenon&nbsp;
 
== Medical Management (current best evidence)  ==
 
add text here
 
== Physical Therapy Management (current best evidence)  ==
 
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<sup>3</sup>. This type of activity has a positive influence on aerobic capacity, fatigue, physical function, and mood.<br>
 
== Alternative/Holistic Management (current best evidence)  ==
 
add text here
 
== Differential Diagnosis  ==
 
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>
 
*[http://www.physio-pedia.com/index.php5?title=Systemic_Lupus_Erythematosus Systemic Lupus Erythematosus]<br>
*[http://www.mayoclinic.com/health/vasculitis/DS00513 Vasculitis]
*[http://www.nlm.nih.gov/medlineplus/thyroiddiseases.html Thyroid disease]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/ Scleroderma]
*Medication side-effects
 
<br>
 
== Case Reports/ Case Studies  ==
 
add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
 
Szturmowicz M, Wilinska E, Paczek A, et al. Primary Sjogren's syndrome with two extraglandular sites involvement-case report. ''Pneumonol. Alergol. Pol''. 2010:78;445-450.
 
== Resources <br>  ==
 
[http://www.niams.nih.gov/Health_Info/Sjogrens_Syndrome/default.asp National Institute of Arthritis and Musculoskeletal and Skin Disease]<br>
 
[http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp American College of Rheumatology]
 
[http://www.sjogrens.org/ Sjogren's Syndrome Foundation]
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed]) <br>  ==
 
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1jeAVjyuKpXiEUVu0BHJQBuRC5NMeqm6NxzqGzoerHIyvWUYT0|charset=UTF-8|short|max=15</rss>  


== References  ==
== References  ==
see [[Adding References|adding references tutorial]].


<references />  
<references />  


[[Category:Bellarmine_Student_Project]]
[[Category:Bellarmine_Student_Project]]
[[Category:Conditions]]
[[Category:Autoimmune Disorders]]
[[Category:Genetic Disorders]]
[[Category:Rheumatology]]

Latest revision as of 21:04, 1 September 2023

Introduction[edit | edit source]

Signs and symptoms: SS

Sjögren's syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It is a relatively common condition that mainly affects the eyes and salivary glands, but can affect different parts of the body.[1][2].

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.[3]

Epidemiology[edit | edit source]

SS is the second most prevalent autoimmune rheumatic disease. In 2008, it was estimated that 1.3 million Americans were affected[2][4] 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.[1]

Etiology[edit | edit source]

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[3].

Clinical Presentation[edit | edit source]

This 90 second video takes a look at this syndrome. 

The more common symptoms of Sjogren’s syndrome include:

  • dry eyes (irritation, feeling gritty or itchy, burning)
  • dry mouth (or difficulty chewing or swallowing).
Symptoms: Sjogren’s

Sjogren’s syndrome is a systemic condition, which means it can affect the entire body, other symptoms can include:

  • 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.

Complications can include:

  • increased risk of dental decay
  • increased risk of developing thrush infections in the mouth
  • vision problems
  • inflammation of internal organs (such as the kidneys, lungs or liver)
  • problems with the circulatory and nervous system.[3]

Associated Co-morbidities[edit | edit source]

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[2]. Common primary diseases associated with Sjogren's Syndrome include: Polymyositis; Rheumatoid Arthritis; Scleroderma; Systemic Lupus Erythematosus; Lymphoma.[5]

Image R: The 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).

Treatment[edit | edit source]

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:

  • 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[3].

Diagnostic Tests[edit | edit source]

Diagnosis of this disease is based on medical history, a physical exam, and results from some clinical and laboratory tests[5][2][4]. 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[2]. Nonetheless, diagnostic testing may include the following:

  • Schirmer’s test – special blotting paper held to the eye is used to assess the degree of tear production
  • eye examination – including the use of special dyes
  • 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[3].

Physical Therapy Management[edit | edit source]

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[4]. This type of activity has a positive influence on aerobic capacity, fatigue, physical function, and mood.

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[4]. 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[6].

Lifestyle Changes[edit | edit source]

There are also several things a patient can do at home in order to manage his or her symptoms. These may include[2]:

  • Over the counter eye drops
  • Chewing gum or sucking on hard candy to increase oral secretions
  • Drinking water to relieve a dry mouth
  • Using lip balm to moisten the lips
  • Practice thorough oral hygiene to avoid oral infection
  • Heavy moisturizing creams or ointment for dry skin
  • Humidifier to hydrate the skin and moisten the air for breathing[3]

References[edit | edit source]

  1. 1.0 1.1 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/(accessed 6/3/2022)
  2. 2.0 2.1 2.2 2.3 2.4 2.5 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).
  3. 3.0 3.1 3.2 3.3 3.4 3.5 Better Health Sjogren's Syndrome Available: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/sjogrens-syndrome#self-management-of-sjogren%E2%80%99s-syndrome(accessed 6.3.2022)
  4. 4.0 4.1 4.2 4.3 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. Denver, CO: Saunders, 2009.
  5. 5.0 5.1 American College of Rheumatology. Sjogren's Syndrome. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp (accessed 17 March 2011).
  6. Medline Plus. Sjogren Syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/000456.htm (accessed 6 April 2011)