Lupus and The Exocrine Gland System: Sjögren’s Disease [Mar ’25 Update]

posted in All on November 22, 2024 by

Gavin Abson

Updated April 25, 2025

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Lupus patients can have dry mouth, eyes, and skin. Most of the time, this is due to having an additional systemic autoimmune disease called Sjogren’s disease. These symptoms can lead to discomfort, infections, vision problems, and poor quality of life. Moisture-producing glands are called exocrine glands, forming the exocrine system.

NOTE: Johns Hopkins University Press, publisher of The Lupus Encyclopedia, is a nonprofit publisher. If you purchase JHUP books, like The Lupus Encyclopedia, you support projects like Project MUSE.

Dryness problems are common with lupus, yet many patients fail to discuss them with their doctors. This blog article outlines how and why dryness occurs and what lupus patients can do to help themselves.

To find out the difference between systemic lupus erythematosus and Sjogren’s disease, click here: lupusencyclopedia.com/lupus-and-the-exocrine-gland-system/#vs

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NOTE: “The Lupus Encyclopedia” is published by Johns Hopkins Press, a non-profit publishing house whose proceeds go towards important endeavors like the “Muse Project.” Thank you for your support.

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This blog post article was edited and contributed to by Donald Thomas, MD; author of “The Lupus Encyclopedia.” Parts of this blog post come from “The Lupus Encyclopedia: A Comprehensive Guide for Patients and Health Care Providers, edition 2“

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What is the Exocrine Gland System?

As explained in The Lupus Encyclopedia:

“The two large groups of body glands are endocrine glands and exocrine glands. Endocrine glands (endo-­ from the Greek for “inside” and -­ crine is used in medicine meaning “to secrete”) produce substances that are secreted into the blood. Examples include the thyroid (secretes thyroid hormones), adrenal glands (secrete cortisol and other substances), and the ovaries (secrete female hormones). Endocrine involvement by SLE is discussed in chapter 17.

“The exocrine glands (exo-­ meaning “outside”) secrete fluids outside the body (such as outside the skin or into the gastrointestinal tract, which is a tube from the mouth to the anus, and technically outside the body). The main autoimmune prob­lem with exocrine glands is Sjögren’s disease, which is the subject of this chapter.”

The Lupus Encyclopedia (2nd ed.), p. 302

Sjögren’s Disease and the Exocrine Gland System

Sjögren’s disease is an autoimmune condition that primarily impacts the exocrine glands responsible for moisture production.. Sjögren’s disease specifically targets glands that produce moisture, such as the salivary and tear glands. Sjögren’s disease most commonly causes xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). These symptoms can lead to discomfort, difficulty swallowing, dental problems, and even vision problems if untreated. Around 25-30% of systemic lupus erythematosus (SLE) patients will also have Sjögren’s.

Lupus and the Exocrine Gland System

Sjögren’s disease causes exocrine gland inflammation, resulting in decreased fluid secretion. For example, lupus can affect the salivary glands, reducing saliva production, which can cause dry mouth, trouble swallowing, and a higher risk of cavities and infections. Similarly, lupus can affect tear glands, leading to less tear production and dry eyes. In severe cases, the damage to these glands can result in permanent dryness and need continuous moisturizing treatment.

Many lupus patients are often unaware that they may also have Sjögren’s disease as these symptoms occur slowly and seem to be unrelated to their lupus. Many, to most, patients do not report these problems to their rheumatologist, not realizing it could be related to their lupus. Patients should inform their doctors about dryness problems while they are mild and most treatable.

Sjögren’s Disease vs SLE: How to Tell the Difference Between Them:

Sjögren’s Disease Definition:

Sjögren’s disease (SjD) is a systemic autoimmune disease closely related to systemic lupus erythematosus (SLE). However, its primary target is the glands that secrete moisture.

Yet, other organs can be affected (like in lupus), e.g., the lungs in 15-20% of patients, low blood counts, sun-sensitive rashes, arthritis, and even kidney involvement.

It produces autoantibodies such as ANA, rheumatoid factor, anti-SSA, anti-SSB, and anti-centromere. However, other antibodies can also occur, such as anti-RNP and anti-chromatin.

It is a B-cell-driven disease where biopsies of involved areas show many B-lymphocytes causing inflammation.

A diagnosis of SjD can be made upon demonstrating objective evidence of dry eyes and/or dry mouth and a +anti-SSA, +anti-centromere, or a high positive ANA plus a high positive RF. Demonstrating dry eyes and dry mouth in someone who has another autoimmune disease (like SLE or rheumatoid arthritis) is adequate to diagnose Sjögren’s in addition to the other autoimmune diseases.

SLE Definition:

SLE is an autoimmune disease in which the immune system treats various body organs like foreign invaders and attacks them. It produces autoantibodies (especially ANAs) as a feature of the disease.

Many problems set it apart from other autoimmune diseases. These problems can include lupus nephritis, low platelets, autoimmune hemolytic anemia, discoid lupus, and a butterfly (malar) rash.

Some blood tests, such as anti-Smith, anti-ribosomal-P, and BC4d, can help distinguish it from other diseases. We can use classification criteria to help guide the diagnosis (eg the 2012 SLICC criteria and 2019 ACR/EULAR classification criteria).

However, it is sometimes difficult to tell if someone has SLE or Sjögren’s since they have many immunological similarities.

The Role of Inflammation in Sjögren’s Disease

Inflammation and damage are key mechanisms by which Sjögren’s impacts the exocrine glands. Sjögren’s causes inflammation in the glands, causing them not to work properly. The inflammation damages the glands over time, permanently reducing fluid production.

Sjögren’s can target many other exocrine glands, including skin, sinuses, ear canals, nasal passages, and the pancreas. Sjögren’s can cause exocrine pancreatic insufficiency (EPI) if it affects the pancreas. The pancreas is responsible for producing digestive enzymes that help food digestion. EPI causes difficulty digesting fats, leading to symptoms like diarrhea, weight loss, and malnutrition. Fortunately, there are now treatments available for EPI.

Death of the Term “Secondary Sjögren’s”

When someone has Sjögren’s disease and SLE, it is essential to realize that this person has two systemic autoimmune diseases; both are important to consider. Previously, these patients were labeled as having “SLE with secondary Sjögren’s.” This led most doctors to concentrate on the SLE part while only addressing the dryness problems from the Sjögren’s component.

Recognizing this issue, international experts in 2024 urged a shift in terminology, recommending that doctors use “secondary” with “associated.” when referring to Sjögren’s in conjunction with SLE and other autoimmune diseases. Therefore, someone who has SLE and Sjögren’s should be labeled as having “SLE with associated Sjögren’s,” or “SLE associated with Sjögren’s disease,” or even “Sjögren’s associated with SLE.”

Sjögren’s and Major Organ Involvement

Between 30% and 50%, or potentially more, Sjögren’s patients experience organ involvement beyond the exocrine gland system. For example, 15% – 20% of Sjögren’s patients develop inflammatory lung problems. SLE is more likely to cause pleuritis (pleurisy), inflammation of the lining of the lungs, rather than inflammation of tissues within the lungs. However, Sjögren’s patients are much more likely to have inflammation within the lung tissues (such as interstitial lung disease) than they are to have pleurisy.

Neurology experts suspect that 50% or more of Sjögren’s patients probably have involvement of the nervous system, especially peripheral neuropathies, small fiber neuropathy, and autonomic neuropathy. Postural orthostatic tachycardia syndrome (POTS) is one example of dysautonomia that can occur with Sjögren’s.

Another organ that can be affected by Sjögren’s disease is the kidney, where it more commonly causes interstitial nephritis. Conversely, SLE most commonly affects a different part of the kidney (the glomerulus), leading to lupus glomerulonephritis (also called lupus nephritis).

Doctors should keep these in mind as possible complications in people who have both SLE and Sjögren’s.

Managing Sjögren’s Disease

Treatment involves reducing inflammation with medications and using therapies to increase moisture in the affected areas to help prevent damage and help patients feel better.

Medications

To reduce inflammation, health care professionals prescribe medicines like hydroxychloroquine (Plaquenil), and/or immunosuppressive drugs such as corticosteroids and disease-modifying antirheumatic drugs (DMARDs, like methotrexate). These medications can help prevent damage and improve moisture production.

Lifestyle Changes

Lifestyle modifications are also important. Stay hydrated by drinking an adequate amount of water. Many doctors recommend drinking at least 9 glasses of water daily. Patients should avoid caffeine, alcohol, and smoking, which worsen dryness problems. All Sjögren’s patients should have humidifiers throughout their home, especially in the bedroom. There is mounting evidence that eating an anti-inflammatory diet may help autoimmune diseases.

Regular eye exams and dental check-ups are essential. Early detection of complications can lead to better outcomes and prevent more severe problems from developing. The Sjögren’s Foundation has developed management guidelines to guide doctors into preventing complications from dry eyes (vision problems) and dry mouth (dental cavities and tooth loss).

Managing Dry Mouth

Dry mouth, or xerostomia, can lead to difficulties in speaking, chewing, and swallowing, as well as an increased risk of dental issues and infections, like thrush (called oral candidiasis).

Dry Mouth Management Tips:

• Stay Hydrated: Sip water regularly throughout the day.
• Stimulate Saliva Production: Chew sugar-free gum or suck on sugar-free candies to encourage saliva flow. Ask your doctor to prescribe medications that stimulate saliva flow: cevimeline (Evoxac) and pilocarpine (Salagen)
• Use Saliva Substitutes: Over-the-counter saliva substitutes can provide relief.
• Maintain Oral Hygiene: Brush and floss after meals and snacks. See a dentist at least twice a year. For more practical advice, go to:

lupusencyclopedia.com/prevent-early-tooth-loss-when-you-have-dry-mouth-from-lupus-or-sjogrens-syndrome

For more detailed guidance, refer to the American Dental Association’s guidelines on managing dry mouth. For example, they recommend:

• Brushing and flossing twice daily
• Using high fluoride toothpaste and mouthwashes
• Obtaining fluoride treatments from their dentist
• Applying prescription-strength remineralizing agents. Dr. Thomas recommends that his patients apply a pea-sized amount of Clinpro 5000 or Prevident 5000 Booster Plus to the teeth twice daily after brushing, swirl around the teeth, and spit, but don’t rinse. Most insurance does not cover these treatments. If not, get a GoodRX coupon. One tube generally lasts 6 months or longer.
• Seeing the dentist regularly, 2-4 times yearly for teeth cleaning and evaluation
• Using medications like pilocarpine and cevimeline to increase saliva production
• Using xylitol-containing lozenges to promote saliva production

Managing Dry Eyes

Dry eyes can cause irritation, redness, gritty sensations in the eye, and blurred vision.

Management Tips:

• Use Artificial Tears: Regularly apply over-the-counter lubricating eye drops.
• Control Inflammation: Most patients should take medications to reduce inflammation may be prescribed: cyclosporine (Restasis and Cequa) or lifitigrast (Xiidra)
• Protect Your Eyes: Wear sunglasses to shield your eyes from wind and sun exposure.

For more practical advice on how to help dry eyes, go to:

lupusencyclopedia.com/dry-eyes-and-lupus

The Mayo Clinic provides comprehensive information on the diagnosis and treatment of dry eyes.

Managing Dry Skin

Dry skin can lead to itching, flaking, and increased sensitivity.

Management Tips:

• Moisturize Regularly: Apply fragrance-free moisturizers after bathing and throughout the day.
• Avoid Hot Showers: Use lukewarm water to prevent stripping natural oils from your skin.
• Use Gentle Cleansers: Opt for soap-free cleansers to maintain skin moisture.

For additional advice, visit Itchy, Dry Skin in Lupus and Sjögren’s: What is the Relationship?

Prognosis for Lupus Patients with Exocrine Gland Involvement

The prognosis for lupus patients with exocrine gland involvement varies depending on the extent of glandular damage and the effectiveness of treatment. Many patients can control their symptoms and prevent complications. However, many patients experience persistent symptoms, such as chronic dryness, pain, or digestive issues. These problems lead to reduced quality of life. There are currently (March 2025) no FDA-approved drugs to treat Sjögren’s systemic inflammation. Fortunately, numerous drugs are in clinical trials for Sjögren’s; early data are encouraging.

The Lupus Encyclopedia discusses Sjögren’s disease in detail in chapter 14. With the help of several Sjögren’s experts, Dr. Thomas lists numerous practical tips essential for patients to learn and incorporate into their lives.

The Importance of Regular Monitoring

Dryness problems in lupus patients need to be identified early if they occur. The Lupus Encyclopedia mentions that damage to the exocrine glands usually develops gradually. Regular follow-ups with a rheumatologist, ophthalmologist, and dentist help to ensure an accurate diagnosis and that effective treatments are prescribed.

In addition, Sjögren’s disease often affects other organs. For example, around 15 to 20% of patients have Sjögren’s lung involvement. Sjögren’s attacks the nervous system in around 50% of patients. Regular monitoring by your rheumatologist can help identify these complications early so that effective treatments can be given to prevent irreversible damage.

Also, Sjögren’s patients have an increased risk for lymphoma (cancer of the lymph glands). Twenty years after their diagnosis, up to 18% of Sjögren’s patients have lymphoma. Make sure to tell your doctor about any swellings that persist.

When to Consult a Healthcare Provider

Most patients do not tell their healthcare providers about dryness problems. This has led to Sjögren’s disease being underdiagnosed. When they tell their doctors about their dryness problems, severe damage may have already occurred, making treatment more difficult. It is critical to tell your healthcare providers about dryness early in the disease process. Your doctors can recommend treatments such as prescription medications to reduce inflammation, prevent organ damage, stimulate saliva or tear production, and minimize other dryness problems.

Take proactive steps now to effectively manage dryness symptoms, safeguarding your health for the future!

The Sjögren’s Foundation: Advocating for Patients

Make sure to join the Sjögren’s Foundation. They are 100% committed to helping people suffering from Sjögren’s disease. They have regular virtual patient education seminars full of the latest research and advice. They have been instrumental in working on and publishing management guidelines so other healthcare providers can know the best ways to help Sjögren’s. They are also instrumental in assisting with new research to find better treatments.

Whether you’re a patient, caregiver, or healthcare provider, The Lupus Encyclopedia is invaluable for understanding how lupus affects the body’s many systems, including the exocrine glands.

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For more in-depth information on Lupus and The Exocrine Gland System: Sjögren’s Disease [Mar ’25 Update]:

Read more in The Lupus Encyclopedia, edition 2

Look up your symptoms, conditions, and medications in the Index of The Lupus Encyclopedia

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