Sjogren's syndrome
What is Sjogren's syndrome?
Sjögren's syndrome is an inflammatory auto immune disease that can affect many different parts of the body, but most often affects the tear and saliva glands. It is an autoimmune disease such that a person's normally protective immune system attacks her/his body and damages moisture-producing glands including salivary glands and lacrimal (tear-producing) glands resulting in decreased tears and saliva (dry eyes and mouth.
It can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It is ten times more common in women than men. About half of the patients also have other autoimmune diseases like rheumatoid arthritis or other connective tissue diseases, such as lupus.
Most patients with Sjögren's syndrome remain healthy, but some rare complications have been described, including an increased risk for cancer of the lymph glands (lymphoma).
Sjögren's syndrome is an inflammatory auto immune disease that can affect many different parts of the body, but most often affects the tear and saliva glands. It is an autoimmune disease such that a person's normally protective immune system attacks her/his body and damages moisture-producing glands including salivary glands and lacrimal (tear-producing) glands resulting in decreased tears and saliva (dry eyes and mouth.
It can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It is ten times more common in women than men. About half of the patients also have other autoimmune diseases like rheumatoid arthritis or other connective tissue diseases, such as lupus.
Most patients with Sjögren's syndrome remain healthy, but some rare complications have been described, including an increased risk for cancer of the lymph glands (lymphoma).
Types of Sjogren's syndrome
- Primary Sjögren's syndrome – not associated with any other rheumatic diseases
- Secondary Sjögren's syndrome –symptoms develop in the presence of another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, or psoriatic arthritis
Causes of Sjogren's syndrome
In autoimmune diseases such as Sjögren's syndrome, the immune system triggers an inflammatory response when there are no foreign infections/ cancer to fight off. This inflammatory response causes the body's white blood cells to attack and damage its own moisture-producing glands.
The exact cause for the abnormal immune response in Sjögren's syndrome is unknown. Research suggests that genetic factors and possibly viral infections may predispose people to developing this condition.
In autoimmune diseases such as Sjögren's syndrome, the immune system triggers an inflammatory response when there are no foreign infections/ cancer to fight off. This inflammatory response causes the body's white blood cells to attack and damage its own moisture-producing glands.
The exact cause for the abnormal immune response in Sjögren's syndrome is unknown. Research suggests that genetic factors and possibly viral infections may predispose people to developing this condition.
Symptoms of Sjögren's syndrome
Sjögren's syndrome is mostly characterized by dry eyes and mouth. In some patients the parotid glands may become enlarged.
The main symptoms are:
Less common features are:
Sjögren's syndrome is mostly characterized by dry eyes and mouth. In some patients the parotid glands may become enlarged.
The main symptoms are:
- Extremely dry eyes, causing a sandy feeling, burning, and redness; itching is not a specific symptom. Increases risk of eye infection and damage to cornea
- Extremely dry mouth and throat:
- difficulty chewing and swallowing especially dry food
- decreased or altered sense of taste
- difficulty speaking
- increase risk of dental decay, gingivitis. candidiasis (thrush)
- dry cough or hoarseness
- Enlarged parotid glands which can be painful
- Excessive chronic fatigue
- Stiffness and pains in muscles and joints, and even the whole body similar to fibromyalgia pain
Less common features are:
- Neuropathy: Feeling of numbness/tingling of the nerves in the arms, hands, legs, or feet
- Skin rashes on the arms and legs related to inflammation in small blood vessels (vasculitis)
- Vaginal dryness
- Gastrointestinal problems, such as acid reflex, bloating, abdominal pain, or diarrhea
- Inflammation of the lungs (pneumonia and bronchitis), kidneys (unlike lupus nephritis), liver (hepatitis and cirrhosis), or pancreas (Rare)
- Cancer of the lymphatic tissue i.e. lymphoma (occurs in up to 5% of patients with the primary disease)
Diagnosis of Primary Sjogren's Syndrome
- Detailed medical history taking and physical examination
- Presence of dry eyes and mouth- typical symptoms of patients. Dry eyes can be diagnosed by an ophthalmologist by measuring tear production or carefully examining the cornea. Schirmer tear test: a small piece of filter paper is placed under your lower eyelid to measure your tear production. (Do note that dry eyes can be caused by other medical conditions)
- Dry mouth: Certain imaging tests can check the function of your salivary glands. Sialogram: An X-ray can detect dye that's injected into the salivary glands and it shows how much saliva flows into your mouth. Salivary scintigraphy: This nuclear medicine test involves the injection of a radioactive isotope intravenously, which is tracked over an hour to see how quickly it arrives in all your salivary glands.
- Blood laboratory tests: Detection of the presence of autoantibodies in the blood such as ANA, anti-SSA, or anti-SSB (also known as anti-Ro or anti-La), and even rheumatoid factor.
- Biopsy of the inner lip: may show the inflammation that is damaging the salivary glands.
Diagnosis of Secondary Sjögren's syndrome
Secondary Sjögren's syndrome is generally diagnosed when the patient already has an established autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus and he/she develops extreme dryness of the eyes and mouth. This diagnosis only rarely requires a lip biopsy.
Secondary Sjögren's syndrome is generally diagnosed when the patient already has an established autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus and he/she develops extreme dryness of the eyes and mouth. This diagnosis only rarely requires a lip biopsy.
Treatment of Sjogren's syndrome
There is no cure for Sjögren's syndrome but it can be treated and managed. The goals of treatment are to lessen the bothersome symptoms and reduce the harmful effects of dryness. Generally, physicians give symptomatic treatment. The type of treatment depend on the parts of the body affected.
1. Managing dry mouth and oral cavity complications
- Medications such as pilocarpine (Salagen) and cevimeline (Evoxac) can increase the production of saliva, and sometimes tears. Side effects include sweating, abdominal pain, flushing and increased urination.
- Treat oral thrush with anti-fungal medications
- Drink more water
- Maintaining proper good oral hygiene: Brush your teeth, use dental floss, regular dental appointment and fluoride treatment
- Chewing sugar-free gum may stimulate saliva production
2. Dry eyes management
- Use artificial tears or gel form which lasts longer to increase hydration
- Decrease eye inflammation: Prescription eyedrops such as cyclosporine (Restasis) or lifitegrast (Xiidra) can reduce inflammation in the eye glands
- A small procedure called punctal plugs can be done to slow the disappearance of tears when artificial tears are not sufficient.
3. Nasal Dryness
- Humidifiers and nasal saline irrigation may improve symptoms
4. Immunosupressants and other medications especially for patients with other autoimmune disease
- Hydroxychloroquine (Plaquenil), an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren's syndrome by reducing joint pain and rash experienced by some patients.
- NSAIDS to reduce pain and inflammation
- Steroids and immunosupressants like methotrexate, azathioprine, mycophenolate and cyclophosphamide will help relieve systemic symptoms.
5. Exercise and good sleep hygiene
- will help improve fatigue and joint stiffness/pain
There is no cure for Sjögren's syndrome but it can be treated and managed. The goals of treatment are to lessen the bothersome symptoms and reduce the harmful effects of dryness. Generally, physicians give symptomatic treatment. The type of treatment depend on the parts of the body affected.
1. Managing dry mouth and oral cavity complications
- Medications such as pilocarpine (Salagen) and cevimeline (Evoxac) can increase the production of saliva, and sometimes tears. Side effects include sweating, abdominal pain, flushing and increased urination.
- Treat oral thrush with anti-fungal medications
- Drink more water
- Maintaining proper good oral hygiene: Brush your teeth, use dental floss, regular dental appointment and fluoride treatment
- Chewing sugar-free gum may stimulate saliva production
2. Dry eyes management
- Use artificial tears or gel form which lasts longer to increase hydration
- Decrease eye inflammation: Prescription eyedrops such as cyclosporine (Restasis) or lifitegrast (Xiidra) can reduce inflammation in the eye glands
- A small procedure called punctal plugs can be done to slow the disappearance of tears when artificial tears are not sufficient.
3. Nasal Dryness
- Humidifiers and nasal saline irrigation may improve symptoms
4. Immunosupressants and other medications especially for patients with other autoimmune disease
- Hydroxychloroquine (Plaquenil), an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren's syndrome by reducing joint pain and rash experienced by some patients.
- NSAIDS to reduce pain and inflammation
- Steroids and immunosupressants like methotrexate, azathioprine, mycophenolate and cyclophosphamide will help relieve systemic symptoms.
5. Exercise and good sleep hygiene
- will help improve fatigue and joint stiffness/pain
