Sjogren's syndrome statistics

Here you will find a collection of facts about Sjogren's syndrome that I have collected over the years. They are in random order. The date has been included where possible. 

In general, statistics are useful in helping us see and understand the overall health of those with certain conditions. They help us summarize and simplify complex populations. They keep us up to date. With these Sjogren's statistics, based on scientific evidence, we can see general trends and how we fit into them. They may provide fact-based answers to our questions and often give us reassurance that something is considered within the normal range for someone with SS. They can help us understand certain risks that we should be aware of.

Sjögren's syndrome frequency appears to increase with age, with a prevalence of about 3% in people above the age of 50 yrs. ~ Drosos AA, Andaonopoulos AP, Costopoulos JS, et al. Prevalence of primary Sjogren's syndrome in an elderly population.

It was estimated that in 2018 there were around 2.3 million people worldwide living with Sjögren’s syndrome. This number is predicted to increase to 2.5 million by the year 2027. ~ Statistia

Sjogren’s syndrome is the third most common rheumatic autoimmune disorder, behind only rheumatoid arthritis and systemic lupus erythematosus. ~ Rare Genomics Institute, 2021.

Kamitaki et al. (2020) concluded that sex differences in complement protein levels may explain the greater risk in women of Sjogren syndrome and lupus. ~ OMIM Johns Hopkins University.

Sjogren syndrome is far from a rare disorder with an incidence approaching approximately one-half of that of rheumatoid arthritis (RA) or affecting 0.5% to 1.0% of the population. Between 400,000 and 3.1 million adults have Sjögren's syndrome.9 Aug 2021 ~ Sjogren Syndrome by Steven E. Carsons; Bhupendra C. Patel

In 9-20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are ILD (interstitial lung disease) and airways disease. ~ The Pulmonary Fibrosis Foundation

The extra-glandular manifestations occur in 30 to 40% of primary SS patients, involving lung, heart, kidney, nervous system, and lymphoproliferative disorders. ~ 2021 Drs Du, Han, Zhu, Xiao, Huang, Che, Tang, Zou, Jiang, and Lu.

Sjögren’s syndrome: Risk factors for more severe disease included male sex, earlier age at diagnosis, black/African-American (BAA) ethnicity, and living in more southerly countries, including below the equator. The authors hailed these factors as potentially helping to predict the presence of systemic disease in newly-diagnosed patients, as well as helping to determine the optimum follow-up strategy. SOURCE: Brito-Zerón P et al. Rheumatology. 2019 Dec 24. doi: 10.1093/rheumatology/kez578.

Primary Sjögren's syndrome (pSS) is a chronic, systemic autoimmune disease. It is the second most common rheumatic autoimmune disorder, affecting 0.7% of European Americans and up to 1% of people globally. ~ Drs Chowdhury F, Tappuni A and Bombardieri M (2021)

What is the prevalence of Sjogren’s Syndrome? It is most common amongst females, who account for 90% of all Sjogren Syndrome cases, and typically affects individuals between 40 and 60. 200K to 3M US cases per year. Few studies report the incidence of the syndrome varies between 3 and 6 per 100,000 per year. ~ 2021 Rare Genomics Institute.

By examining data from a 40-year period (1976-2016), the team has found that the disease affects 0.02 to 0.1 percent of the adult population, with a progressive increase of the rate of new cases over the last four decades. Further analyses of this cohort are ongoing. ~ the Rochester Epidemiology Project.

By ethnicity, the highest ESSDAI scores occurred in black/African-American patients (6.7), followed by white (6.5), Asian (5.4), and Hispanic (4.8). ESSDAI is European League Against Rheumatism Sjögren’s syndrome disease activity index. ~ Big data breaks down Sjögren’s syndrome. Publish date: January 17, 2020 By Jim Kling

Sjogren syndrome is far from a rare disorder with an incidence approaching approximately one-half of that of rheumatoid arthritis (RA) or affecting 0.5% to 1.0% of the population. Between 400,000 and 3.1 million adults have Sjögren's syndrome. ~ Sjogren Syndrome by Steven E. Carsons; Bhupendra C. Patel. Last Update: February 25, 2021.

Around half the people who have Sjogren’s syndrome also have some form of arthritis (conditions affecting the joints, such as lupus and rheumatoid arthritis) according to Arthritis Australia.

Sjogren syndrome - Up to one-half of affected individuals also develop extra-glandular involvement in organs such as the joints, skin, lungs, gastrointestinal (GI) tract, nervous system, and kidneys. ~ Professor Steven Carsons, 2021 StatPearls

Primary Sjögren’s in the elderly Although the mean age of onset of pSS is usually in the 4th to 5th decade, onset of the disease in the 6th or 7th decade is not uncommon. An Italian cohort of 322 consecutive patients with pSS between 1990 and 2007 was studied retrospectively, where pSS was noted in 6% of adults over 65 years of age. A Spanish cohort of 223 consecutive pSS patients noted the disease in 14% of adults aged 70–87 years.

Neurological Symptoms Strike One in Five Primary Sjogren Patients according to a 2016 study - a French ASSESS (Assessment of Systemic Signs and Evolution in Sjogren's Syndrome)

Despite a prevalence ranging between 1% and 3% of the general population, more than 50% of Sjogren's patients has not received a correct diagnosis and approximately 30% of patients presenting other autoimmune diseases suffered from pSS. ~ Sjögren's syndrome, the old and the new. Peri Y, Agmon-Levin N, Theodor E, Shoenfeld Y (2012)

The estimated prevalence of SS (including primary and secondary forms) in the adult population in Spain was 0.33% in this study. Extrapolating to the entire population of the country aged 20 years or older (around 37.7 millions) there would be approximately 125,000 cases of SS in Spain. Based on these results, SS cannot be considered a rare disease. Nature article.

A third of children with childhood Sjögren's have symptoms of dry mouth and dry eyes. Uniquely, there is much higher rate of recurrent and persistent parotitis in childhood presentations. Parotitis is inflammation of the salivary gland by the cheek. ~ Sjögren's Foundation

American Indians have a Higher Risk of Sjögren's Syndrome and more Disease activity than European Americans and African Americans. 2019 study. ~ Wiley Online Library

"Among those with swallowing disorders, 42% sought treatment, with approximately half reporting improvement." From Swallowing Disorders in Sjögren's Syndrome: Prevalence, Risk Factors, and Effects on Quality of Life PMID: 26482060 DOI: 10.1007/s00455-015-9657-7

A 2015 meta-analysis of epidemiological studies in primary SS, computed an overall prevalence of 60.82 per 100,000 inhabitants worldwide or 1 person in 1,644. Based on these results, pSS should no longer be considered a “rare disease”. The European Commission on Public Health defines a rare disease as a condition with prevalence below 1 in 2,000 persons. The question of whether pSS is rare or not has important consequences, not only for the public health system management of disease burden, but also for the development of future therapeutic agents for this indication. Info from Narváez, J., Sánchez-Fernández, S.Á., Seoane-Mato, D. et al. Prevalence of Sjögren’s syndrome in the general adult population in Spain: estimating the proportion of undiagnosed cases. Sci Rep 10, 10627 (2020).

"costs for cases were 2.7 times higher than matched controls" Conclusion: The incremental healthcare burden of Sjogren's Syndrome is high (averaging $7,535/Per Year in the year after diagnosis), and is sustained over time. This was from Longitudinal, Incremental Healthcare Costs of Sjogren’s Syndrome in British Columbia, Canada: A General Population-Based Cohort Study. They estimated the incremental healthcare costs of a general population-based cohort of incident SS for five years after diagnosis.

The European Commission on Public Health defines a rare disease as a condition with prevalence below 1 in 2,000 persons. In most studies conducted in Europe Sjogren's syndrome #SS would not be considered a rare disease.

Presence of anti-SSA and anti-SSB antibodies is associated with a younger age of onset of primary Sjogren's Syndrome #pSS and longer disease duration. ~ NCBI

Primary Sjögren’s in children #pSS is rare in children; only 81 cases have been reported in the literature from 2000–2010. The age of diagnosis ranges from 4 to 16 years (mean age of 9.84 years). Female to male ratio of 5:1 is observed in children. ~ de Souza TR, Silva IH, Carvalho AT, et al. Juvenile Sjögren syndrome: distinctive age, unique findings. Pediatr Dent. 2012;34:427–430.

Although the mean age of onset of primary Sjogren's syndrome #pSS is usually in the 4th to 5th decade, onset of the disease in the 6th or 7th decade is not uncommon. ~ NCBI

This study shows that health expenses of patients with Sjogren's Syndrome are double of primary care patients and similar to those of patients with rheumatoid arthritis. ~ Direct healthcare costs and predictors of costs in patients with primary Sjogren’s syndrome. ~ Direct healthcare costs and predictors of costs in patients with primary Sjogren's syndrome

Sjögren syndrome is a relatively common disorder; it occurs in 0.1 to 4 percent of the population. It is difficult to determine the exact prevalence because the characteristic features of this disorder, dry eyes and dry mouth, can also be caused by many other conditions. ~ Genetics Home Reference

Sjögren's syndrome: This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects ten times as many women as men. About half of patients also have rheumatoid arthritis or other connective tissue diseases, such as lupus. ~ American College of Rheumatology.

Audiovestibular involvement in patients with SS has been reported in the literature with a prevalence ranging from 22% to 46%. Find out more facts here and learn all about TINNITUS

Over a 15-20 year period of observation, 8-15% of Sjögren’s patients develop a lymphoma. Screening for symptoms of early lymphoma include asking a patient about swollen glands, fevers, weight loss and new onset of fatigue. A physical examination can detect lymph nodes, evidence for a “wasted” appearance, or an enlarged spleen. ~ Dr Daniel J. Wallace at Sjögren’s Syndrome Foundation, Inc

The prevalence rate of Sjogren’s syndrome is estimated to be 0.1 to 4% of the global population. The major symptoms of Sjögren’s syndrome are dryness in eyes and mouth followed by joint pain, swollen salivary glands and others. ~ DataBridge Market Research.

Laboratory abnormalities in Sjogren’s Syndrome: Elevated protein levels will be seen in 80 percent of those with SS. ~ The Lupus Foundation of America

"more than 50% of patients with neurological manifestations of Sjögren’s syndrome may not have autoantibodies. In patients who have neuropathy and compelling glandular symptoms of dry eyes and dry mouth, negative blood tests for SS-A and SS-B antibodies do not exclude the diagnosis of Sjögren’s syndrome." ~ Johns Hopkins Sjögren’s Center

In a US survey, of 2,962 respondents with Sjögren’s syndrome, it was found that 31 percent of them were also diagnosed with hypertension.

It was estimated that in 2018 there were around 2.3 million people worldwide living with Sjögren’s syndrome. ~ Statista

Sjögren’s Syndrome is the UK’s second most common autoimmune rheumatic disease, yet the condition remains under recognised and frequently under treated. It does not command a high profile within the medical profession, and the general public is largely unaware of the problems faced by sufferers. In reality, Sjögren’s Syndrome can be a debilitating, distressing and miserable condition.  It affects approximately 0.6% of adults in the UK, with a mean age of 50 years.  90% of patients are women. (BSSA British Sjogren's Syndrome Association)

About 5%-10% of pSS patients in rheumatological care suffer from severe extraglandular manifestations, which generally occur soon after disease onset.~ Epidemiology of primary Sjörgren's syndrome

According to the Sjögren’s Foundation, it currently takes an average of three years to diagnose Sjögren’s. Sjögren’s is often undiagnosed or misdiagnosed because disease symptoms frequently overlap or mimic those of menopause, allergies, or medical conditions such as lupus, rheumatoid arthritis, fibromyalgia, and chronic fatigue syndrome.

The Anti-Nuclear Antibody test is a blood test that can be performed to pinpoint the presence of ANAs, a group of antibodies that react abnormally with the cell nucleus, and is found within 70% of all Sjogren patients. ~ Rare Genomics Institute 2021

By examining data from a 40-year period, the team has found that the disease affects 0.02 to 0.1 percent of the adult population, with a progressive increase of the rate of new cases over the last four decades. ~ Center for Immunology and Immune Therapies at Mayo Clinic.

SS occurs in approximately 1-3% of the general population, but 20-30% of people with lupus. ~ Workshop at Hospital for Special Surgery

About one-third to one-half of people with SS will experience a painless enlargement of a major salivary gland, and this manifestation of the disease is usually bilateral (will occur on both sides). ~ Workshop at Hospital for Special Surgery by Paul D. Freedman, DDS Director, Section of Oral Pathology, New York Hospital Queens.

People with Sjogren’s are twice as likely as those without it to be diagnosed with heart failure, a disorder in which the heart can’t pump enough blood.  These patients should be screened for cardiovascular comorbidities and considered for preventive interventions. ~ Cardiovascular Morbidity and Mortality in Primary Sjögren's Syndrome, 2018.

Arthritis Research UK estimates that there may be up to half a million people in the United Kingdom who have Sjögren's syndrome. (1 Nov 2021)

Obstructive sleep apnea was detected in 84% of people in this study with primary Sjogren's Syndrome who underwent all-night polysomnography (PSG). 2021 study