What Is Secondary Sjögren’s Syndrome?

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Sjögren’s syndrome is an autoimmune disease that damages moisture-producing glands, making it harder to produce tears and saliva. While it can occur on its own, it can also be triggered by other autoimmune diseases, including rheumatoid arthritis (RA) and lupus. When that is the case, it is known as secondary Sjögren’s syndrome.

Anyone with an autoimmune disease can develop secondary Sjögren’s syndrome regardless of age, though it's typically diagnosed in older people. It's also more common in women, possibly because of hormonal differences.

Since there is no cure, treatment is designed to manage symptoms.

secondary sjogren's syndrome
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Symptoms of Secondary Sjögren’s Syndrome

The main symptoms of both primary and secondary Sjögren’s syndrome include:

  • Dryness of the eyes, mouth, throat, and upper airways 
  • Problems with swallowing food and medication
  • Changes in sense of taste
  • Chronic cough
  • Hoarseness
  • Dental problems
  • Difficulty speaking 
  • Vaginal dryness

Some people with primary and secondary Sjögren’s syndrome may also experience:

  • Fatigue
  • Brain fog
  • Fever
  • Loss of appetite
  • Joint, muscle, and/or nerve pain

Secondary Sjögren’s syndrome is often less severe than primary Sjögren's, but not always. Autoimmune conditions do often overlap, and it can be difficult to tell if a certain symptom is related to Sjögren’s or the primary autoimmune condition. For example, RA can similarly cause pain, fevers, fatigue, and loss of appetite.

Talk to your doctor about any symptoms that concern you regardless of whether they relate to secondary Sjögren’s, a primary autoimmune disease, or another health condition.

Complications

It's not unusual for people with Sjögren’s to experience infections. In both primary and secondary Sjögren’s, that includes dental and eye infections, sinusitis, bronchitis, and vaginitis.

People with Sjögren’s syndrome have a slightly elevated risk of developing lymphoma, a cancer of the lymph glands. The risk increases the longer you have Sjögren’s. You should report any lymph node swelling to your doctor.

Another very serious complication of Sjögren’s is vasculitis, a condition that causes inflammation of the blood vessels. Vasculitis can damage the tissues and organs that are supplied by affected blood vessels.

Causes

Sjögren’s is the result of the immune system triggering an inflammatory response in which white blood cells attack and damage the body's own moisture-producing glands. The exact cause of this abnormal immune response is unknown.

Autoimmune diseases most often associated with secondary Sjögren’s include:

Secondary Sjögren's impacts a substantial portion of people with autoimmune diseases, including about 20% of those with RA and and 14% of people with lupus, according to a 2018 study.

Common risk factors for Sjögren’s syndrome include:

  • Age: Though Sjögren’s can affect anyone, most people diagnosed are 40 or older.
  • Sex: Women are up to 10 times more likely to develop Sjögren’s syndrome, possibly because of the impact of female sex hormones on the immune system.

Interestingly, primary Sjögren’s syndrome affects between 2 and 10 out of every 10,000 people, the majority of whom are women as well.

A Connection to Fibromyalgia?

Sjögren's syndrome frequently overlaps with fibromyalgia, which is not currently considered an autoimmune disease. In one study, 20% of participants diagnosed only with Sjögren's fit the diagnostic criteria for fibromyalgia as well. Another study found that about 33% of people with fibromyalgia who reported dry eyes and mouth also had Sjögren's. The authors suggested that this might provide evidence of an autoimmune component to fibromyalgia, but more research is needed.

Diagnosis

There's no one test that can offer a definitive diagnosis of secondary Sjögren’s syndrome. Your doctor will start by looking at your complete medical history and conduct a physical exam.

A secondary Sjögren’s diagnosis includes:

  • Blood work that looks for proteins and markers specific to Sjögren’s
  • A lower lip biopsy to look for clusters of inflammatory cells (in some cases)
  • A referral to an eye doctor to test for dry eye
  • Tests to rule out other potential causes of your symptoms

Make sure you follow through with your doctor’s testing recommendations so you can get an accurate diagnosis and proper treatment.

You should tell your doctor about any over-the-counter (OTC) and prescription medications you are taking. Some medications may cause symptoms similar to those associated with Sjögren’s syndrome. This includes some blood pressure medications, oral contraceptives, antihistamines, and antidepressants.

Radiation treatments may also cause similar symptoms, especially if the head or neck is treated. Other autoimmune diseases may also mimic Sjögren’s syndrome.

Treatment

Treatment for secondary Sjögren’s syndrome depends on the parts of the body affected. OTC eye drops can help you to manage dry eye, and sipping water frequently can help with dry mouth. For vaginal dryness, your doctor may suggest a water-based vaginal lubricant.

If you need prescription treatments, your doctor may prescribe medications to:

  • Reduce eye inflammation: Prescription eye drops, such as Restasis (cyclosporine), may help reduce moderate to severe eye dryness.
  • Increase glandular activity: Medications such as Salagen (pilocarpine) can help increase tear and saliva production. Side effects of this medication include blurred vision, sweating, flushing, abdominal pain, and increased urination.
  • Treat complications: If you develop additional symptoms, such as a yeast infection of the mouth (oral thrush), your doctor will prescribe medications to treat them.
  • Treat the main cause of symptoms: Drugs that suppress the immune system, including methotrexate and Plaquenil (hydroxychloroquine), may be helpful in treating Sjögren’s syndrome.

A surgical procedure called punctual occlusion may be done when all other treatment options for managing eye dryness have failed. This surgical option involves sealing the tear ducts with small plugs to reduce the drainage of tears from the eye. This helps keep the eyes lubricated for longer periods. 

A Word From Verywell       

While there is no cure for secondary Sjögren’s syndrome or the primary autoimmune diseases that cause it, treatment can help alleviate symptoms and improve your quality of life.

Learn as much as you can about secondary Sjögren’s syndrome so you're aware of potential problems and worsening symptoms. Routine follow-ups with your doctor and early treatment are also important for reducing potential complications and improving treatment outcomes.

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Article Sources
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