What is Sjögren's Syndrome

Normally, our body has a defence system  called the immune system that produces antibodies to fight against foreign invaders and to provide  immunity. But there are conditions called autoimmune disorders where our body starts recognising normal or healthy cells or even parts of the human body as foreign and starts to attack them by producing  autoantibodies. 

Sjögrens syndrome (pronounced show-grins) is a chronic autoimmune condition mainly affecting the  glands involved in the production of tears, saliva, digestive juices, sweat and breast milk. The two glands being focused on in this article are the lacrimal gland and the salivary gland. The lacrimal gland is important for tear production and the salivary gland is important for saliva production, resulting in  symptoms such as dry eyes and dry mouth. 

Besides dryness of the oral cavity, pharynx, larynx, and vagina,Sjögren’s syndrome is a systemic disease, meaning it affects the entire body, and harms  various other organs and tissues.

This  article briefly outlines the overview, causes, symptoms, diagnosis and management of Sjögren’s syndrome.

Overview

Sjögren’s syndrome is an autoimmune disease whereby our own immune system attacks the exocrine gland that produces the necessary mucous secretion of the body. Usually those who are assigned female at birth and middle aged  are more likely to be  affected .The disease also shows extraglandular involvement, meaning it affects  various organs and tissues over time, mostly  the joints,thyroid, kidney, liver, lungs, skin and nerves. 

The common symptom of dry eye  is also a symptom of vitamin A deficiency (xerophthalmia). Hence Sjögren’s syndrome was referred to as keratoconjunctivitis sicca or sicca syndrome by the Danish ophthalmologist Sjögren.

Sjögren’s syndrome is classified into:

• Primary Sjögren’s syndrome- When  patients are diagnosed with Sjögren’s  syndrome alone and it is not associated with any other autoimmune disorder
• Secondary Sjögren’s  syndrome- When  the development of Sjögren’s  syndrome is due to a pre-existing autoimmune disorder such as a rheumatic disease (rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis)

Causes of Sjögren's syndrome

The main pathogenesis of Sjögren’s  syndrome is the destruction of the epithelial lining of the exocrine gland which has a crucial role in production and secretion of various products such as  tears, saliva and sweat.

The risk factors associated with primary Sjögren’s syndrome is:

• Genetics: A family history of Sjögren’s  syndrome or any other autoimmune disorder 
• Infectious triggers: Cytomegalovirus or Epstein-Barr viral infection.
• Age and gender: People who were assigned female at birth and are middle aged are more likely to suffer from Sjögren’s syndrome  

Signs and symptoms of Sjögren's syndrome

The two main symptoms observed are:

• Dry eye 
• Dry mouth

However due to the involvement of other organs and tissues other symptoms observed are:

• Arthritis
• Joint pain
• Muscle pain
• Fatigue
• Raynaud's phenomenon (numbness or cold due to reduced blood flow towards hand and feet)
• Peripheral neuropathy (numbness and tingling sensation on the arms and legs) 
• Xeroderma (dry skin)¹
• Annular erythema leading to itching and burning 
• Eyelid dermatitis
• Cutaneous vasculitis
• Dry hair
• Breathing disturbances
• Sleep disturbances
• Anxiety 
• Depression        

Dentists are the first medical professionals to come across Sjögren’s  syndrome, because of the hallmark of oral symptoms.¹ Since there is a decrease in salivary flow in the mouth, patients are more prone to infections and can develop the following dental problems: 

• Dental caries
• Gingivitis (inflammation of gums)
• Periodontitis 
• Plaque accumulation 
• A fungal infection called candidiasis
• Mucositis (inflammation of oral mucosa)
• Angular cheilitis (a painful crack on the side of lips)
• Traumatic oral lesions
• Difficulty wearing or retaining oral prostheses

Management and treatment for Sjögren's syndrome

Diagnosis

Diagnosis is challenging due to the fact that some of the symptoms are observed in other disorders and as a side effect of some  drugs.

First, there is a  physical examination to check for ocular and oral dryness.  Patients will be looked by a  dentist or rheumatologist  for salivary pooling on the floor of the mouth to see if further  diagnostic tests are needed, such as:

• Assessing salivary flow rate
• Salivary gland biopsy
• Blood tests 
• Microbial tests³  

Blood tests

Testing the presence of autoantibodies in blood is used as a common test for diagnosing autoimmune disorder. Blood tests for autoantibodies such as serum (anti-Sjögren’s syndrome–related antigen A )anti-SSA/Ro and anti-SSB/La antibodies is used as a diagnostic approach for Sjögren’s  syndrome. 

Other than these blood tests, the different immune cells are counted, and indication of problems to other organs are also investigated

Biopsy:

Along with the autoantibody detection, salivary gland biopsy is required to r see  if there are signs of focal lymphocytic sialadenitis (FLS).  

Usually the sample for labial salivary gland biopsy is taken from the lip where minor salivary glands are situated, especially the lower lip biopsy as it is easy to obtain and reduces the chances of excessive bleeding.⁴ Pathological examination of the biopsy will help in understanding the seriousness and progression of the disease. 

Imaging:

Salivary gland ultrasonography (SGUS), magnetic resonance imaging (MRI), sialography, and positron emission tomography/computed tomography (PET/CT) are also  used in the diagnosis and prognosis of Sjögren’s syndrome. However,  due to the invasiveness, cost and not so proven relation with the histopathological features they are not often used. 

Ultrasonography is useful in identifying any morphological changes in the baseline tissue structure of the salivary gland.    As the tissue becomes dense or solid showing  it is likely to be  cancerous. 

A small proportion of  patients develop cancer of the lymph nodes called  lymphoma which is why imaging is helpful. Although SGUS are not part of formal diagnostic classification criteria, they are helpful in monitoring the condition of patients. ⁵ 

Treatment

Until now there are no specific treatments available for the treatment of primary Sjögren’s  syndrome.Keeping a track of the symptoms and palliative care is the major form of therapy. Treating  the complications are the only form  of treatment available.

Dry eye

Artificial tears or eye drops and lubricating ointments are usually given by an eye doctor to comfort dry eye symptoms. Protecting the eye from any sorts of dust, breeze and wind can prevent the worsening of dry eye. Tear ducts help in the draining of tear and blocking it can preserve the tear and help in treating dryness.

Dry mouth

Salivary stimulants (Sialogogues )  are prescribed by medical professionals to stimulate saliva production and glandular secretion thus relieving dry mouth symptoms. 

Chewing to increase salivation is also recommended to relieve dry mouth. 

Keeping the mouth hygienic with regular cleaning, floss and brushing can prevent bacterial and fungal infections. As well as keeping hydrated by drinking water  regularly .

Dry skin

Moisturising can get rid of the dry skin symptoms and associated rashes and erythema.

Abstaining from alcohol and smoking and other environmental factors such as wind and humidity that can exaggerate the sicca symptoms.

Treating other complications

Patients with rheumatic joint pains and arthritis symptoms may require Non Steroid Anti Inflammatory Drugs(NSAIDs), Corticosteroids, Disease Modifying Anti Rheumatic Drugs(DMARDs) and drugs to manage other complications as well depending on the severity.

FAQs

How is Sjögren's syndrome diagnosed?

The primary symptoms such as dry eye and dry mouth and related symptoms are examined followed by blood test, biopsy and imaging if necessary can diagnose Sjögren’s  syndrome.

Who is  at risk of Sjögren's syndrome?

Usually people assigned female at birth  who  in their  mid 40s and 50s  are at higher incidence of developing Sjögren’s  syndrome. 

Moreover genetic and environmental factors can drive towards developing the condition.

When should I see a doctor?

As soon as you experience the initial symptoms consult a doctor with proper diagnosis.

Summary

Sjögren’s  syndrome is an autoimmune disorder attacking the gland involved in the production of various secretions required by the body. Sjögren’s  syndrome is also called as sicca syndrome or keratoconjunctivitis sicca because of the common symptoms such as dry eye and dry mouth. 

Since it  is an autoimmune disorder the immune system is  constantly being  attacked and thus  patients experience various other organ disorders and rheumatic disease. 

Diagnosis is through various blood tests, biopsy and imaging to arrive at a conclusion. However there are no specific treatments available while managing the symptoms can prevent the patient from flare up.