Menu
eMediHealth Logo
Subscribe
No Result
View All Result
eMediHealth Logo
Subscribe
No Result
View All Result

Home > Allergy & Immunology > What Is Sjogren’s Syndrome and How Is It Diagnosed?

What Is Sjogren’s Syndrome and How Is It Diagnosed?

July 6, 2020 - Updated on August 2, 2021
7 min read
By David Lans, DO, FACP | Rheumatologist

In this article:

  • Body Parts Affected by Sjogren’s Syndrome
  • Prevalence of Sjogren’s Syndrome
  • Symptoms of Sjogren’s Syndrome
  • Diagnosis of Sjogren’s Syndrome
  • Conditions That Mimic Sjogren’s Syndrome
  • Final Word

Sjogren’s syndrome is a multisystem, chronic autoimmune disease of unknown cause.

sjogren's syndrome

It frequently occurs in conjunction with other autoimmune syndromes, such as lupus or rheumatoid arthritis, in which case, it is often termed secondary Sjogren’s syndrome.

When presenting as a distinct illness, it is sometimes called Sjogren’s disease or primary Sjogren’s syndrome.

Body Parts Affected by Sjogren’s Syndrome

Sjogren’s syndrome may affect many body systems, including the lungs and respiratory system, gastrointestinal system, joints, and nervous system.

Advertisements

Sjogren’s syndrome often emerges insidiously. It develops gradually with a host of seemingly disparate symptoms that may not be recognized as representatives of a single syndrome.

As such, the diagnosis is often delayed and even missed by physicians.

Prevalence of Sjogren’s Syndrome

Sjogren’s syndrome is not a common disorder, as evidenced by the following facts:

  • The prevalence of this condition is estimated to be 40–90 cases per 100,000 population. (1)
  • Women are affected more often than men by a factor of 7–20. It most commonly manifests in women in their 50s and 60s. (2)
  • Complaints of dry eye and mouth are quite common, especially in older individuals, where up to 30% of the elderly may experience sicca symptoms. (3)

Because most cases are due to other causes, Sjogren’s syndrome is often not recognized as a more serious explanation for dry eye and mouth symptoms.

Symptoms of Sjogren’s Syndrome

signs and symptoms of sjogren's syndrome

The hallmark of the condition is inflammation of the secretory glands, such as those responsible for saliva, perspiration, and tears. As a result, dryness of the eyes and mouth (sicca syndrome) along with dry and itchy skin is a common prominent symptom.

One of the reasons that the diagnosis of Sjogren’s syndrome is so challenging is that the symptoms and severity of the condition are so variable.

Advertisements
  • Some patients experience mild ocular dryness and fatigue with aches and pains.
  • Others may have disabling arthritis or respiratory, nervous, or intestinal system involvement.
  • In some instances, patients become seriously ill, with compromising lung disease, severe neuropathy, and even non-Hodgkin’s lymphoma.

Other common symptoms include:

  • Fatigue and low energy
  • Joint pains
  • Weakness
  • Dry cough
  • Corneal injury and keratoconjunctivitis sicca, often affecting vision
  • Swollen lymph nodes
  • Anemia and low white blood cell count
  • Salivary gland swelling, often with pain and tenderness
  • Indigestion, irritable bowel, difficulty swallowing, esophageal reflux, and other gastrointestinal problems
  • Memory loss and brain fog
  • Burning or tingling pains in the extremities
  • Dry, itchy skin
  • Vaginal dryness with resulting urinary and sexual difficulties
  • Interstitial cystitis
  • Liver problems, which range from mild liver test abnormalities to chronic autoimmune hepatitis
  • Dental problems, such as tooth decay and periodontitis, due to loss of saliva
  • Loss of sense of smell and taste
  • Swollen glands, both lymph nodes, and salivary glands
  • An increased risk of lymphoma

Diagnosis of Sjogren’s Syndrome

diagnosis of sjogren's syndrome

Most patients with mild to moderate Sjogren’s syndrome have prominent sicca symptoms. Since dryness of the eyes and/or mouth is common, clinicians may fail to recognize that the symptoms represent a systemic and inflammatory disorder.

Instead, the symptoms may be attributed to aging or a side effect of medication prescribed for other reasons. Hence, a delay in diagnosis is a common problem for sufferers of Sjogren’s syndrome. (3)

The biggest challenge in diagnosing Sjogren’s syndrome is simply considering the diagnosis! The condition mimics many other disorders, such as fibromyalgia, chronic fatigue syndrome, symptomatic menopause, and adverse drug reactions.

Moreover, sicca symptoms of dry eyes and mouth are common complaints in older people and have many causes other than Sjogren’s. Consequently, doctors may not recognize the systemic and inflammatory process and may attribute the patient’s complaints to other causes.

But once the doctor considers the possibility of Sjogren’s syndrome after excluding other causes for the sicca symptoms, it can easily be diagnosed based on clinical features, blood tests, and in some cases, a biopsy of the salivary glands.

Advertisements

The diagnostic tests for Sjogren’s syndrome include:

1. Blood tests

Blood tests often reveal the presence of various autoantibodies, but these antibodies are not restricted to Sjogren’s syndrome alone and can be indicative of other problems, such as systemic lupus. (4)

Therefore, while the antibody tests are quite useful in investigating Sjogren’s syndrome, they cannot be depended upon solely for the diagnosis. Other useful but non-specific blood tests include markers of inflammation, such as ESR and C-reactive protein.

2. Eye tests

When complaints of dry eyes are considered, the Schirmer test is used to measure tear production. Ocular surface staining tests may be used to assess any damage to the corneal tissue. Usually, these tests are performed by ophthalmologists.

3. Salivary function tests

The salivary glands can also be tested to establish glandular hypofunction.

4. Imaging tests

Ultrasonography and nuclear imaging studies (technetium scanning) may be helpful. An MRI of the parotid glands (the large salivary glands located under each cheek, in front of the ear) can detect structural abnormalities. 

Advertisements

5. Biopsy

Biopsy of a salivary gland may be employed to detect the presence of inflammation – infiltration of the tissue with lymphocytes. This test is positive in about 80% of individuals with Sjogren’s syndrome. (5)

However, this test can be quite painful, result in persistent lip numbness, and even produce a false negative in some cases. Hence, it is not performed unless absolutely necessary.

For instance, 20%–30% of individuals with Sjogren’s syndrome do not possess autoantibodies and therefore require a biopsy to confirm the illness. (6)

Advertisements

Conditions That Mimic Sjogren’s Syndrome

other conditions often confused with sjogren's syndrome

A number of similar conditions are often confused with Sjogren’s syndrome, mostly due to the symptoms of sicca or glandular swelling. It is important to consider these possibilities, as well.

1. Age-related sicca syndrome

Ocular and oral dryness is frequent among those over age 65 years, but without any associated systemic symptoms, autoantibodies, and markers of inflammation.

2. Sarcoidosis

This is an immunological disorder that often presents with sicca symptoms and swelling of the salivary and tear glands, as well as other systemic symptoms, such as fatigue, joint pains, and respiratory problems, so there may be considerable overlap.

3. IgG4-related disease

This is a rare inflammatory condition that affects the secretory glands along with many other body systems and can appear much like Sjogren’s syndrome, but the laboratory and biopsy features are substantially different.

4. Lymphoma

Cancer of the lymphatic system and leukemias affect the salivary glands and can present with swelling due to tumor infiltration of the glands.

5. Sialadenitis

This is an infection of the salivary glands that can trigger sicca symptoms. It is common in people with diabetes or a blocked salivary duct due to a stone or some other reason.

6. Hepatitis C and HIV

These conditions occasionally affect the salivary glands and present with swelling and sicca symptoms.

It is also important to recognize the presence of secondary Sjogren’s syndrome in patients with other rheumatic disorders, such as lupus, rheumatoid arthritis, and psoriatic arthritis.

This is important because some of the treatment options differ, and patients will benefit when an associated Sjogren’s disease is recognized and addressed.

Final Word

Sjogren’s syndrome is a challenging problem that is associated with a significant reduction in the quality of life. (7)

Fortunately, for most sufferers, the mortality rate is not much different from that of the general population, and there are methods to manage the condition and treat symptoms fairly effectively. However, there is increased mortality in patients with severe conditions, such as those with interstitial lung disease, kidney disease, or lymphoma.

Continue Reading Continue ReadingSjogren’s Syndrome Treatment and Home Remedies
References
  1. Maciel G, Crowson CS, Matteson EL, Cornec D. Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome: Time Trends Over a 40-Year Period in a Population-Based US Cohort. Mayo Clinic proceedings. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5470777/. Published May 2017.
  2. Stefanski A-L, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T. The Diagnosis and Treatment of Sjögren’s Syndrome. Deutsches Arzteblatt international. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471601/. Published May 26, 2017.
  3. Baer AN, Walitt B. Sjögren Syndrome and Other Causes of Sicca in Older Adults. Clinics in geriatric medicine. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5125547/. Published February 2017.
  4. Yoshimi R, Ueda A, Ozato K, Ishigatsubo Y. Clinical and pathological roles of Ro/SSA autoantibody system. Clinical & developmental immunology. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3523155/. Published 2012.
  5. Selifanova E, Beketova T, Spagnuolo G, Leuci S, Turkina A. A Novel Proposal of Salivary Lymphocyte Detection and Phenotyping in Patients Affected by Sjogren’s Syndrome. Journal of clinical medicine. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7074317/. Published February 14, 2020.
  6. Fayyaz A, Kurien BT, Scofield RH. Autoantibodies in Sjögren’s Syndrome. Rheumatic diseases clinics of North America. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4955792/. Published August 2016.
  7. Vivino FB, Carsons SE, Foulks G, et al. New Treatment Guidelines for Sjögren’s Disease. Rheumatic diseases clinics of North America. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5812283/. Published August 2016.
  • Was this article helpful?
  • YES, THANKS!NOT REALLY
Spread the Love❤️
Advertisements
Advertisements
Advertisements
Advertisements

Newsletter

Get our BEST updates delivered to your inbox:



This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.

Categories

  • Nutrition
  • Wellness
  • Oral Care
  • Eye Care
  • Heart Health
  • Skin & Beauty
  • Women’s Health
  • Ear, Nose & Throat
  • Respiratory Health

Links

  • Home
  • About Us
  • Contact
  • Privacy Policy
  • Terms and Conditions
  • Cookie Policy
  • Editorial Policy
  • Advertising Policy
  • Subscribe to Newsletter
eMediHealth

This site provides content for informational purposes only. The information provided is not intended for use as medical advice, diagnosis, or treatment. In case of a medical concern or emergency, please consult your healthcare provider.

This website is certified by Health On the Net Foundation.

Our Network: Little Extra

© 2019 eMediHealth. All rights reserved.

No Result
View All Result
  • Nutrition
  • Skin & Beauty
  • Wellness
  • Yoga & Meditation
  • Women’s Health
  • Heart Health
  • Allergy & Immunology
  • Bones & Joints
  • Child Health
  • Digestive
  • Ear, Nose & Throat
  • Eye Care
  • Glands & Hormones
  • Health News
  • Infectious Diseases
  • Mental Health
  • Nervous System
  • Oral Care
  • Pain Management
  • Respiratory Health
  • Sleep Disorders
  • Urological Health

© 2019 eMediHealth. All rights reserved.