Introductory Remarks

Overview about Sjogren’s syndrome

Sjogren’s syndrome, also known as Sjogren’s disease, is a chronic auto-immune condition. Being auto-immune, the immune system attacks healthy cells instead of invading disease-causing microbes such as bacteria and viruses.  In this condition, the immune system attacks glands which make moisture in the mouth, eyes and other body parts.

This is a life-long condition that requires regular care. It should be noted that most individuals with Sjogren’s disease lead healthy lives and do not have serious health problems.

Sjogren’s syndrome can affect organs such as the kidneys and lungs and even systems like the nervous system. It occurs by itself or alongside other auto-immune conditions like;

•  Rheumatoid arthritis.
•  Reynaud’s phenomenon.
•  Lupus erythematosus.

Unfortunately, there is no cure for treating Sjogren’s syndrome. Various ways to treat and manage the symptoms are utilized to manage this condition.

Sjogren’s syndrome is named after a physician from Sweden who in the early 1900s first described a group of women whose chronic arthritis was also accompanied by dry eyes and dry mouth. His name was Henrik Sjogren.

 

 Essential Statistics and Key Facts

• Sjogren syndrome is a rare disorder.
• It is estimated that over 1 million adults suffer from this condition.
• It affects adults and rarely children, without ethnic or geographical bias.
• This condition has a marked predilection for women. The female to male ratio is approximately 9:1.

 

 Causal Factors

What are the causes of Sjogren’s syndrome?

This is an auto-immune disorder. It occurs when the immune system which is supposed to the body from infections and disease attacks the normal healthy tissues (glands that make tears, saliva and other various body fluids) in the body.

It is believed that both genetic and environmental factors play a role auto-immunity.

When the immune system attacks glands that produce body fluids, the resulting inflammation damages these glands and limits fluid production.

The immune system can also attack other parts of the body, leading to injury of other organs and tissues. This causes a variety of other symptoms.

 

 Risk Elements

What are the risk factors associated with Sjogren’s syndrome?

• Sex. Women and people assigned female at birth (AFAB) are 10 times  more likely to suffer from Sjogren’s syndrome as compared to men and people assigned male at birth.
• Age. Although younger adults and children can have Sjogren’s disease, it usually affects individuals aged 40 years and above.
• Other auto-immune conditions. Almost one-half of all individuals with Sjogren’s disease also have another auto-immune condition. For example, rheumatoid arthritis.

 

 Signs & Symptoms

How does Sjogren’s syndrome manifest?

• Signs and symptoms of Sjogren’s syndrome are:

1. Dry eyes. Dryness of the eyes can cause blurry vision or sensitivity to light.
2. Burning or itching sensation in the yes. One may feel as if there are sand particles in the eyes.
3. Dry mouth.
4. Chalky feeling in the mouth.
5. There is trouble with speaking, and also swallowing and tasting food.
6. Due to withdrawal of the protection by saliva,  mouth infections such as oral candidiasis (thrush) may develop.

 

• Sjogren’s syndrome can affect other tissues and organs and has more widespread effects on the body. These other effects can cause;

1. Skin rashes.
2. Vaginal dryness.
3. Trouble sleeping.
4. Muscle weakness.
5. Dyspnea (shortness of breath).
6. Dry skin.
7. Dry nasal passages and throat.
8. Fatigue.
9. Joint pain.
10. Poor concentration.
11. Numbness and weakness in the extremities.

 

Types

What are the types of Sjogren’s syndrome?

Two known types of Sjogren’s syndrome are:

1.  Primary Sjogren’s syndrome.
2.  Secondary Sjogren’s syndrome.

 

Primary Sjogren’s syndrome

This refers to Sjogren’s syndrome that occurs without any other auto-immune or rheumatic disease.

Secondary Sjogren’s syndrome

This is Sjogren’s syndrome which occurs along with another auto-immune or rheumatic disease, such as scleroderma or rheumatoid arthritis.

 

 Diagnostic Procedures

How is Sjogren’s syndrome diagnosed?

• Sjogren’s disease can be hard to diagnose as the signs and symptoms vary from one individual to another and can be same as those caused by other diseases.
• Side effects of various medications also mimic signs and symptoms of Sjogren’s disease.
• Tests can be conducted to aid in ruling out other conditions and pinpoint a diagnosis of Sjogren’s syndrome.

1. Blood tests.
2. Imaging.
3. Eye tests.
4. Biopsy.

 

Blood tests

• These can be done to check for;

1.  Presence of antibodies against Sjogren’s syndrome.
2.  Inflammatory cells.
3.  Levels of different blood cell types.
4.  Indications of problems related to liver and kidneys.

 

Imaging

• Imaging tests that can be carried out to check the functionality of the salivary glands are;

1.  Sialgram.
2.  Salivary scintigraphy.

Eye tests

• Dryness of the  eyes can be measured using a test known as Schirmer test. A small piece of filter paper is placed under the lower lid to measure production of tears.

Biopsy

• Lip biopsy can help detect clusters of inflammatory cells, which indicate Sjogren’s syndrome.

 

Treatment & Management

• Artificial tears applied regularly during the day or gels applied at night for eyes.
• Eye drops which reduce inflammation in glands around the eyes, for example cyclosporine, are utilized to increase production of tears.
• Hydration to relieve dry mouth.
• Prescription medications which stimulate saliva flow such as pilocarpine and cevimuline.
• Hydroxychloroquine to treat inflammatory polyarthritis.
• Oral/parenteral corticosteroids as the first approach to major-organ system disease.
• Humidifiers and nasal saline iirigation to deal with nasal dryness.

 

Complications

What are the complications associated with Sjogren’s syndrome?

Dental cavities.

• Yeast infections.
• Vision problems.
• Lymphoma.
• Peripheral neuropathy.
• Pneumonia and bronchitis due to inflammation of the lungs.
• Hepatitis.
• Liver cirrhosis.
• Premature deliveries and spontaneoous abortions.

 

Differential Diagnosis

• Parkinson’s disease.
• Mumps.
• Lymphoma.
• AIDS.
• Lupus.
• Rheumatoid arthritis.
• Use of medications such as anticholinergic and antidepressant drugs.
• Dehydration.
• Sarcoidosis.

 

Outcomes

• Mild Sjogren’s disease has good pronosis.
• Moderate to severe Sjogren’s syndrome is associated with very poor quality of life.