Sjögren's syndrome is a long-term autoimmune disease in which the immune system attacks the glands that make tears and saliva. The three most common signs are dry eyes, dry mouth and deep fatigue, often with joint pain. It can also affect the skin, lungs, kidneys, nerves and blood. The name "Sjögren's disease" is now preferred by specialists, but "Sjögren's syndrome" is still the name used by the NHS.
The Sjögren's Foundation (US) estimates that about 4 million Americans live with it, and 9 in 10 people diagnosed are women, usually between 45 and 55. Diagnosis combines blood tests (anti-SSA/Ro, ANA, rheumatoid factor), a tear test, a saliva test and sometimes a lip biopsy. There is no cure yet, but treatment controls dryness and protects the eyes, teeth and organs, and the first targeted drugs have now succeeded in phase 3 trials.
Key takeaways
- Sjögren's syndrome mainly causes dry eyes, dry mouth, fatigue and joint pain, but it can involve the lungs, kidneys, nerves, skin and blood.
- The anti-SSA/Ro antibody is the key blood test; a lip biopsy can confirm the disease when antibodies are negative.
- Treatment combines eye drops, saliva care, dental prevention and, for whole-body disease, drugs such as hydroxychloroquine or rituximab.
- Most people have a normal life span, but the risk of lymphoma is several times higher than average: swollen glands that do not go down need a check.
- Women with anti-Ro antibodies who become pregnant need fetal heart monitoring, because about 2% of babies develop congenital heart block.
What is Sjögren's syndrome?
In Sjögren's syndrome, white blood cells called lymphocytes gather in the salivary and tear glands and damage them. The eyes, mouth, nose, skin and vagina become dry, and the immune system makes autoantibodies, mainly anti-SSA/Ro and anti-SSB/La.
The disease can occur alone or together with another autoimmune disease. About half of people with Sjögren's also have a condition such as rheumatoid arthritis, lupus or scleroderma. Doctors now call this "associated" rather than "secondary" Sjögren's.
It is easily missed because dryness develops slowly: the Sjögren's Foundation reports that the average time to diagnosis fell from more than 6 years in 2012 to 2.8 years. See also our guide to autoimmune disease symptoms and lab tests.
What causes Sjögren's syndrome and who is at risk?
What causes Sjögren's syndrome is not fully known. Genes, hormones and possibly a virus activate the salivary gland cells, which release type I interferons and attract T and B cells. A B-cell survival signal called BAFF keeps self-reactive B cells alive, which is why many new drugs target B cells.
Known risk factors:
- being female: women are affected about 10 times more often than men;
- age: symptoms usually begin between 45 and 55, although children and older adults can be affected;
- another autoimmune disease, such as rheumatoid arthritis, lupus or psoriatic arthritis;
- a family history of autoimmune disease;
- possible viral triggers named by Cleveland Clinic: hepatitis C, cytomegalovirus, Epstein-Barr virus, HTLV-1 and COVID-19.
Hepatitis C and IgG4-related disease can mimic Sjögren's, so a hepatitis C antibody test is part of the workup if you have risk factors.
Sjögren's symptoms and warning signs
What are three signs of Sjögren's syndrome? In a Sjögren's Foundation survey of 6,360 patients, the most frequent symptoms were dry eyes (95%), dry mouth (93%) and fatigue (90%), followed by joint pain (81%) and brain fog (77%).
Eyes and mouth
- Eyes: burning, itching, a gritty feeling, redness, sensitivity to light.
- Mouth: thick or stringy saliva, trouble swallowing dry food, loss of taste, hoarseness, mouth sores.
- Teeth and gums: new cavities, gum inflammation and oral thrush (candida).
Symptoms in the rest of the body
- Fatigue, brain fog, and joint or muscle pain.
- Dry skin, rashes, and small purple spots on the legs (purpura) from vasculitis.
- Raynaud phenomenon: fingers turning white or blue in the cold.
- Numbness, tingling or burning pain from nerve damage (neuropathy).
- Dry cough or shortness of breath: lung involvement affects up to 20% of patients, according to the Sjögren's Foundation.
- Heartburn, dry nose with nosebleeds, vaginal dryness and painful sex.
If your tests are normal, our guide to fibromyalgia explains how doctors tell the two apart.
Sjögren's symptoms in women
Because 9 in 10 patients are women, Sjögren's symptoms in women are the classic picture: dry eyes and mouth, fatigue and joint pain, plus vaginal dryness, pain during sex and repeated yeast infections.
What a flare feels like
Flares are periods of days or weeks when symptoms worsen: heavier fatigue, more joint pain, worse dryness, swollen glands, low-grade fever or a rash. A symptom diary helps your rheumatologist adjust treatment.
Diagnosis: how doctors confirm Sjögren's syndrome
There is no single test for Sjögren's. Johns Hopkins describes the diagnosis as proof of an autoimmune process affecting the tear and salivary glands, after other causes of dryness are ruled out. A rheumatologist usually leads, with an eye doctor and a dentist. The workup combines blood tests, a tear test (Schirmer test) with an eye surface stain, a saliva flow test (sialometry) and, when needed, a lip biopsy or a salivary gland ultrasound.
Sjögren's blood tests
In the Sjögren Big Data Project, which analysed 10,500 patients from 22 countries at diagnosis, the frequency of positive markers was:
- ANA (antinuclear antibodies): 79.3%. Our article on the ANA blood test explains titers and patterns;
- anti-SSA/Ro: 73.2%, the antibody that counts in the official criteria;
- rheumatoid factor (RF): 48.6%;
- anti-SSB/La: 45.1%;
- low C3 complement: 13.4%, low C4: 14.5%;
- cryoglobulins: 7.3%.
These antibodies also occur in lupus or rheumatoid arthritis, and some healthy people have a positive ANA, especially women over 65. That is why doctors order an autoimmune panel and read it with your symptoms, never alone.
Inflammation markers give extra clues but are not specific. The ESR (sedimentation rate) can rise in autoimmune diseases, anaemia and infections, and a clearly high CRP makes doctors look for an infection. A high IgG level can reflect the B-cell overactivity of Sjögren's: our guide to the IgG blood test explains the ranges. A complete blood count can show low white cells, low lymphocytes or low platelets.
Schirmer test, eye staining and saliva flow
The Schirmer test places a thin strip of filter paper under the lower eyelid for 5 minutes and measures how far the tears wet it. An eye doctor may also apply a dye (lissamine green or rose bengal) to show dry, damaged spots on the eye surface, scored as the ocular staining score. For the salivary flow test, you collect all the saliva you make over a set period, without chewing or stimulating it.
Lip biopsy and focus score
A lip biopsy removes a few tiny minor salivary glands, usually from the inside of the lower lip, through a small cut. A pathologist looks for clusters ("foci") of lymphocytes and counts them per 4 square millimeters of gland tissue. A focus score of 1 or more is typical of Sjögren's. The biopsy is most useful when anti-SSA/Ro is negative.
The 2016 ACR/EULAR classification criteria
The American College of Rheumatology (ACR) and EULAR criteria give points to five findings. A person with dryness or other suggestive signs who scores 4 or more meets the criteria. In the validation cohort, they had 96% sensitivity and 95% specificity.
| Finding | Threshold | Points |
|---|---|---|
| Lip biopsy: focal lymphocytic sialadenitis | Focus score 1 or more per 4 mm² | 3 |
| Anti-SSA/Ro antibody | Positive | 3 |
| Ocular staining score | 5 or more (or van Bijsterveld 4 or more) | 1 |
| Schirmer test | 5 mm or less in 5 minutes | 1 |
| Unstimulated whole saliva flow | 0.1 mL per minute or less | 1 |
In practice, this means a diagnosis needs either a positive anti-SSA/Ro test or a positive lip biopsy, plus at least one of the gland tests. Anti-SSB/La, ANA and RF do not count on their own. The Sjögren's Foundation stresses that these criteria were built for research: some people who do not meet them still have Sjögren's.
How to read your Sjögren's test results
Normal ranges vary between laboratories: always compare with the range printed on your report.
| Test | Usual normal range (traditional units / SI units) | Typical finding in Sjögren's |
|---|---|---|
| Anti-SSA/Ro, anti-SSB/La | Negative | Anti-Ro positive in about 73%, anti-La in about 45% |
| ANA | Negative (low titers can occur in healthy people) | Positive in about 79% |
| Rheumatoid factor | Below your lab's cutoff | Positive in about half |
| ESR | Women under 50: below 20 mm/h; over 50: below 30 mm/h. Men: below 15 and 20 mm/h (same in SI) | May be raised; not specific |
| CRP | Below 0.3 mg/dL (below 3 mg/L) in most healthy adults | Not specific; a clearly high CRP calls for a search for infection |
| IgG | 700 to 1,600 mg/dL (7.0 to 16.0 g/L) | Can be high (B-cell overactivity) |
| Complement C3 | 88 to 201 mg/dL (0.88 to 2.01 g/L) | Low in about 13%: a sign of more active disease |
| Complement C4 | Within your lab's range | Low in about 15%: linked with lymphoma risk |
| Cryoglobulins | Not detected | Present in about 7%: linked with vasculitis and lymphoma |
| Schirmer test | More than 5 mm in 5 minutes | 5 mm or less |
| Unstimulated saliva flow | More than 0.1 mL/min | 0.1 mL/min or less |
| Lip biopsy focus score | Below 1 focus per 4 mm² | 1 or more |
Low complement and cryoglobulins signal more active disease: in the Big Data Project, the average activity score was 17.7 with cryoglobulins versus 3.8 with negative markers. Our guide to the C4 complement test explains what a low value means. Protein electrophoresis, part of the standard workup, looks for abnormal antibody proteins.
Sjögren's syndrome treatment
There is no cure, and as of November 2026 no disease-modifying drug is licensed specifically for Sjögren's. Treatment follows the EULAR recommendations (2019) and the British Society for Rheumatology guideline (2024): conserve, replace and stimulate secretions, prevent damage, and calm whole-body disease when it is active.
Dry eyes
- Artificial tears during the day, gels or lubricating ointments at night.
- Prescription anti-inflammatory drops such as ciclosporin (Ikervis), which a specialist eye doctor can prescribe.
- Tear duct plugs (punctal plugs) to keep tears on the eye longer.
- Serum tear drops or short courses of steroid drops for severe cases.
Dry mouth and teeth
- Sip water often, chew sugar-free gum, and use saliva substitutes.
- Pilocarpine tablets (Salagen), which stimulate the remaining glands through muscarinic receptors.
- Careful brushing and flossing, mouth rinses recommended by your dentist, and regular dental checkups.
- Antifungal treatment for thrush, such as sugar-free miconazole or nystatin.
- Avoid drying medicines when you can, such as antihistamines and decongestants, and limit alcohol.
Joint pain, fatigue and whole-body disease
For joint pain, fatigue and other whole-body symptoms, doctors often use hydroxychloroquine. Our article on hydroxychloroquine blood levels and heart safety explains the monitoring it needs. Over-the-counter pain relievers (NSAIDs or paracetamol) can help short term; Cleveland Clinic advises not taking them more than 10 days in a row without medical advice.
When organs are involved, a rheumatologist may add short courses of corticosteroids or immunosuppressants such as methotrexate, azathioprine or mycophenolate. Rituximab, which removes B cells, is used for severe involvement, and IVIG for some nerve problems.
Daily habits that help
Use a humidifier (the Sjögren's Foundation suggests 55% to 60% indoor humidity), keep water at hand, and use vaginal moisturizers or lubricants for vaginal dryness; some women are offered oestrogen therapy.
Complications: lymphoma and organ involvement
Lymphoma risk
Sjögren's raises the risk of non-Hodgkin lymphoma, a cancer of lymphocytes. A 2024 meta-analysis of 15 cohort studies (50,308 people) found a risk about 9 times higher than in the general population (standardized incidence ratio 8.78). A 2022 meta-analysis found the overall cancer risk about doubled, mostly from blood cancers. The American College of Rheumatology still describes lymphoma as a rare complication, but its warning signs should never be ignored.
A systematic review of over 15,000 patients identified the most consistent predictors of lymphoma: swollen lymph nodes, persistent enlargement of the parotid glands (in front of the ears), palpable purpura, low C4 and cryoglobulins. Anti-Ro, ANA and RF did not predict lymphoma. Our guide to lymphoma symptoms and tests lists the signs to watch: night sweats, unexplained fever and weight loss, and lumps in the neck, armpits or groin.
Other organs
- Lungs: dry cough or shortness of breath, sometimes interstitial lung disease (scarring of lung tissue).
- Kidneys: involvement is often silent, so it is picked up by blood and urine tests rather than symptoms.
- Nerves: peripheral neuropathy, and less often brain or spinal cord involvement.
- Blood vessels: vasculitis with purpura, particularly with cryoglobulins.
- Thyroid: thyroid tests are part of the workup, since many patients have a second autoimmune condition (see hypothyroidism).
Sjögren's syndrome and pregnancy
Most babies of mothers with Sjögren's are born healthy. The main issue is the anti-SSA/Ro antibody, which crosses the placenta and can cause neonatal lupus in the baby. The most serious form is congenital heart block, damage to the baby's heart rhythm system.
- In 10 prospective studies of 1,248 pregnancies in anti-Ro or anti-La positive mothers, heart block occurred in 1.9%.
- The highest-risk window is between 16 and 28 weeks of pregnancy, and some cases occur later, so fetal heart monitoring may continue past 24 weeks.
- After one affected baby, the risk in the next pregnancy is about 18%. In the PATCH trial, hydroxychloroquine 400 mg a day started before week 10 lowered it to 7.4%.
- Fewer than one in three mothers of affected babies already had a diagnosed rheumatic disease: many learn they carry anti-Ro antibodies only then.
Planning a pregnancy? See your rheumatologist and obstetrician first. Our guide to blood tests during pregnancy explains the usual prenatal checks.
When to see a doctor
See your GP, who can refer you to a rheumatologist, if you:
- have dry eyes or a dry mouth that lasts for weeks or keeps coming back, especially with fatigue or joint pain;
- notice new cavities despite good brushing;
- have a positive ANA or anti-SSA/Ro result and are not sure what it means;
- are pregnant or planning a pregnancy and have Sjögren's or anti-Ro antibodies.
Get a prompt appointment for a salivary gland or lymph node that stays swollen, night sweats, unexplained fever or weight loss, purple spots on the legs, numbness, or a new cough or shortness of breath. Eye pain with sudden blurred vision needs same-day eye care. Call 999 if you cannot breathe or swallow, have chest pain, or have sudden weakness on one side of the body.
Latest scientific advances in Sjögren's syndrome
Ianalumab: the first positive phase 3 trials
Ianalumab is an antibody that removes B cells and blocks the BAFF receptor. In the NEPTUNUS-1 (275 patients) and NEPTUNUS-2 (504 patients) trials (NEJM, October 2026), monthly injections of 300 mg reduced disease activity more than placebo at week 48, but the difference was small (1.0 to 1.3 points on the ESSDAI scale), and dryness and fatigue scores did not improve more than with placebo.
Nipocalimab lowers harmful antibodies
Nipocalimab blocks the neonatal Fc receptor, lowering IgG autoantibodies. In the phase 2 DAHLIAS trial (Lancet 2025, 163 anti-Ro positive patients, 93% women), the 15 mg/kg dose reduced disease activity versus placebo, with a similar safety profile. A phase 3 trial with 655 participants (NCT06741969) is underway, with results expected around 2028.
Blocking the CD40 pathway: dazodalibep and iscalimab
Dazodalibep blocks CD40 ligand, a signal between T cells and B cells. In a phase 2 trial (Nature Medicine 2024), it improved both disease activity and patient-reported symptoms. Two phase 3 trials by Amgen have finished enrolling: one in 651 patients with systemic disease (NCT06104124) and one in 434 patients with a heavy symptom burden (NCT06245408). Iscalimab, an antibody against CD40, also reduced disease activity in the phase 2b TWINSS trial (Lancet 2024).
A new name and better trial tools
The 2023 International Rome consensus, published in Nature Reviews Rheumatology in 2025, made "Sjögren disease" the official name and replaced "secondary" with "associated". A 2026 network meta-analysis of 27 trials (2,261 participants) found that several drugs lowered disease activity, but none clearly improved patient-reported symptoms yet. Telitacicept, which blocks the B-cell signals BAFF and APRIL, has also reported positive phase 3 results, and deucravacitinib, a TYK2 inhibitor, is being tested in 774 patients (NCT05946941).
Which tests can help?
Tests often ordered together:
- ANA, anti-SSA/Ro and anti-SSB/La, rheumatoid factor and anti-CCP antibodies;
- ESR and CRP, IgG and serum protein electrophoresis;
- complement C3 and C4, and cryoglobulins;
- a complete blood count, liver and kidney tests, urinalysis and thyroid tests;
- hepatitis C and HIV tests if you have risk factors;
- a Schirmer test, a saliva flow test and, if needed, a lip biopsy.
After diagnosis, your rheumatologist may repeat the blood count, complement and protein electrophoresis to follow the disease.
Glossary
- Anti-SSA/Ro and anti-SSB/La: autoantibodies typical of Sjögren's; anti-Ro is the one that counts in the criteria.
- ANA: antinuclear antibodies, a screening test for autoimmune diseases.
- Schirmer test: a paper strip test that measures tear production in 5 minutes.
- Focus score: the number of clusters of lymphocytes per 4 mm² of lip gland tissue.
- Cryoglobulins: proteins that clump in the cold and can inflame blood vessels.
- ESSDAI: the EULAR Sjögren's syndrome activity index, scored from 0 to 123, used in trials.
- Congenital heart block: damage to the electrical system of a baby's heart, caused by maternal anti-Ro antibodies.
Frequently asked questions
What are three signs of Sjögren's syndrome?
The three most common signs are dry eyes, dry mouth and fatigue, reported by 95%, 93% and 90% of patients in a large Sjögren's Foundation survey. Swollen salivary glands are another clue.
What is the life expectancy of someone with Sjögren's syndrome?
For most people, Sjögren's is not life-threatening and life expectancy is close to normal. A 2021 meta-analysis of 14 studies (14,584 patients) found a death rate about 1.5 times higher than average overall, with higher risk in older people, men, and people with vasculitis, interstitial lung disease, low complement, anti-La antibodies or cryoglobulins.
What does Sjögren's do to your face?
It can make the parotid glands in front of the ears or under the jaw swell, giving the cheeks a fuller look. The eyes may look red, and the lips and skin dry. A gland that stays swollen for weeks should be checked, because it can be a lymphoma warning sign.
What does a Sjögren's flare feel like?
Usually like crushing fatigue with more joint and muscle pain, worse dryness, brain fog, swollen glands and sometimes a low fever or rash, for days to weeks. Tell your rheumatologist, who may check blood tests and adjust treatment.
Can Sjögren's syndrome kill you?
Rarely. Most people live a normal life span. The serious risks are lymphoma, severe lung or kidney involvement and vasculitis, which is why doctors monitor blood counts, complement and protein electrophoresis and examine the glands and lymph nodes.
Is there a blood test for Sjögren's?
No single blood test confirms it. Anti-SSA/Ro, positive in about 73% of patients, is the most useful; ANA, RF and anti-SSB/La add support. A negative result does not rule it out: a lip biopsy can confirm it.
What does a positive anti-SSA antibody mean?
A positive anti-SSA/Ro (also written anti-Ro or anti-SSA antibody) means your immune system makes antibodies against a protein in your own cells. It is common in Sjögren's and lupus and can occur in people without symptoms. If you could become pregnant, your doctor will plan heart monitoring for the baby.
Sources
- Shiboski CH et al. — 2016 ACR/EULAR classification criteria for primary Sjögren's syndrome — Ann Rheum Dis, 2017 — doi.org
- Brito-Zerón P et al. — Immunological profile and phenotype of 10,500 patients (Sjögren Big Data Project) — Clin Exp Rheumatol, 2018 — pubmed.ncbi.nlm.nih.gov
- Ramos-Casals M et al. — EULAR recommendations for the management of Sjögren's syndrome — Ann Rheum Dis, 2020 — doi.org
- Price EJ et al. — British Society for Rheumatology guideline on Sjögren disease — Rheumatology (Oxford), 2025 — doi.org
- Ramos-Casals M et al. — 2023 International Rome consensus for the nomenclature of Sjögren disease — Nat Rev Rheumatol, 2025 — doi.org
- Ritter J, Dörner T — Pathophysiology of primary Sjögren's syndrome — Z Rheumatol, 2026 — doi.org
- Ansari N et al. — Primary Sjögren's syndrome and non-Hodgkin's lymphoma: meta-analysis of cohort studies — Clin Rheumatol, 2024 — doi.org
- Zhong H et al. — Primary Sjögren's syndrome and risk of malignancies: meta-analysis — Autoimmun Rev, 2022 — doi.org
- Nishishinya MB et al. — Lymphoma predictors in primary Sjögren's syndrome: meta-analysis — Rheumatol Int, 2015 — doi.org
- Huang H et al. — Mortality in primary Sjögren's syndrome: meta-analysis — Rheumatology (Oxford), 2021 — doi.org
- Makadia L et al. — Autoimmune congenital complete heart block: how late can it occur? — AJP Rep, 2023 — doi.org
- Manolis AA et al. — Congenital heart block: pace earlier than later — Trends Cardiovasc Med, 2020 — doi.org
- Izmirly P et al. — Hydroxychloroquine to prevent recurrent congenital heart block (PATCH) — J Am Coll Cardiol, 2020 — doi.org
- Mariette X et al. — Ianalumab in Sjögren's syndrome (NEPTUNUS-1 and -2) — N Engl J Med, 2026 — doi.org
- Noaiseh G et al. — Nipocalimab in Sjögren's syndrome (DAHLIAS) — Lancet, 2025 — doi.org
- St Clair EW et al. — Dazodalibep in Sjögren's syndrome, phase 2 — Nat Med, 2024 — doi.org
- Fisher BA et al. — Iscalimab in Sjögren's syndrome (TWINSS) — Lancet, 2024 — doi.org
- Yang M et al. — Pharmacological treatment for Sjögren's syndrome: network meta-analysis — Z Rheumatol, 2026 — doi.org
- Patel V et al. — Clinical trials and new therapies in Sjögren's syndrome — Curr Opin Immunol, 2026 — doi.org
- ClinicalTrials.gov — Nipocalimab, phase 3 — NCT06741969 — clinicaltrials.gov
- ClinicalTrials.gov — Dazodalibep, systemic disease, phase 3 — NCT06104124 — clinicaltrials.gov
- ClinicalTrials.gov — Dazodalibep, symptom state, phase 3 — NCT06245408 — clinicaltrials.gov
- ClinicalTrials.gov — Deucravacitinib (POETYK SjS-1), phase 3 — NCT05946941 — clinicaltrials.gov
- Sjögren's Foundation — Understanding Sjögren's — sjogrens.org
- American College of Rheumatology — Sjögren's Disease — rheumatology.org
- Cleveland Clinic — Sjögren's Syndrome — my.clevelandclinic.org
- MedlinePlus — Sjögren syndrome — medlineplus.gov
- Johns Hopkins Sjögren's Center — Diagnosis of Sjögren's Disease — hopkinssjogrens.org
- MedlinePlus — Sedimentation rate — medlineplus.gov
- MedlinePlus — C-reactive protein — medlineplus.gov
- MedlinePlus — Quantitative immunoglobulins — medlineplus.gov
- MedlinePlus — Complement component 3 — medlineplus.gov
- NHS — Sjögren's syndrome — National Health Service (England) — nhs.uk
Further reading
- Autoimmune panel: ANA, RF and anti-CCP tests
- Antinuclear antibodies (ANA) blood test guide
- What is lupus? Causes, symptoms and treatment
- Rheumatoid arthritis: symptoms and treatment
- Autoimmune disease symptoms and lab tests
- C3 complement blood test: reading your results
- Lymphoma symptoms: causes, tests and treatment
Understand your lab results with AI DiagMe
A Sjögren's workup often produces a long report: ANA with titer and pattern, anti-SSA/Ro and anti-SSB/La, rheumatoid factor, ESR and CRP, IgG, complement, protein electrophoresis and a blood count. AI DiagMe reads your lab report in minutes, explains each result in plain language, shows what needs follow-up and suggests questions to ask your doctor. Upload your lab results to AI DiagMe for a clear, personal report.
