Sjögren's syndrome:
dry eyes, dry mouth — more than a nuisance

1. What is Sjögren's syndrome?

In Sjögren's syndrome (pronounced roughly “SHOW-grin's”), immune cells migrate into the glands that produce tears and saliva and disrupt their work. The result is persistent dryness of the eyes and mouth — medically known as sicca syndrome (from the Latin siccus = dry). Because the immune system turns against the body's own tissue, the condition is classed as an autoimmune disease and, within rheumatology, as a connective tissue disease.^¹,² Internationally, the term “Sjögren's disease” is increasingly being used, because it involves more than just one set of symptoms.

After all, Sjögren's syndrome is more than a nuisance: many of those affected also experience profound exhaustion (fatigue) and joint pain, and in some the lungs, kidneys, nerves, skin or blood count are involved. The risk of certain lymphomas is also increased — although in absolute terms this affects only a minority.^¹

2. Types: primary and secondary

• Primary Sjögren's syndrome — occurs on its own, without any other underlying rheumatic disease.
• Secondary (associated) Sjögren's syndrome — accompanies another autoimmune disease, for example rheumatoid arthritis, lupus erythematosus or scleroderma.

Independently of this, rheumatologists distinguish by extent:^¹

Most people affected have a fairly stable course that is limited to the glands. How active the disease is systemically is recorded with a standardised index (ESSDAI).

3. Symptoms: from dryness to organ involvement

Eyes

• A gritty or foreign-body sensation, burning, redness, sensitivity to light, tired eyes when reading.
• Paradoxically: watery eyes — irritated eyes sometimes reflexively produce watery tears that do not moisten the surface properly.

Mouth

• Dry mouth, difficulty chewing and swallowing dry food, a glass of water by the bed.
• Dental damage — without protective saliva, tooth decay develops more quickly, often at the neck of the tooth; fungal infections and cracked corners of the mouth are more common.
• Swollen salivary glands — especially the parotid gland in front of the ear, on one or both sides.

Other symptoms

• Exhaustion — a leaden fatigue that many find the most distressing symptom.
• Joint pain and muscle pain, less often genuine joint inflammation.
• Dry skin, nose and vagina — itching, nosebleeds, vaginal dryness with pain during sex.
• Organ involvement — a dry cough or breathlessness (lungs), impaired kidney function, numbness or burning in the hands and feet (nerves), small bleeds into the skin of the legs (vasculitis), fingers turning white in the cold (Raynaud's phenomenon).

4. Causes and what makes dryness worse

The exact cause is not known. According to current knowledge, a hereditary predisposition meets triggers — viral infections and hormonal factors are being discussed, which could explain why women are affected so much more often. Certain immune cells (B cells) are permanently overactive and produce autoantibodies.^²

• Predisposition — other autoimmune diseases in the family, for example thyroid inflammation such as Hashimoto's thyroiditis, occur more often.
• Female sex and age around the menopause.
• Other causes of dryness — hepatitis C, HIV, sarcoidosis, an IgG4-related disease or radiotherapy to the head and neck can produce a similar picture and are ruled out during diagnosis.

5. Diagnosis: antibodies, tear and saliva tests

The diagnosis is usually made by a rheumatologist, often together with ophthalmology and ENT specialists. The 2016 classification criteria of the ACR and EULAR are used for studies and as a guide; they combine antibodies, tissue findings and function tests.^³

• Antibodies in the blood: anti-Ro/SSA is the most important marker, often accompanied by anti-La/SSB, antinuclear antibodies (ANA) and rheumatoid factor. Some of those affected have no detectable antibodies.
• Schirmer test: a strip of filter paper placed in the lower eyelid measures how much tear fluid is produced in five minutes.
• Staining test of the eye: using dyes, the eye specialist shows damaged areas of the eye's surface.
• Saliva flow: how much saliva collects in a set time without stimulation.
• Lip biopsy: a small tissue sample from the salivary glands of the lower lip shows typical clusters of immune cells. It is particularly important when no antibodies can be detected.
• Ultrasound of the salivary glands: shows typical changes and helps monitor the course.
• Organ check: blood count, kidney values and urine, proteins in the blood (immunoglobulins, complement), and, if there are symptoms, lung function, imaging or nerve tests.
• Medication list: drying medicines are specifically asked about.

What counts for the diagnosis is the combination of symptoms and findings. A positive ANA result on its own does not prove Sjögren's syndrome — it is also found in many healthy people.

6. Treatment: dryness and organ involvement

Treatment depends on the extent of the disease. For dryness, the EULAR recommendations rely first on local measures and reserve medicines that dampen the immune system for active organ involvement. Put honestly: for the dryness itself and the fatigue, there is as yet no medicine that tackles the disease at its root — relief is realistic, a cure is not.^¹

Several new substances that specifically target overactive immune cells are currently being tested in large studies or are in the approval process. Which treatment suits you is decided by the treating rheumatology practice — ideally in coordination with eye and dental specialists.^¹

7. Hydroxychloroquine, cortisone & co. in everyday life

Using eye drops correctly

If you use drops several times a day, single-dose units without preservatives are a good choice — they irritate the surface of the eye less. Leave about five minutes between different drops, and apply gels and ointments last. Contact lenses are often poorly tolerated with very dry eyes; details in the guides Using eye drops correctly and Medications and contact lenses.

Hydroxychloroquine: slow to work, check your eyes

• Patience: the effect only sets in after weeks to months.
• Eye checks: over the years, hydroxychloroquine can damage the retina. According to the summary of product characteristics and ophthalmological recommendations, a baseline examination and regular checks are part of treatment, annually after several years of use.^⁴
• Heart rhythm: hydroxychloroquine can prolong the QT interval on the ECG. In combination with other medicines that do the same — for example some antibiotics such as azithromycin, antidepressants or antipsychotics — the risk increases.
• Blood sugar: it can lower blood sugar; watch out for low blood sugar if you take diabetes medicines.
• Stomach: taking it with a meal often improves tolerability.

Cortisone and immunosuppressants

Cortisone taken over a long period can strain blood sugar, blood pressure, weight and bones and put the eyes at additional risk (cataracts, raised eye pressure) — more in the cortisone guide. Regular blood tests are part of treatment with methotrexate, azathioprine or mycophenolate. Methotrexate is taken once a week, not daily; mix-ups have led to life-threatening overdoses. Ideally, check your vaccinations before starting immunosuppression, see Vaccines and medications.

8. Lymphoma risk: which signs matter

The permanent activation of B cells explains why people with Sjögren's syndrome more often develop non-Hodgkin lymphoma, usually a slow-growing lymphoma of the salivary glands (MALT lymphoma). The relative risk is markedly increased, but the large majority of those affected never develop it.^¹,²

It is important to know the signs that point to a higher risk:

• Persistent swelling of a parotid or submandibular salivary gland for weeks.
• Enlarged lymph nodes or an enlarged spleen.
• Skin vasculitis with small, palpable bleeds into the skin of the legs.
• Abnormal lab results such as low complement levels (C4), cryoglobulins or abnormal proteins in the blood.
• General symptoms such as fever, night sweats and weight loss.

That is why regular check-ups — with physical examination, lab tests and ultrasound if needed — remain part of care even when the dryness is well under control. The typical slow-growing lymphomas in Sjögren's syndrome are often very treatable — all the more so when they are detected early.

9. Planning a family and pregnancy

Pregnancy is generally quite possible with Sjögren's syndrome. One special feature concerns women with anti-Ro/SSA antibodies: these can cross the placenta to the baby. Rarely, they cause congenital heart block in the baby (a disturbance of electrical conduction in the heart) or a temporary skin rash after birth (neonatal lupus).^¹

• Plan ahead: discuss your antibody status and medicines with rheumatology and gynaecology before the pregnancy.
• Check-ups: with anti-Ro/SSA antibodies, frequent ultrasound examinations of the baby's heart in the second trimester are usual.
• Hydroxychloroquine: is considered acceptable during pregnancy and is often continued or started in anti-Ro-positive women, because it can lower the risk of heart block.^⁴,⁵
• Stop beforehand: methotrexate, mycophenolate and leflunomide must be stopped in good time before a pregnancy, and leflunomide additionally requires a washout procedure.

The basics are covered in the guide Medications during pregnancy.

10. Everyday life with Sjögren's syndrome

• Humidify the air — use a humidifier, keep the room temperature moderate, avoid fans or air conditioning blowing directly into your face; wear glasses with side protection outdoors.
• Screen breaks — we blink less often when reading and at a screen; deliberate breaks and drops before long periods of work help.
• Protect your teeth consistently — fluoride, sugar-free drinks, no constant sipping of juice or sweet things, dental check-ups several times a year; treat gum problems such as periodontitis early.
• Wash tablets down well — take them with plenty of water while sitting or standing; otherwise, with a very dry mouth, tablets easily get stuck in the oesophagus. Tips under How to swallow tablets.
• Check for drying medicines — ask with every new prescription and with over-the-counter products such as sleeping or allergy tablets. Keep your digital medication plan up to date.
• Stay active — adapted endurance training is the best-evidenced remedy for the exhaustion.
• Connect with others — the Deutsche Rheuma-Liga (German Rheumatism League) offers information, courses and self-help groups, including some specifically for Sjögren's syndrome.

For most people, Sjögren's syndrome is a condition they can live with well for a long time — provided the eyes and teeth are consistently protected and the rare serious courses are detected early. That is exactly what regular check-ups are for.

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