Sjögren’s syndrome

Sjögren syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren syndrome is also associated with rheumatic disorders such as rheumatoid arthritis or systemic lupus erythematosus. The hallmark symptoms of the disorder are dry mouth and dry eyes. In addition, Sjogren syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain. Treatment is symptomatic and supportive and may include moisture replacement therapies, nonsteroidal anti-inflammatory drugs and, in severe cases, corticosteroids or immunosuppressive drugs.

A chronic systemic inflammatory autoimmune disease that can cause death if : pulmonary infection, a lymphoma, or renal failure.
T & B lymphocytes of the immune system invades the exocrine glands in your body.
Eventually this invasion leads to ductal epithelial cell hyperplasia, which cause a duct obstruction.
Later it leads to atrophy, fibrosis and hyalinization of acini.
Still later there is atrophy and replacement with fat of parenchyma.
The B lymphocytes can cause lymphoid follicles with germinal centers in the salivary glands.40 times more likely to develop B cell non-Hodgkin lymphomas.
Females are affected more than males
Symptoms: Alopecia (hairloss), Dry: skin, eyes, nose, mouth, respiratory tract, vagina.
The dry skin can be treated with lubricants.
The dry vagina can cause dyspareunia, which is a painful sexual intercourse. This can also be helped with lubricants.
The dry eyes, also called keratoconjunctivits sicca for more than 3 month is an important sign for Sjögrens syndrome.
The patient is usually complaining about bad vision, photosensitivity and irritation of the cornea.
Tests: Schirmers test, Slit-lamp (tear film breakup time)
The treatment for dry eyes: Avoiding drugs that cause dryness for example anticholinergics, diuretics, antihistamines, antidepressants, use artificial tears, or lubricant drops like, methylcellulose or hypromellose.
Stimulate lubrication of the eyes locally with cAMP or cyclosporine 2% olive solution, or we can stimulate systemically with pilocarpine or cevimeline.
tiny plugs placed in the tear drainage ducts, corneal transplantation.
The dry mouth, or also called xerostomia of more than 3 months is also an important sign of Sjögrens syndrome.
This patient is saying that she have a daily dry mouth with difficulty chewing, swallowing and even the taste of food is not as good as before.
Treatment for dryness of mouth is to drink water daily to help swallowing for example.
We can stimulate the salivary glands locally by sugarfree gum, lozenges, or mouthwash containing carboxymethylcellulose.
We can also stimulate salivary glands systemically with drugs, like, Pilocarpine or cevimeline.
We can avoid drugs that decrease salivary gland secretion, like anticholinergics, antidepressants or antihistamines.
This patient also have tooth decay, which can be prevented by regular dental visits with topical fluoride application, or simply by keeping a good oral hygiene after each meal.
She is complaining of secondary Candida infection.
In this case we give topical nystatin, lozenges, or clotrimazole.
Stones can appear in the salivary ducts, which have to be removed to preserve the salivary tissue.
We can measure the saliva production, which will be low in Sjögrens syndrome, meaning less than 1,5 ml in 15 min.
We can also use techniques like, salivary scintiscanning or sialography.
Except dryness, the patient may present with enlarged parotid glands
There are also extraglandular symptoms.
So the most common is Arthralgias and arthritis. We treat it with hydroxychloroquine or methotrexate and prednisolone.
Raynauds phenomenon can be seen, and its treated with nifedipine and gloves which protect from cold.
Lymphadenopathy in the cervical or axillary region can be seen.
Vasculitis can present itself as rashes on the skin called purpuras, which can be treated with glucocorticoids or immunosuppressive agents like Cyclophosphamide.
Kidney diseases like interstitial nephritis, kidney stones, impaired concentrating ability or renal tubular acidosis. Bicarbonate replacement can be given for renal tubular acidosis.
Chronic hepatobiliary diseases
Risks of non-Hodgkin lymphomas can be 40 times higher in Sjögrens syndrome patients.
This can be treated with antiCD20 therapy together with a CHOP regimen, which is a chemotherapy regimen consisting of Cyclophosphamide, Hydroxydaunorubicin, Oncovin (Vincristine), Prednisolone.
Rarely splenomegaly or myositis may be seen.
If we make blood tests, we can see leukopenia, anemia, an increased Erythrocyte sedimentation rate, and some autoantibodies.
The autoantibodies are Rheumatoid factors and antinuclear antibodies called SS-A (Ro) and SS-B (La).
Except blood tests, we can confirm that this is Sjögrens syndrome by taking a biopsy from the lip, more specifically from the minor salivary glands in the buccal mucosa.
This will show in the microscope that lymphocytes have attacked the salivary glands
There will be many large areas of lymphocytes with atrophy of the tissue.
Genetic tests can show an association of Sjögrens syndrome with HLA DR3.

Rheumatoid arthritis, Systemic lupus erythematosus, Systemic sclerosis, mixed connective tissue disease, vasculitis, Hashimoto’s thyroiditis, Polymyositis, primary biliary cirrhosis and Chronic autoimmune hepatitis.