Chongren Syndrome: Symptoms, Causes, Diagnosis, Tests and Treatments

Definition:

It is a chronic autoimmune disorder of the immune system. It is an autoimmune disease, which means that healthy tissues and cells are mistakenly attacked by the immune system.

It can develop at any age. However, most patients are diagnosed after the age of 40 years. Women make up 90 percent of patients, but men and boys are also affected.

It affects approximately 0.1 to 4 percent of people in the United States. 90 percent of people with chongren syndrome are women.

It affects the glands that produce moisture. Causes the eyes and mouth to dry, and can also cause tooth decay, recurrent mouth sores and a persistent dry cough.

It can also affect multiple systems and can be difficult to diagnose. However, blood tests, ophthalmological tests and measurement of the salivary flow rate can identify the condition.

In chongren syndrome, white blood cells attack the salivary glands, the lacrimal glands, and other tissues. This leads to a dry mouth and eyes due to the decrease in the production of tears and body saliva. It can also cause dry skin, nose, upper respiratory tract and vaginal.

The condition is also associated with other autoimmune disorders. These include rheumatoid arthritis, systemic lupus erythematosus and primary biliary cholangitis.

Classification

Doctors classify chongren syndrome as primary or secondary. The primary chongren does not develop as a result of another condition, while the secondary chongren co-occurs with another condition, such as lupus. However, the symptoms of primary and secondary chongren are similar, and both can be serious.

The reason for directing the moisture producing glands is not clearly known and requires further investigation.

Chongren syndrome is a serious disease, but timely treatment can mean that complications are less likely to develop and tissue damage is less likely to occur. Once treated, a person can usually handle the condition well.

Symptoms of Chongren Syndrome

The most common symptom associated with chongren syndrome is the inability to produce moisture for the eyes and mouth. Women can also report vaginal dryness.

Other symptoms may include:

  • Dental decay and eventual loss of teeth.
  • Persistent dry cough
  • Problems chewing and swallowing
  • Hoarsely.
  • Difficulty speaking.
  • Swollen salivary glands.
  • Recurrent oral candidiasis, a fungal infection in the mouth.

Signs and symptoms associated with dry eyes include:

  • A sensation in one or both eyes similar to irritation caused by foreign material, such as sand or gravel.
  • Tired and heavy eyes.
  • A sensation of itching.
  • Secretion of mucosa in the eyes.
  • Photophobia or sensitivity to light.
  • Burning eyes.
  • Swollen and irritated eyelids.
  • Blurry vision.

Smoking, traveling by plane, fans and environments with air conditioning or wind can exacerbate the symptoms.

In some cases, the patient’s immune system attacks other parts of the body, causing the following signs and symptoms:

  • General fatigue.
  • Muscle pain.
  • Inflammation of the joints, as well as stiffness and pain.
  • Peripheral neuropathy, or numbness and occasional pain in the arms or legs.
  • Lung disease.
  • Raynaud’s phenomenon, in which the hands feel sore, cold and numb.
  • Vasculitis, where the blood vessels become inflamed.

Complications

Chongren syndrome comes with serious complications if left untreated, including:

  • An increased risk of lymphoma and multiple myeloma.
  • Oral fungal infections.
  • Eye sight problems.
  • Inflammation that leads to bronchitis, pneumonia and other lung problems.
  • Problems of renal function.
  • Autoimmune hepatitis or cirrhosis in the liver.
  • Give birth to a baby with heart problems or lupus.
  • Peripheral neuropathy
  • Interstitial cystitis of the bladder.

It is important to seek treatment for chongren syndrome as soon as possible to prevent this.

Causes

The causes of chongren syndrome remain largely unknown.

Studies have indicated that a viral or bacterial infection can trigger the disease, but that the underlying cause is primarily genetic and environmental. The nervous system and the endocrine system, or hormonal system, have also been implicated in the appearance of chongren syndrome.

An environmental factor can change the immune system and cause immune problems later, such as infection with hepatitis C or Epstein-Barr virus.

Since most people who develop chongren syndrome are women, doctors still believe that estrogen, a female hormone, plays an important role.

Menopause is the most common time for the diagnosis of chongren syndrome. Some studies theorize that estrogen protects against the syndrome, and decreasing levels of the hormone can alter the immune function and trigger the disease.

Diagnosis

As the signs and symptoms of chongren are similar to some other health conditions, it can sometimes be difficult to diagnose.

The patient may be seeing several health professionals, perhaps a dentist for dry mouth and tooth decay, a gynecologist for vaginal dryness and an ophthalmologist for dry eyes.

Some medications can also cause symptoms similar to those of chongren syndrome.

The criteria for a definitive diagnosis are the following:

  • The need to apply eye drops that contain tears more than 3 times a day.
  • A constant feeling of irritation in the eyes.
  • Persistent dry eyes and mouth for more than 3 months.
  • Swelling in the salivary glands between the jaw and the ears, known as parotid glands.
  • A frequent need to drink when food is swallowed.
  • Evidence of dry eye on the results of ophthalmological tests.
  • Measure salivary flow rates.
  • A labial biopsy showing focal lymphocytic inflammation.

Tests

Chongren syndrome occurs in different ways for different people. A doctor may order some additional tests, which include:

Blood tests: chongren syndrome activates the presence of special antibodies in the blood. As these antibodies only appear in approximately 60 to 70% of people with chongren syndrome, a negative result does not mean that the syndrome can be ruled out. This frequently causes confusion in the initial diagnosis.

Ophthalmological tests: an ophthalmologist can perform different types of eye exams.

Salivary flow rate: the doctor measures the weight of the saliva produced in a cup for 5 minutes. If insufficient saliva was produced, it is one of the indicators of chongren syndrome.

Sialogram: a dye is injected into the parotid glands. An x-ray is then taken to determine the amount of saliva that flows into the patient’s mouth.

Salivary gammagraphy: a radioactive isotope is injected and followed by images to measure the function of the salivary gland.

Chest x-ray or computed tomography scan: this determines the presence of inflammation in the lungs, which can be caused by chongren syndrome.

Skin biopsy: this determines the presence of small fiber neuropathy.

Urinalysis: Urine samples are taken and analyzed to determine if the kidneys have been affected.

Treatment

Eye drops and artificial tears are effective methods to control dry eyes. The goal of treatment of chongren syndrome is to lubricate the affected areas and prevent the onset of complications.

Treatment options include:

Drugs to stimulate the flow of saliva: these include pilocarpine and cevimeline. They have a short-term impact, limited to a few hours, so several doses are usually required per day.

Artificial saliva : saliva substitutes and buccal lining gels can relieve dry mouth. They are available as aerosols, pre-treated swabs and liquids. These can be important at night, as the mouth becomes drier during sleep.

Artificial Lubricators: Artificial lubricators can help lubricate the eyes and are available without a prescription. There are prescription options that reduce the need for artificial lubricators, such as cyclosporin ophthalmic emulsion and hydroxypropyl cellulose granules.

Prescription eye drops: Include cyclosporine and lifitegrast.

Moisture camera glasses: These are special glasses that avoid irritants and retain moisture.

Prescribed prescription and throat medications: In cases of respiratory dryness, medications such as those used to stimulate the flow of saliva, as well as flaxseed extract, sorbitol, xylitol or malic acid, can moisturize the area.

Nonsteroidal anti-inflammatory drugs: Anti-inflammatory medications such as aspirin, naproxen, and ibuprofen can relieve people with chongren syndrome who experience joint pain.

Disease-modifying antirheumatic drugs: In cases where joint pain is accompanied by fatigue and rashes, these medications can relieve symptoms.

Examples include hydroxychloroquine or methotrexate. If it appears that chongren syndrome is affecting the muscles, nerves, lungs or kidneys, stronger corticosteroids may be prescribed.

Antifungal medications: If oral fungal infections occur, medications may be prescribed to fight the fungus.

Vaginal lubricants: for vaginal dryness, water-based vaginal lubricants can provide a solution, especially during sexual intercourse.

Occlusion punctual: when all the options of conservative treatment have been exhausted, this surgical option seals the tear ducts with small plugs to reduce the drainage of the tears of the eye. This keeps the eye moist longer.

It is likely that temporary silicone plugs are used until the success of the procedure is confirmed.

Autologous eye serum: In severe cases of dry eyes, eye drops can be made from the person’s blood serum.

Here are some easy ways to keep your mouth lubricated:

  • Consuming more liquids
  • Sucking ice cubes.
  • Rinse the mouth regularly to prevent infection and soothe the area.
  • Maintaining excellent oral and dental hygiene.
  • Stop smoking, as the smoke irritates the mouth and accelerates the evaporation of saliva.
  • Chew gum without sugar, which stimulates the production of saliva.
  • Applying coconut oil to dry areas, as it is both moisturizing and antimicrobial.

There is no cure for chongren syndrome. However, by successfully managing the dryness of the affected regions and treating any affected organ, a person with this condition can maintain a good quality of life.

Is it possible to prevent chongren syndrome?

Because the chongren syndrome can be hereditary, there is no particular way to prevent the development of the disease. However, prevention of complications of chongren syndrome, such as infection, eye irritation, etc., can be achieved using the methods described above.

What is the prognosis for patients with chongren syndrome?

With adequate attention to the eyes and oral attention, the prognosis for patients with chongren syndrome is generally encouraging.

Ocular dryness can cause serious injury to the eye, especially the cornea, and this should be avoided by consulting an eye doctor. Dry mouth can cause tooth decay and infection of the parotid gland and parotid glands. Optimal oral hygiene is essential.

Severe complications of primary biliary cirrhosis and lymphoma can drastically affect prognosis and are controlled in regular routine consultations.

What types of doctors treat chongren syndrome?

Doctors who treat chongren syndrome include general practitioners, including general practitioners, family practice doctors, and internists, as well as specialists that include rheumatologists, ophthalmologists, and otolaryngologists.

conclusion

Chongren syndrome is an area of ​​active immunology research. Many new treatments will be available in the near future. Research has suggested that rituximab may be beneficial for many characteristics of chondren syndrome.

In the early 1900s, Henrik Sjögren called the syndrome “keratoconjunctivitis sicca”. The name of the syndrome of sicca is now used technically only to describe the combination of dry mouth and eyes, regardless of the cause. The term sicca refers to the dryness of the eyes and mouth.