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Sjogren’s syndrome symptoms, causes, and treatment.

Sjogren’s syndrome is a common autoimmune condition. In the following article, we will review the essentials of this condition. 

Sjogren’s syndrome affects around 0.1% to 4% of the whole US population. It similarly affects the rest of the world, with approximately 1-2 million people having the disease. Sjogren’s syndrome typically affects older patients but can affect young individuals too. Also, Sjogren’s syndrome affects 9 to 10 times more women than men. This may be related to female hormones. 

In the following reading, you will learn everything about this condition. Everything, explained by a doctor with simple terms for your comprehension. Here, you will find the answer to the most frequently asked questions about this disease. So, keep reading if you want to know about the causes, symptoms, diagnosis, and treatment of Sjogren’s syndrome. 

What is an autoimmune disease? 

Typically, your immune system is in charge of attacking foreign agents that enter your body. This protects us from harmful viruses and bacteria. When a foreign agent is recognized, your immune system will respond with an inflammatory reaction to attack it. The purpose of this is to eliminate possibly harmful pathogens.

It is pretty easy for your immune system to differentiate an external agent from your own cells most of the time. However, your immune system can sometimes make mistakes. This is when an autoimmune disease occurs.

An autoimmune disease is basically a condition in which your immune system attacks your own body. This can affect many different tissues and organs in your body, making you very sick. Not only because it damages your own tissues, but because it leaves you unable to fight real foreign agents. Some of the most common autoimmune diseases include type 1 diabetes, systemic lupus, rheumatoid arthritis, and Sjogren’s syndrome. 

What is Sjogren’s syndrome? 

Sjogren syndrome is a chronic inflammatory disease. Its main characteristic is the infiltration of white blood cells in exocrine organs. Therefore, it is considered an autoimmune disorder.

The function of exocrine organs is to secrete substances into another surface of the body through little ducts. Some of the most important exocrine organs are salivary glands, breasts, pancreas, stomach, and lacrimal glands. All of these organs can be affected when a patient suffers from Sjogren’s syndrome. However, this disease affects more commonly the salivary and lacrimal glands. 

File:Blausen 0780 SalivaryGlands.png
Salivary Glands
Source

There are two major types of Sjogren’s syndrome: primary and secondary. The primary Sjogren syndrome occurs in people who don’t have any other autoimmune disease. On the other side, secondary Sjogren syndrome happens in patients who already have another autoimmune disease like lupus or rheumatoid arthritis. There is no clinical difference between primary or secondary Sjogren’s syndrome. Sjogren’s syndrome doesn’t affect their overall health for most patients and doesn’t decrease their life expectancy. 

What is the cause of Sjogren’s syndrome? 

The exact cause of Sjogren’s syndrome is not fully understood. As we mentioned before, Sjogren’s syndrome can occur as a primary disease of exocrine glands or in association with other rheumatic diseases. The basic reason for this condition to happen is an autoimmune response.

Usually, your autoimmune system is in charge of destroying external agents that can cause harm. In an autoimmune disease, this attack occurs towards your own and healthy cells. Depending on the condition, there will be some specific tissues affected. In the case of Sjogren’s syndrome, the affected tissue is the exocrine glands. These glands are in charge of producing and releasing substances into other organs or organ surfaces. The most affected glands in Sjogren’s syndrome are the salivary and the lacrimal glands. This will cause symptoms in both your mouth and your eyes.

Some viruses include EBV (Epstein Barr Virus), Human Herpes Virus, HIV, and Hepatitis C virus. These viruses can trigger an inflammatory response that ends up hurting your body’s glands and tissues. Doctors think it’s possible that some viral infections can also act as triggers for this disease. However, this is not confirmed yet. 

What are the risk factors for developing Sjogren’s syndrome? 

Since the cause of Sjogren’s syndrome is not fully understood, it is hard to know its risk factors. At this point, doctors believe that there’s a combination of genetic and external factors that can cause it. Amongst the most critical risk factors we find: 

  • Genetics: Doctors think genetics are 50% involved in developing this condition. This means that some patients will be more likely to develop Sjogren’s disease because of their genes. This also means that if you have a relative with Sjogren syndrome, you are more likely to have it too. 
  • Infections: As we mentioned before, viral infections can be a trigger for developing Sjogren’s. Especially viruses like Epstein-Barr, Hepatitis C, Human Herpes, and HIV. Viral infections can activate an immune response that can become chronic and turn to affect your own tissues. This is especially important to patients who suffer from repeated infections. 
  • Age: Most patients with a diagnosis of Sjogren’s syndrome are older than 40 years old. However, it can also affect young individuals and even children. 
  • Gender: Women are at least 10 times more likely to develop Sjogren’s syndrome. The explanation for this may rely on some specific hormones women have. This also happens with other autoimmune or connective tissue diseases. 
  • Suffering from another rheumatic disease: This is what doctors call secondary Sjogren’s syndrome. A person with an autoimmune condition like systemic lupus erythematosus is more likely to develop this condition. In fact, around half the patients with Sjogren’s disease have been diagnosed with another autoimmune condition. 

Who’s affected by Sjogren’s syndrome? 

Anyone can be affected by Sjogren’s syndrome. In fact, around 1 to 4 million people in the US have been diagnosed with this disease. However, as we mentioned before, some patients are most likely to develop it. This includes patients with genetic predisposition, history of viral infections, older than 40 years old, and with a diagnosis of autoimmune conditions. There is a special focus on women since they are almost ten times more likely to develop it. 

What happens to patients with Sjogren’s syndrome? 

Sjogren’s syndrome foundation is an autoimmune condition in which your immune cells attack your healthy cells. Also, your immune system produces autoantibodies that trigger an inflammatory reaction within the affected tissues. In this case, the tissues are the exocrine glands. This results in impaired function of these glands and a poor liberation of the substances they produce. This affects the mucous membranes that need these substances to function properly as well. 

This causes the Sjogren’s syndrome symptoms that include: 

  • Dry mouth: As a result of the affectation of salivary glands like the parotid gland. This happens because of alterations in saliva production. This is one of the most common symptoms. 
  • Dry eyes: The second most common symptom of this condition. It’s related to the affectation of tear glands. This affects the production of tears that lubricate your eye. This is why patients with this condition manifest dry eyes that may burn or feel itchy. This is also known as keratoconjunctivitis sicca. 
  • Dry throat, lips, or nose: Sjogren’s syndrome also affects the mucose membranes of your body. This can result in a dry feeling of your throat, nose, and lips. 
  • Change in taste or smell: Dry mucose is not able to fulfill its job. This is why people with this condition can experience changes in smell or taste.
  • Dry skin: one of the organs with more exocrine or moisture-producing glands. 
  • Swollen salivary glands: This can cause a swollen neck or face because of the location of these glands. When these glands can’t produce saliva properly, they become inflamed. Also, Sjogren’s disease is characterized by an autoimmune and inflammatory reaction that also affects these glands. 
  • Joint pain: To move smoothly and painless, your joints need a liquid between them. This is called synovial fluid. Exocrine glands also produce this fluid. Patients with Sjogren’s syndrome have less production of synovial fluid and therefore experience joint pains. 
  • Yeast infections: In women, the vagina also depends on glands to maintain its natural humidity. Without it, a dry vagina is much more likely to suffer from yeast infections. 

What are some extraglandular manifestations of Sjogren’s syndrome? 

Even though it mostly affects the exocrine glands, Sjogren’s syndrome can also affect other organs. The most common extraglandular manigestations are: 

  • Arthritis: Inflammation of the joints that cause the common symptoms of joint pain. 
  • Pulmonary disease: That may result in cough and alteration of the respiratory system.
  • Gastrointestinal disease: The gastrointestinal tract depends almost completely on exocrine glands to complete their function. This can cause symptoms like heartburn, a burning sensation from your stomach to your chest.
  • Blood alterations: Like anemia and low white blood cells.
  • Vasculitis: The inflammation of the blood vessels of your body. 
  • Kidney affectations
  • Lymphoma: A rare type of cancer that affects the lymphatic system.

What are the complications? 

In general terms, Sjogren’s syndrome usually has a good prognosis or possible outcomes. However, like any other autoimmune disease, it can lead to complications if left untreated. Some of the most common complications include the extraglandular manifestations we talked about before. 

  • Eye: In excessive ocular dryness that doesn’t get treatment, the eye’s outer layer can be damaged. This damage can lead to vision problems that may be permanent. 
  • Mouth: Saliva acts as a protection for teeth against bacteria. A dry mouth is more likely to develop cavities in teeth.
  • Lungs: Untreated Sjogren syndrome can lead to lung infection and scarring of the lung tissue. 
  • Pregnancy: During pregnancy, your autoantibodies produced by Sjogren’s disease can affect your baby. 
  • Cancer: As we mentioned before, a small percentage of people with this condition can develop lymph node cancer (lymphoma).
  • Kidneys: Affectation of blood vessels can lead to the affectation of organs that need big blood flow, like kidneys. 
  • Liver: The white blood cells can invade tissues different than the exocrine glands. This includes organs like the liver, resulting in hepatitis. However, this is not common and almost only happens in advanced diseases. 

How is it diagnosed?

If you think you may be suffering from Sjogren’s disease, you should go to the doctor. This condition is not easy to diagnose, even for a medical professional. This happens because the symptoms may vary from patient to patient.

Moreover, many other conditions can cause similar symptoms. You must tell your doctor if you suffer from other autoimmune conditions. When you go to the doctor, they will first ask some questions about your symptoms and medical history. Then, they will perform a physical examination looking for possible signs of the disease.

If your doctor suspects you have Sjogren’s syndrome, they may indicate some laboratory tests. These tests can include blood tests looking for specific antibodies like antinuclear antibodies (ANA) and anti-Sjogren’s syndrome antibodies (anti-SSA).

Additionally, doctors may need to examine the tissue of your lips or salivary glands. Through a minor salivary gland biopsy, doctors can see if there is inflammation of white blood cell invasion.

Sometimes, your doctor may redirect you to an ophthalmologist or eye doctor for an eye exam. With this exam, doctors can see your eye and determine if it’s suffering any damage from dryness. Two useful imaging studies help in the diagnosis of Sjogren’s disease: sialometry and salivary scintigraphy. The sialometry allows your doctor to see how much saliva you produce. This is done with a special contrast injected into your salivary glands. The salivary scintigraphy measures how long it takes a radioactive component to travel from your blood to your salivary glands. 

What types of doctors treat Sjogren’s syndrome?

Many doctors and healthcare professionals can treat Sjogren’s syndrome. This includes family medicine doctors and internal medicine doctors. These are primarily in charge of keeping an eye on Sjogren’s patients, making sure they stick to treatment. Also, these physicians can refer you to some more specialized doctors. Specialists that treat Sjogren’s disease include rheumatologists, ophthalmologists, and otolaryngologists. 

How is Sjogren’s syndrome treated? 

There is no specific or definite cure to treat Sjogren’s disease. The treatment includes medications to control your symptoms and live your life as normal as possible. The treatment will depend on the symptoms that affect you the most. 

  • For your eyes: Artificial tears and prescription eye drops make a great improvement in dry eyes. There are also punctual plugs; tiny silicone plugs to close your tear duct. If this works, your doctor can also perform surgery to close the tear duct permanently. 
  • For your mouth: Saliva producers like gum or hard candies sweetened with sorbitol or xylitol increase saliva production. Medications like pilocarpine increase the production of saliva too. 
  • For your joints: Over-the-counter pain killers that help with joint pain and muscle aches.
  • Anti-rheumatics: Like hydroxychloroquine can improve salivary gland inflammation. 
  • Immunosuppressants: These lower the uncontrolled response of the immune system. They prevent organ damage by lowering inflammatory reactions. 
  • Steroids: They can also prevent inflammation of joints, skin, and many other organs. 

Do you have symptoms of this disease?

This tool is a Sjogren’s Syndrome Symptoms Checker. It gathers the most important signs, symptoms, and risk factors for the disease. Therefore, the tool will tell anybody who uses it the likelihood that their symptoms are because of Sjogren’s syndrome. Using the tool is free and would only take a few minutes.

What do you think?

Written by Dr. Esteban Kosak

Doctor of Medicine - MD Recently Graduated from Medical School and inspired to aid the global population during this situation. I think that we shall no longer be waiting to see a doctor when we feel sick. Several times we feel disease searches in Google drive us to a rabbit hole and come out thinking that we may die of cancer or something very serious, given that symptoms may seem to fit a wide variety of illnesses. Since I recently graduated from medical school. I have all the medical information fresh in my mind. My thorough experience as an expert researcher allows me to very-well known the different diseases and conditions that affect human bodies. Empowered by the United Nations 17 Sustainable Development Goals (SGDs). I think that we all can provide a grain of sand to help humanity. That's why we created Symptoms.Care a place where you can come and screen your symptoms and find what different illnesses can be related to them. Armed with the right information you can instantly, discretely, secure and from the comfort of your home talk with a Doctor that can Evaluate your Symptoms and help you seek the right treatment.

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