- What is it?
- Causes
- Symptoms
- Diagnosis
- Treatment
- Evolution of the disease
- Living with the disease
- Research
- Frequently asked questions
Treatment for Sjögren Syndrome
Despite extensive work and research, no cure has yet been found for Sjögren Syndrome, and palliative measures are the central treatment focus. There are many saliva substitutes and artificial tears that help to reduce and alleviate the symptoms of a dry mouth and eyes. This type of local palliative treatment focuses specifically on dryness symptoms (dry eyes and mouth). Preservative-free gels and mouthwashes to combat a dry mouth and artificial tears, in single doses, under the supervision of an ophthalmologist, are very helpful.
The treatment must be carried out by a multidisciplinary medical team coordinated by a specialist in internal medicine (or rheumatologist), but which also includes other specialists in dryness (ophthalmologists, dentists, otolaryngologists, and gynaecologists) and experts on the possible effects on internal organs (pulmonologists, dermatologists, nephrologists, neurologists, haematologists, etc.). It is equally important that the primary care physician is involved in the care process, especially for patients with no systemic or internal effects, and that patients are educated about their disease so that they can help manage their own treatment.
Monitoring the symptoms at least every six months, by the specialist, helps prevent or limit organ damage and improves the patient's quality of life. If the disease progresses favourably, these controls can be up to one year apart.
The treatment, which varies from patient to patient, also depends on the symptoms the patient is experiencing at any given time. If the effects are severe, more continuous monitoring may be required and hospital admission may even become necessary.
It is becoming increasingly clear that a patient's lifestyle has an impact on autoimmune diseases. The following recommendations may be crucial in controlling the disease:
Avoid (and control) stress.
Sleep the required number of hours (at least 7) and in an effective way.
Exercise regularly (aerobically whenever possible).
Eat a balanced (Mediterranean) diet.
Avoid toxins. It is advisable not to smoke, drink alcohol, or consume any toxins.
Because dryness symptoms are the most characteristic of Sjögren Syndrome, it is important for patients to avoid treatments that worsen symptoms by decreasing the function of the salivary glands. Drugs that may accentuate these symptoms include diuretics, antihypertensive drugs (to control high blood pressure), antidepressants, and antihistamines, among others. However, some patients prefer to use them because of their benefits, despite any adverse effects they may have.
Dry mouth. A dry mouth is the main symptom of Sjögren Syndrome. The following are recommended:
- Good, regular oral hygiene.
- Fluoride toothpastes.
- Chewing sugar-free gum or sugar-free candy to stimulate saliva production.
- Drinking water during the day to hydrate, but not in excess (very large quantities of excess water in the mouth can "wash away" the small quantity of actual saliva that is being produced, cancelling out the benefits of that saliva).
- Trying the speciality mouth moisturisers, gels, sprays, and mouthwashes available in pharmacies for patients suffering a dry mouth.
- Alerting healthcare personnel that the dry mouth requires additional care.
- Reviewing the list of medications with the healthcare provider to see if any cause side effects in the mouth and consider other options to help with salivary function.
- Using a humidifier at home.
Dry nose. Nasal dryness varies from person to person. Saline spray is generally considered safe and provides a great deal of relief, although it does not last long. There are also saline nasal gels that can help resolve nasal ulcers that can cause dryness. Washing the nose once a day may have a longer-lasting effect.
Dry ears. Itchy ears may be the easiest dryness problem to treat. A drop of ear wax remover or mineral oil can solve the problem for several days. However, you should always consult medical personnel if the problem persists, to determine whether there is another problem, such as an infection, in addition to Sjögren.
Dry skin. Some recommendations for dry skin are:
- Use gloves when handling strong soaps or cleaning chemicals.
- Use warm, not hot, water for bathing.
- Use soap sparingly.
- Apply lotion as soon as possible after bathing to seal in moisture; hydrate immediately, while skin is still moist. Vaseline or bath oil may be used.
- When you come out of the shower, dry naturally, as the skin will absorb moisture from the water.
- Use sunscreen when outdoors.
- Avoid fabric softeners in the washer and dryer.
- After swimming, shower and immediately use a moisturiser to reduce dry skin symptoms.
Vaginal dryness. Vaginal dryness may be caused by Sjögren as well as other conditions, such as the menopause. No matter what the cause, if this occurs you should talk to a gynaecology specialist who will usually prescribe a vaginal lubricant.
Pharmacological treatment is only used in the most severe cases of dryness. Pilocarpine tablets can be taken for mouth dryness, and cyclosporine eye drops may be used for dry eyes. These should always be prescribed by your normal medical team, who have clinical experience of the disease.
Treatment of systemic involvement may include:
- Anti-inflammatory and anti-malarial drugs. To treat joint, muscle, and general symptoms.
- Corticosteroids. For the most severe cases of organ damage.
- Immunosuppressant drugs or biological therapies. In cases where the patient's life is endangered and when the disease does not respond to the usual treatments.
All these drugs must be prescribed and controlled by the medical team that is coordinating the disease.
The drugs used to treat systemic involvement of Sjögren Syndrome are the same as those used to treat lupus.
There is no surgical treatment for Sjögren Syndrome, although there is advice for a Sjögren patient who has surgery for another reason:
- The most common postoperative problems in patients with Sjögren Syndrome include corneal abrasions, sore throat, difficulties swallowing, and outbreaks of other symptoms listed in international classifications (SICCA).
- Before surgery, you must inform the medical and nursing staff that you have Sjögren Syndrome.
- Take any artificial tears, saliva or other treatments to the hospital or surgical centre on the day of the operation, as they cannot be provided there.
- Suspend anti-inflammatories at least 72 hours prior to surgery (unless otherwise directed by the medical team).
- Patients on corticosteroids should receive somewhat higher doses because of perioperative stress, and until they are clinically stable. Ask the medical team in charge of the surgery about the possibility of consulting the specialist in autoimmune diseases.
- On the day of the surgery, follow your usual oral care and medication routine. Eye drops should be requested before and after surgery, according to the patient's routine. During surgery, the eyes should be lubricated every 30 minutes.
- After the operation has finished, eye care should be resumed according to the patient's routine. Patients should be allowed ice chips, liquids, or artificial saliva as soon as possible.
The main new treatment aspects for Sjögren Syndrome focus on the increasingly rational (appropriate) use of drugs such as corticosteroids and immunosuppressants and, above all, on the use of the so-called biological therapies, a new type of medication manufactured in the laboratory that is similar to various molecules making up part of the immune system itself.
The most commonly used biological drugs for treating Sjögren Syndrome sufferers are those that act against B lymphocytes (mainly rituximab, as well as belimumab) for the main systemic complications of the disease, in addition to treating lymphoma.
The most common complications related to the drugs most frequently used to treat Sjögren Syndrome are:
- This is contraindicated in patients with clinically significant and uncontrolled heart disease, uncontrolled asthma, and other chronic diseases that worsen with cholinergic agonists.
- It should be used with caution in patients who sweat or are expected to sweat excessively and who are unable to drink enough fluids, as this may cause dehydration.
- It should be administered with caution in patients who have cholelithiasis or biliary tract disease, a peptic ulcer, cognitive or psychiatric disorders, renal failure, nephrolithiasis, or narrow-angle glaucoma.
Most of the adverse effects observed during treatment with pilocarpine are a consequence of increased parasympathetic stimulation. These adverse effects are dose-dependent and generally mild and self-limiting. However, serious adverse effects may occasionally occur, and the patient should therefore be carefully monitored.
Adverse effects classified as very frequent or frequent are: headache; dizziness; watering eyes; blurred vision; abnormal vision; conjunctivitis; eye pain; flushing (vasodilatation); high blood pressure; palpitations; rhinitis, dyspepsia; diarrhoea; abdominal pain; nausea; vomiting; constipation; sweating; allergic reactions, rash; itching; and increased urinary frequency.
Using external corticosteroids increases the total quantity of amount of these hormones in circulation, which is added to the main natural hormone manufactured by the body, cortisol. An increase in corticosteroid hormones can cause the face to appear rounded (known as "full moon face").
They can also cause intestinal problems, insomnia, and stomach ulcers. Calcium can also be lost from the bones (osteoporosis is the most common side effect of long-term corticosteroid treatments). They increase body hair and acne. The skin becomes thinner and stretch marks or spider veins appear. They raise blood sugar and cholesterol. Corticosteroids cause the liver to make more glucose and, through other internal mechanisms, they also cause the body to make worse use of that glucose. There is, therefore, a greater risk of hyperglycaemia, and people with diabetes may not be able to control their disease.
All these complications are typical in people treated with very high doses and for long periods of time. However, corticosteroids are a life-saving medication that prevents significant autoimmune damage to major organs, which is why they are the first line of treatment in the most serious conditions. However, it is currently recommended that large doses are administered intravenously (over 3-5 days) at the most serious point. Treatment then continues with doses lower than 30 mg a day, going down slowly, but progressively, until the drugs are completely stopped, if the clinical situation permits.
Some patients do not tolerate these medications and suffer fever, joint pain, rash, discomfort, or nausea. At any time during the treatment there may be a decrease in blood cells (leukocytes, red blood cells, or platelets) or an alteration in the hepatic analysis, making it necessary to adjust the dose of the drug.
In order to control possible alterations, periodic analyses are made every 3-6 months (at the beginning of treatment, this may be every month).
As these drugs interfere with the immune system, people who take them have a greater risk of infection. The immune system protects against infections, so when a drug cancels out or decreases the work of the immune system, there is a greater likelihood that the patient will be affected by an infection.
Substantiated information by:
Published: 28 May 2019
Updated: 28 May 2019
Subscribe
Receive the latest updates related to this content.
Thank you for subscribing!
If this is the first time you subscribe you will receive a confirmation email, check your inbox