7/29/2019 Oncology Sjogren

http://slidepdf.com/reader/full/oncology-sjogren 1/5

SJOGREN’S SYNDROME 

Xerostomia-Sjogren syndrome; Keratoconjunctivitis sicca - Sjogren; Sicca syndrome

WHAT IS SJOGREN SYNDROME?

In the early 1900s, Swedish physician Henrik Sjögren (SHOW-gren) first described a group of women

whose chronic arthritis was accompanied by dry eyes and dry mouth. Today it is described as a chronic

disorder of the immune system - a long-term autoimmune disease - in which the patient's white

 blood cells attack the saliva and tear glands, leading to dry mouth and eyes which reduces saliva

and tears production.

**Sometimes the gland responsible for keeping the vagina moist is also affected, resulting in

vaginal dryness. There can also be pain and stiffness in the joints and aching muscles.

TYPES:

  Primary Sjogren's syndrome - the condition does not develop as a result of another

condition, it is not the consequence of another condition.

  Secondary Sjogren's syndrome - the condition develops because of or alongside

another condition, such as lupus or rheumatoid arthritis. 

CAUSE OF SJOGREN’S SYNDROME: 

**Experts are not sure why Sjogren's syndrome affects some individuals, and not others.

Studies have indicated that some viral or bacterial infections can trigger the disease.

  Primary Sjogren's syndrome- it is more likely to be a combination of environmental

and genetic factors such as Hepatitis C viral infection or an infection such as Epstein Barr

Virus may have triggered the immune system not to work properly. Doctors believe that

it is best to diagnose during menopausal stage, and they believe estrogen a female

hormone plays an important part. Possibly falling levels of estrogen might in some way

disrupt the immune system, some experts theorize. 

  Secondary Sjogren's syndrome - the syndrome can develop as another autoimmunecondition progresses, such as:

  Polymyositis  Rheumatoid Arthritis

  Scleroderma  Systemic lupus erythematosus

GENDER AND AGE PREDILECTION:

  40-50 year (rare in children.)

  Female

7/29/2019 Oncology Sjogren

http://slidepdf.com/reader/full/oncology-sjogren 2/5

SIGNS AND SYMPTOMS: 

Signs and symptoms associated with dry mouth:

  Tooth decay, and eventual loss of 

teeth

  Persistent dry cough

  Problems chewing

  Problems swallowing

  Voice becomes hoarse

  Some people may have difficulty

speaking

  Salivary glands become swollen

  Recurring oral thrush (fungal

infection in the mouth)

*The parotid gland is at the edge of the jaw and can become swollen and inflamed in some

 people with Sjögren's Syndrome.

Signs and symptoms associated with dry eyes:

  Eyes become and look tired

 Itchy eyes

  Mucus discharge from the eyes

  Photophobia - sensitivity to light

  Stinging or burning eyes

  Swollen eyelids

 Irritated eyelids

  Sensation that there is something inthe eyes such as gravel or sand

In some cases, the patient's immune system may attack other parts of the body, causing the

following signs and symptoms: 

  General Tiredness

  Aching muscles

  Inflammation of joints, as well asstiffness and pain

  Peripheral Neuropathy- numbnessand sometimes pain in the arms and

legs

  Raynaud’s phenomenon- hands feel

 painful, cold and numb

  Vasculitis- the blood vessels becomeinflammed

How is Sjogren’s Syndrome diagnosed? 

  Screening questions

**do you apply eyedrops containing tears more than three times a day?, do you feel as if there is gravel

or sand in your eyes?,has dry eyes bothered you persistently, for over three months?,do the glands

between your jaw and your ears keep swelling?,do you often need to drink when you are trying to

swallow something? Mostly if all of the questions answers to a yes further tests must be done like:

(For the eyes.)  Rose Bengal test- measures the state and function of the tear glands. 

** a non-toxic dye, is dropped onto the surface of the eyes. The dye's distinctive color helps the

specialist determine how well the tear film is functioning and how long it takes for the tears to

evaporate.

  The Schirmer test- strips of blotting paper are placed under the eyelid for about fiveminutes. 

**They are removed and analyzed to determine how much liquid the paper absorbed.

7/29/2019 Oncology Sjogren

http://slidepdf.com/reader/full/oncology-sjogren 3/5

 

(For the Oral Cavity.)

  Lip biopsy- the doctor removes a sample of tissue from the patient's inner lip and

examines it under a microscope. **If there are clusters of lymphocytes, it could point towards Sjogren's syndrome. Lymphocytes are

types of white blood cells.

  Salivary flow rate- the patient is asked to spit as much as he/she can into a cup for about

five minutes. 

  Sialogram - this is a special X-ray. A dye is injected into the parotid glands (behind the jaw and in front of the years). The aim is to determine how much saliva flows into the

 patient's mouth. 

  Sialograph-demonstrate the formation of punctate, cavity defects which are filled with

radiopaque contrast media.

**giving it a “cherry blossom” or “fruit ladden tree” effect radiographically.It is suggested that the

contrast material extravasates to the weakend salivary gland ducts to produce the sialographicfeatures. Poor elimination of contrast media is noted because of the retention of the material for

over amonth.

(Other parts of the body.)

  Blood tests - includes antinuclear antibodies (ANA), which are present in nearly all

patients.when the immune system is affected by Sjogren's syndrome, there are special

antibodies in the blood, known as SS-A and SS-B (or anti-Ro and anti-La). ** As these antibodies only show up in about 60% of Sjogren's syndrome patients, a negative result does

not necessarily mean the syndrome can be ruled out.

  Chest X-ray - this is to determine whether there is lung inflammation, which can be

caused by Sjogren's syndrome.

  Urine sample - the urine is tested to determine whether the kidneys have been affected.

TREATMENT AND MANAGEMENT:

For the eyes:

  Artificial tears- treats moderate cases of dry eye effectively If the eyes become severelyirritated, the doctor may prescribe a short course of corticosteroid-containing eye drops. 

**Long-term usage raises the risk of undesirable and serious side-effects, such as Cushing's

syndrome. Cyclosporine (Restasis), may be used to increase tear production. 

 Moisture chamber spectacles- these are special glasses that wrap around the eyes, a bitlike goggles. They help keep the moisture in, and also protect the eyes from dust,

 particles and other irritants. 

  Surgery - small plugs seal the tear ducts which drain the tears away. If they are sealedtears will stay in the eye longer. This procedure is called punctual occlusion. 

** It is generally only recommended if all else fails. Initially, the doctor will probably place temporary

silicone plugs to see whether the procedure was successful. If it was, the silicone plugs are later replaced

by permanent ones.

7/29/2019 Oncology Sjogren

http://slidepdf.com/reader/full/oncology-sjogren 4/5

 

For the mouth:

  Consume more liquids

  Ice cubes

  Mouth rinses

 Oral hygiene - good oral hygiene prevents gum disease and tooth

decay.

  Smoking - give up. Smoking speeds

up the rate at which saliva

evaporates. Smoke irritates the

mouth.  Sugar free chewing gum - this

stimulates the production of saliva.

  Saliva substitutes - these are available as a lozenge, gel, gum or spray. They help

lubricate the mouth. 

  Pilocarpine - a medication for dry eyes and mouth. The drug stimulates the saliva andtear glands to produce more tears and saliva. 

**(Salagen) or cevimuline (Evoxac)

  Hydroxychloroquine - a medication that slows the immune system's attack on saliva and

tear glands. It may also help with symptoms of joint pain and stiffness, and muscle pain.Patients will not notice any effects for several weeks. 

(Others)

  Vaginal dryness - lubricants, such as KY Jelly help. HRT (hormone replacementtherapy) and using estrogen creams are also options.

  Joint and muscle pains - OTC  NSAIDs (non-steroidal anti-inflammatory drugs), such as

ibuprofen are usually effective. The doctor may prescribe a stronger NSAID if needed. **Arthritis

  Disease modifying drugs (DMARDs)-similar to those used for rheumatoid arthritis may improvethe symptoms of Sjogren syndrome. These include tumor necrosis factor (TNF) inhibiting drugs. 

Other diseases associated with Sjogren’s Syndrome: 

  Thyroiditis (Hashimoto's thyroiditis) 

**Which can lead to abnormal thyroid hormone levels detected by thyroid blood tests. 

  Gastroesophageal Reflux Disease (GERD) 

**Which is a result of heartburn and difficulty in swallowing.

  Primary Biliary Cirrhosis (uncommon and rare)

** A rare and serious disease, autoimmune disease of the liver that leads to scarring of the liver tissue.

  Lymphoma**Cancer of the lymph glands, usually develops only after many years with the illness.

7/29/2019 Oncology Sjogren

http://slidepdf.com/reader/full/oncology-sjogren 5/5