autoimmune diseases

Sjögren's Syndrome - Diagnosis and Treatment

Diagnosis

As seen in the introductory article, the symptoms of Sjögren's syndrome are numerous and affect more organs and tissues. Therefore, the diagnosis of the syndrome is based on several investigations. The main ones are:

  • Ophthalmological tests
  • Blood tests
  • Labial biopsy
  • Scintigraphy and sialography
  • Scialometria

OPHTHALMOLOGICAL TESTS

In Sjögren's syndrome, they play a leading role. They consist of the Schirmer test and the Bengal pink test . The first measures the production of tears; it uses a strip of absorbent paper, resting on the lower part of the conjunctiva. The second reveals if the corneal or conjunctiva epithelium is damaged.

BLOOD TESTS

They are essential to make a count and to assess the appearance of white blood cells present in the bloodstream. A higher than normal number and an abnormal shape indicate the presence of a lymphoma .

Moreover, they serve to detect the presence of auto-antibodies, that is the anomalous antibodies directed against the tissues of the organism. These include nuclear antibodies, anti-phospholipids, anti-gastric mucosa, anti-thyroid, anti-Ro, anti-La and finally rheumatoid factors .

LABIO BIOPSIA

The labial biopsy is the most comfortable histological examination to know the health status of glandular cells. It is performed on the inner lip.

SCANNING AND SKYLOGRAPHY

These are two diagnostic techniques that provide radiological images of the salivary glands. They both use a contrast medium to visualize the anatomy of glandular tissue. These are two minimally invasive tests.

SCIALOMETRIA

It is used to measure the amount of saliva produced in a given period of time.

OTHER DIAGNOSTIC TESTS

Then there are other methods of investigation, less practiced, but equally revealing of the disease. Through some laboratory tests, it is possible to measure the erythrocyte sedimentation rate ( ESR ) and the amounts of lysozyme, in tears and saliva. In patients with Sjögren's syndrome, the ESR is increased, while the lysozyme content is lower than normal.

Another possible diagnostic test is the renal creatinine clearance . In about half of patients with Sjögren's syndrome it is increased.

Finally, to evaluate the presence of a lymphoma or not, you can use a CT scan . TAC uses ionizing radiation, therefore it is an invasive test.

Treatment

There is currently no specific cure for Sjögren's syndrome. Therapy, therefore, aims to alleviate:

  • Local symptoms, such as xerostomia, xerophthalmia or vaginal dryness.
  • Systemic manifestations, typical of autoimmune diseases (the adjective systemic indicates that more organs and tissues are affected by the disease).

LOCAL THERAPY FOR XEROSTOMY

Patients are advised, first of all, to always keep their mouth hydrated, either through the intake of liquids, or through the application of a special moisturizing gel.

To stimulate the production of saliva, pilocarpine 5 mg tablets should be taken 4 times a day. Pilocarpine is effective only if the salivary glands have retained some of their functions; in the case of complete glandular atrophy, in fact, the treatment does not give results.

Oral hygiene and dental health are also very important. In fact, the use of antifungals is required as protection from oral candidiasis, while avoiding sugars and periodic dental check-ups serve to prevent the formation of tooth decay.

LOCAL THERAPY FOR XEROFTALMIA

To treat dry keratoconjunctivitis, the patient must take artificial tears and eye drops based on methylcellulose or polyvinyl alcohol. In this way, the feeling of sand in the eyes, burning and dry eyes are relieved. The number of applications depends on the degree of dryness.

To stimulate glandular secretion, oral pilocarpine can be used. The efficacy of this treatment depends, also in this case, on the state of atrophy of the lacrimal glands. Finally, periodic eye checks are recommended to the patient, to prevent a possible eye infection and corneal injury.

LOCAL THERAPY FOR VAGINAL DRYING

The remedy, in these cases, involves the use of lubricating gels, based on propionic acid. Also in this case, hygiene is important to avoid the danger of an infection (vaginal candida).

SYSTEMIC THERAPY

The systemic therapy of Sjögren's syndrome aims to alleviate extraglandular manifestations.

As mentioned, the cause of these disorders is the auto-antibodies and other cells of the immune system, which rebel against the body and attack it.

Therefore, several drugs are administered, such as:

  • Corticosteroids
  • Preparations with an immunosuppressive action (immunosuppressants)
  • NSAIDs

Corticosteroids, at low doses, are indicated in the primitive forms of Sjögren's syndrome. They are used to relieve pain due to arthralgia and asthenia. Higher doses, on the other hand, are taken in the most severe cases, when vasculitis and renal deficits appear.

Immunosuppressive drugs include cyclophosphamide, methotrexate, hydroxychloroquine and cyclosporine A. Their main action is to reduce the number of auto-antibodies in the blood. But they can also be useful in the treatment of vasculitis and interstitial nephritis, due to the lymphocyte infiltrate. Immunosuppressive drugs are particularly indicated when Sjögren's syndrome is associated with other autoimmune diseases, such as rheumatoid arthritis or systemic lupus erythematosus.

NSAIDs are non-steroidal anti-inflammatory drugs and are used to relieve pain due to joint and muscle disorders.

Prognosis

Patients with Sjögren's syndrome have a different prognosis from case to case. Some patients show only the main symptoms: xerostomia and xerophthalmia. For these the prognosis is good, as long as they undergo periodic medical checks and adhere to strict hygiene rules, both oral and ocular. Otherwise, the quality of life may be affected.

Very different is the case of patients with secondary forms of the syndrome. For them, the prognosis becomes worse, since other organs and tissues of the body are more easily affected. One of the most dangerous consequences of Sjögren's syndrome is the possibility of developing lymphomas .