An Overview of Secondary Sjogren’s Syndrome

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Sjogren’s syndrome is an autoimmune disease that damages moisture-producing glands, making it harder to produce tears and saliva.  It's identified by its two most common symptoms—dry mouth and dry eyes and is usually seen with other autoimmune diseases, including rheumatoid arthritis and lupus.

If you already have another autoimmune disease and develop Sjogren’s, the condition is called secondary Sjogren’s. When Sjogren’s is secondary, you might have a milder form of the condition but still experience symptoms. Sjogren's is commonly seen in people with rheumatoid arthritis (RA), another autoimmune disease known for causing inflammation throughout the body, especially in the joints and organs.

Anyone can develop secondary Sjogren’s syndrome, regardless of age, but most people who are diagnosed are over age 40 and it's more common in women. Since there is no cure, treatment aims to manage symptoms.

Symptoms

Primary Sjogren’s syndrome affects 2 to 10 per 10,000 people, the majority of which are women. The main symptoms of both primary and secondary Sjogren’s syndrome include dryness of the eyes, mouth, throat, and upper airways. Some people may also have problems with swallowing food and medication and difficulties with tasting. Some may also develop a chronic cough, hoarseness, dental problems, and difficulty speaking. Some women may experience vaginal dryness.

Additional symptoms of primary and secondary Sjogren’s syndrome include:

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

Sjogren’s syndrome is the most frequent disorder to occur secondary to other autoimmune diseases. A 2016 study of people with RA found the prevalence of sicca symptoms (dry mouth and dry eyes) among participants was 40% and confirmed secondary Sjogren’s syndrome diagnoses among the study participants was 6%.

Having secondary Sjogren’s syndrome doesn't mean that your Sjogren’s is less severe but it can be. Autoimmune conditions do often overlap, and it can be difficult to tell if a certain symptom is related to Sjogren’s or the primary autoimmune condition. For example, RA can similarly cause fevers, fatigue, and loss of appetite.

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

Causes

Because Sjogren’s is an autoimmune disease, it causes the immune system to trigger an inflammatory response where the body’s white blood cells attack and damage its own moist-producing glands. An exact cause of why an abnormal immune response occurs in Sjogren’s is unknown. 

Autoimmune diseases that trigger Sjogren’s syndrome include:

The most common risk factors for Sjogren’s syndrome are:

  • Age: Though Sjogren’s can affect anyone, regardless of age, most people diagnosed are 40 or older.
  • Gender: Women are up to 10 times more likely to develop Sjogren’s syndrome possibly due to how the sex hormones impact a women’s immune system.
  • Having an autoimmune disease: Those with another autoimmune disease, like those mentioned above, are more likely to develop Sjogren’s syndrome.

Diagnosis

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

A secondary Sjogren’s diagnosis includes:

  • Blood work that looks for proteins and markers specific to Sjogren’s
  • A lower lip biopsy may also be done to look for clusters of inflammatory cells.
  • A referral to an eye doctor to test for dry eye
  • Tests to rule out other potential causes of your symptoms

Make sure you are following through with your doctor’s testing recommendations to determine the cause of your symptoms.

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 Sjogren’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 Sjogren’s syndrome.

Treatment

Treatment for Sjogren’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 to reduce moderate to severe eye dryness.
  • Increase saliva: Medications, such as Salagen (pilocarpine), can help increase saliva production. Salagen may also help with increasing tear production. Side effects of this medication include sweating, flushing, abdominal pain, and increased urination.
  • Treat complications: If you develop additional symptoms, such as a yeast infection of the mouth, your doctor will prescribe medications to treat these complications.
  • Treat the main cause of symptoms: Drugs that suppress the immune system, including Methotrexate and Plaquenil (hydroxychloroquine), may be helpful to treating Sjogren’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 will help to keep the eyes lubricated for longer periods. 

Complications

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

People with Sjogren’s syndrome have a small risk for lymphoma, a cancer of the lymph glands. The risk increases the longer a person has Sjogren’s. You should report any lymph node swelling to your doctor.

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

A Word From Verywell       

While there is no cure for secondary Sjogren’s syndrome or its primary autoimmune disorder causes, treatment can help to improve your quality of life.

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

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