Materiały źródłowe

• #1 Epidemiology of Sjögren syndrome | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-01057-6

  Sjgren syndrome is a phenotypically varied autoimmune disorder that can occur alone in primary Sjgren syndrome or in association with other connective tissue diseases (CTDs), including rheumatoid arthritis, systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). The estimation of the prevalence and incidence of Sjgren syndrome varies depending on diagnostic criteria and study design, making it difficult to estimate geographical and temporal trends. […] Assessment of the prevalence of pSS varies according to study methodology, but can be estimated at between 0.01% and 0.05%, although some countries might be under-represented in this estimation. […] Sjgren syndrome can be associated with other connective tissue diseases, mainly rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis. […] Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjgrens syndrome.

• #2

  https://link.springer.com/article/10.1007/s40744-023-00611-8

  Primary Sjgrens is a multi-system autoimmune disease affecting patients physical, mental, and emotional wellbeing. The epidemiology of Sjgrens is not well understood, and up-to-date epidemiological evidence is needed to improve knowledge and awareness of Sjgrens among patients and healthcare professionals, and to ascertain the global burden of disease. […] The SLR found that incidence and prevalence of primary Sjgrens was highest in females and in older age groups (65 years), while the average age at onset and diagnosis of primary Sjgrens ranged between 3457 years and 4067 years, respectively. […] This SLR identified a paucity of incidence and prevalence data for primary Sjgrens, highlighting a need for further epidemiological studies. […] The global Sjgrens community must work together to follow the defined classification criteria of primary Sjgrens and reporting guidelines for incidence and prevalence data to allow for meaningful epidemiological comparisons across studies, settings, and countries.

• #2

  https://link.springer.com/article/10.1007/s40744-023-00611-8

  A total of 7/62 (11.3%) studies reported on the incidence of primary Sjgrens within a sample cohort taken from the general population. […] Incidence estimates ranged from 3.53.9 per 100,000 person-years and 0.326.1 per 100,000 persons. […] A total of 9/62 (14.5%) studies reported on the prevalence of primary Sjgrens. […] Prevalence was reported with a variety of metrics and ranged from 0.03%0.77%, 5.510.3 per 10,000 persons, 2232 per 100,000 persons, and 12.4 (unadjusted) or 13.1 (age-adjusted to the US population in 2000) per 100,000 person-years. […] This SLR identified an unmet need for studies on the epidemiology of primary Sjgrens, and in particular, a paucity of incidence and prevalence data across a diverse range of geographies.

• #3 Epidemiology of Sjögren’s: A Systematic Literature Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10796897/

  Primary Sjgrens is a multi-system autoimmune disease affecting patients physical, mental, and emotional wellbeing. The epidemiology of Sjgrens is not well understood, and up-to-date epidemiological evidence is needed to improve knowledge and awareness of Sjgrens among patients and healthcare professionals, and to ascertain the global burden of disease. […] The objective of this research was to conduct a de novo systematic literature review (SLR) to identify and synthesise evidence on global epidemiology of primary Sjgrens. […] This SLR identified a paucity of incidence and prevalence data for primary Sjgrens, highlighting a need for further epidemiological studies. The global Sjgrens community must work together to follow the defined classification criteria of primary Sjgrens and reporting guidelines for incidence and prevalence data to allow for meaningful epidemiological comparisons across studies, settings, and countries.

• #3 Epidemiology of Sjögren’s: A Systematic Literature Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10796897/

  The SLR found that incidence and prevalence of primary Sjgrens was highest in females and in older age groups (65 years), while the average age at onset and diagnosis of primary Sjgrens ranged between 3457 years and 4067 years, respectively. […] This SLR provides an up-to-date summary of the available evidence for the incidence, prevalence, age at symptom onset, and age at diagnosis of primary Sjgrens from a global perspective, following the introduction of the 2016 ACR/EULAR diagnostic criteria. Results from 62 studies suggest that women and those in older age groups have the highest incidence and prevalence of Sjgrens, and that age at symptom onset and diagnosis ranges between 3457 years and 4067 years, respectively. […] This SLR identified an unmet need for studies on the epidemiology of primary Sjgrens, and in particular, a paucity of incidence and prevalence data across a diverse range of geographies. There is a need for the Sjgrens community to align on the methodology used for the classification of Sjgrens and reporting of incidence and prevalence estimates, to allow meaningful epidemiological comparisons across studies.

• #4 Sjogrens Syndrome Market Size and Forecast 2024-2034

  https://www.imarcgroup.com/sjogrens-syndrome-market

  The female-to-male ratio for this illness is observed to be 9:1. […] The prevalence of primary Sjogren syndrome varies depending on the study methodology, although it can be estimated at 0.01% to 0.05%. […] Sjogren syndrome affects persons of all ages, however it is more common in the elderly. Onset usually occurs in the fourth or fifth decade of life. […] Mortality in primary Sjogren syndrome appears to be highest in patients with extra-glandular symptoms, lung involvement, and non-Hodgkin lymphoma. […] What is the number of prevalent cases (2018-2034) of Sjogren’s syndrome across the seven major markets? […] What is the size of the Sjogren’s syndrome patient pool (2018-2023) across the seven major markets? […] What would be the forecasted patient pool (2024-2034) across the seven major markets? […] What are the key factors driving the epidemiological trend of Sjogren’s syndrome? […] What will be the growth rate of patients across the seven major markets?

• #5 Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/74/11/1983

  Epidemiological studies of primary Sjgren’s syndrome (pSS) are crucial for describing the burden to society and the public medical system and for shedding light on aetiology. […] According to a random-effects model, the pooled IR for pSS was 6.92 (95% CI 4.98 to 8.86) per 100000 person-years. The overall PR was 60.82 (95% CI 43.69 to 77.94) cases per 100000 inhabitants with a slightly lower estimate of 43.03 (25.74 to 60.31) cases per 100000 inhabitants when only considering population-based studies. […] Incidence and prevalence rates of pSS vary widely around the world. The results help us better understand the global epidemiology of pSS. Large population-based studies combining meticulous case-finding and case-ascertainment strategies are needed.

• #6 Sjogren Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK431049/

  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 Sjgren’s syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. About half of patients also have rheumatoid arthritis or other connective tissue diseases, such as lupus. […] Sjogren has been reported worldwide in adults and more rarely in children, and there appears to be no racial, or geographic bias in incidence. The disorder, however, has a marked predilection for women and similar to SLE, the female:male ratio is approximately 9:1. The disease usually presents in middle age but may occur in children as well as the elderly. […] As there is no evidence-based standardized screening tool to decide which dry eye patients to refer for Sjogren syndrome workup, there is an underreferral of dry eye patients for Sjogren syndrome workups: hence there is continued underdiagnosis of the disease.

• #7 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology

  https://emedicine.medscape.com/article/332125-overview

  In the United States, Sjgren syndrome is estimated to be the second most common rheumatologic disorder, behind SLE. Sjgren syndrome affects 0.1-4% of the population. This wide range, in part, reflects the lack of uniform diagnostic criteria. […] Internationally, comparative studies between different ethnic groups have suggested that Sjgren syndrome is a homogeneous disease that occurs worldwide with similar prevalence and affects 1-2 million people. […] The female-to-male ratio of Sjgren syndrome is 9:1. Sjgren syndrome can affect individuals of any age but is most common in elderly people. Onset typically occurs in the fourth to fifth decade of life. […] The systemic phenotype of primary Sjgren syndrome is strongly influenced by personal factors (eg, age, gender, ethnicity, place of residence, according to an analysis by the Sjgren Big Data Consortium, a five-continent multicenter registry, of a cohort that included 10,007 patients (9352 female, mean age 53 years) with recorded European League Against Rheumatism’s Sjgren syndrome disease activity index (ESSDAI) scores available. Findings (all P 0.001) were as follows:

• #7 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology

  https://emedicine.medscape.com/article/332125-overview

  – Males had a higher mean ESSDAI than females (8.1 vs 6.0, respectively). […] – Patients diagnosed at 35 years had a higher mean ESSDAI than those diagnosed at 65 years (6.7 vs 5.6). […] – By ethnicity, the highest global ESSDAI scores were reported in blacks/African Americans (6.7), followed by whites (6.5), Asians (5.4), and Hispanics (4.8). […] – Black/African-American patients showed the highest frequencies in the lymphadenopathy, articular, peripheral and central nervous system, and biological domains. […] – White patients showed the highest frequencies in the glandular, cutaneous, and muscular domains. […] – Asian patients showed the highest frequencies in the pulmonary, renal, and hematological domains. […] – Hispanic patients showed the highest frequencies in the constitutional domain. […] – Systemic activity and disease activity was higher in patients from southern countries.

• #8 Sjögren’s Syndrome: Epidemiology, Clinical, Economic, and Humanistic Burden – Marksman Healthcare

  https://marksmanhealthcare.com/case-study/sjogrens-syndrome-epidemiology-clinical-economic-and-humanistic-burden/

  Sjgrens syndrome is the second most common rheumatic autoimmune disorder, and is traditionally classified as primary or secondary, depending on the absence or presence of other major autoimmune diseases. […] Our client was interested in exploring the epidemiology, clinical, economic, and humanistic Burden of illness in patients with Sjgrens syndrome. […] we assisted our client by extracting data pertaining to epidemiology from over 120 articles using the data extraction template provided by the client.

• #9 Sjögren’s syndrome: Epidemiology and clinical manifestations | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-sjogrens-syndrome-epidemiology-clinical-manifestations-S2444440522000723

  There are various population studies that have attempted to describe the incidence and prevalence of SS in different countries; the data are to some extent discordant between one registry and another, and will often depend on the diagnostic criteria used to classify the patients. […] Comparatively, the incidence rates of the studies that used the European classification criteria of 1993 were much lower, compared to those of the studies carried out according to the criteria of the European/American consensus of 2002. […] The global incidence rates range between 3 and 11 cases per 100,000 patients, while the prevalence is around 0.010.72%. […] Most likely these figures are underestimated, since many asymptomatic patients will never be diagnosed. […] As for the affectation by sex, it is much more frequent in women, with a ratio close to 10:1 (woman:man), which was reported in a study that included more than 14,000 patients.

• #9 Sjögren’s syndrome: Epidemiology and clinical manifestations | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-sjogrens-syndrome-epidemiology-clinical-manifestations-S2444440522000723

  However, there are other differences in its behavior with respect to sex: severe ocular involvement predominates in men, while the manifestations and systemic involvement are much greater in women. […] Although it can occur at any age, most patients are between the third and fifth decades of life. […] In children is much rarer, maintaining the predominance in women, although in a lower proportion, with an average age of onset of the disease between 9.4 and 10.7 years, being girls younger at the age of diagnosis. […] A higher prevalence was documented in Asian populations, compared to Caucasian subjects. […] Likewise, variations were found between the studies of one country and another: this is the case of a prevalence of 0.21% in Turkey, where the criteria of the European/American consensus were used; 0.77% in the Chinese population, using the Copenhagen criteria, while the lowest rates were reported in Japan, between 0.03 and 0.05%.

• #9 Sjögren’s syndrome: Epidemiology and clinical manifestations | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-sjogrens-syndrome-epidemiology-clinical-manifestations-S2444440522000723

  Sjgrens syndrome is an entity of rheumatic origin, with complex autoimmune characteristics, in which the salivary and lacrimal glands are mainly compromised. […] Population studies that have attempted to describe the incidence and prevalence of Sjgrens syndrome in various countries throughout the world are to some extent discordant between one registry and another. […] The global incidence of SS is estimated at approximately 7 per 100,000 person-years; however, the estimates of its incidence and prevalence vary widely depending on the specific classification criteria, the study design, and the population examined. […] According to the studies, the highest incidence rates were reported in Europe and Asia. […] In Colombia, Santos et al.39 carried out a study whose main objective was to describe the prevalence of rheumatic diseases and associated factors in the Colombian population over 18 years of age, for which they included data from 6 major cities and used the Copcord (Community Oriented Program for the Control of Rheumatic Diseases) questionnaire adapted for Colombia.

• #9 Sjögren’s syndrome: Epidemiology and clinical manifestations | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-sjogrens-syndrome-epidemiology-clinical-manifestations-S2444440522000723

  The researchers concluded that the prevalence of SS was 0.08%.39 These data are comparable to those published by Fernndez-vila et al.,18 who analyzed slightly more than 58,000 cases of SS, from the database of the official registry of the Colombian Ministry of Health, most of the patients coming from Bogot, Antioquia and Valle del Cauca, and found a total prevalence of 0.12%, which varies widely according to the age group evaluated, being lower in those under 15 years of age and higher in the population between 65 and 69 years of age (0.5%). […] The female population predominated, with a ratio of 4.63:1 (woman:man), which resulted in a prevalence of 0.31% in women and 0.07% in men. […] SS constitutes an entity of autoimmune etiology, of unknown cause until now; not only its marked impact on the quality of life of the patients who present it is well known, but also the burden of disease that it means for caregivers and health systems worldwide.

• #10 The epidemiology of Sjögren’s syndrome | CLEP

  https://www.dovepress.com/the-epidemiology-of-sjoumlgrenrsquos-syndrome-peer-reviewed-fulltext-article-CLEP

  Depending on the studies, female to male ratio varies from as high as 20:1 to 9:1. […] The estimated prevalence of pSS in Beijing, China, is 0.77% using the Copenhagen criteria. […] The prevalence of pSS in the elderly population is between five to eight times higher, depending on the age used to define the population. […] The prevalence rates can vary considerably depending on the classification criteria used. […] The reported prevalence in Greece ranges from 0.09%0.23% and prevalence in Slovenia is estimated at 0.60%. […] The prevalence of sSS in Secondary Sjgrens syndrome and systemic lupus erythematosus (SLE) is wide-ranging, varying from 6.5%19%, which can be partly attributed to the application of different criteria. […] Overall, sSS is a common extra-articular manifestation of RA.

• #10 The epidemiology of Sjögren’s syndrome | CLEP

  https://www.dovepress.com/the-epidemiology-of-sjoumlgrenrsquos-syndrome-peer-reviewed-fulltext-article-CLEP

  The prevalence of sSS in RA patients is reported at 29%. […] The association of SS and anti-neutrophilic cytoplasmic antibodies-associated vasculitides is rare, described in only three cases. […] The lack of standardized criteria also leads to difficulty interpreting epidemiologic studies with a wide range in prevalence, making it challenging to estimate the true incidence and prevalence.

• #11 Prevalence of Sjögren’s syndrome in the general adult population in Spain: estimating the proportion of undiagnosed cases | Scientific Reports

  https://www.nature.com/articles/s41598-020-67462-z

  Even using the same classification criteria for pSS (the 2002 AECG criteria), the prevalence rates in the studies that analyzed this issue are very much influenced by population size, case source, and study design. This makes it a challenge to determine the true prevalence of the disease. The EPISER 2016 study is the first to report the prevalence of pSS in Spain: 0.25% (95% CI 0.150.43) or 1 person in 400. Based on these results, SS cannot be considered a rare (orphan) disease. This study also confirms that a non-negligible proportion of pSS cases in the general population remain undiagnosed. Strategies for outreach and management of such undiagnosed cases are still necessary. Reliable prevalence rates should help improve the planning of health-care and disability compensation in national systems with universal coverage.

• #11 Prevalence of Sjögren’s syndrome in the general adult population in Spain: estimating the proportion of undiagnosed cases | Scientific Reports

  https://www.nature.com/articles/s41598-020-67462-z

  To estimate the prevalence of Sjgrens syndrome (SS) in the adult Spanish population we performed a population-based multicenter cross-sectional study. The estimated prevalence of SS (including primary and secondary forms) in the adult population in Spain was 0.33% (95% CI 0.210.53). Extrapolating to the total population of the country aged 20 years (around 37.7 million persons), there would be around 125,000 cases of SS in Spain. Considering only primary SS, the estimated prevalence was 0.25% (95% CI 0.150.43) or 1 person in 400. Only 50% of cases had already been diagnosed with SS prior EPISER 2016 study, confirming the existence of a non-negligible proportion of undiagnosed cases in the general population. […] The prevalence of SS has never been evaluated in Spain. This observation prompted the Spanish Society of Rheumatology (SER) to include this disease in EPISER 2016, a population-based study to estimate the prevalence of rheumatic diseases in the adult population. The prevalence of pSS in Spain is about three times lower than RA (0.9% in EPISER 2016) but quite similar to SLE (0.21%). Results from the EPISER 2016 confirm the existence of a non-negligible percentage of undiagnosed pSS cases in our community (50% of the patients detected in our study had not been previously diagnosed).

• #12 Prevalence and demographic characteristics of Sjögren’s syndrome in Colombia, based on information from the Official Ministry of Health Registry | Reumatología Clínica

  https://www.reumatologiaclinica.org/en-prevalence-demographic-characteristics-sjogren-s-syndrome-articulo-S2173574320300800

  58,680 cases of Sjgren’s syndrome were identified, with a prevalence in those over 18 years of age of 0.12%; 82% were women, with a female:male ratio of 4.6:1, with a higher prevalence in the age group of 65-69 years. […] We present demographic and epidemiological information on Sjgren’s syndrome in Colombia. There are very few epidemiological studies of this disorder. However, a prevalence similar to that reported in countries of the region such as Brazil (0.17%) and Argentina (0.17%) was documented. […] Epidemiological studies are essential to describe the burden of this disease on society and to clarify any possible factors associated with its aetiology. SS is one of the most common autoimmune conditions, with an estimated worldwide prevalence within a range from 0.05% to 4.6%. […] The higher rate of involvement of the female sex is similar to that reported by other studies, and likewise a concentration of patients was recorded in older age groups (65 to 69 years old).

• #13 Prevalence and demographic characteristics of Sjögren’s syndrome in Colombia, based on information from the Official Ministry of Health Registry | Reumatología Clínica

  https://reumatologiaclinica.org/en-prevalence-demographic-characteristics-sjogren39s-syndrome-articulo-S2173574320300800

  Prevalence and demographic characteristics of Sjgren’s syndrome in Colombia, based on information from the Official Ministry of Health Registry […] To calculate the prevalence and describe the main demographic characteristics of Sjgren’s syndrome in adults in Colombia. […] 58,680 cases of Sjgren’s syndrome were identified, with a prevalence in those over 18 years of age of 0.12%; 82% were women, with a female:male ratio of 4.6:1, with a higher prevalence in the age group of 65-69 years. […] We present demographic and epidemiological information on Sjgren’s syndrome in Colombia. There are very few epidemiological studies of this disorder. However, a prevalence similar to that reported in countries of the region such as Brazil (0.17%) and Argentina (0.17%) was documented. […] Epidemiological studies are essential to describe the burden of this disease on society and to clarify any possible factors associated with its aetiology. SS is one of the most common autoimmune conditions, with an estimated worldwide prevalence within a range from 0.05% to 4.6%.

• #14 Sjögren’s syndrome: Epidemiology and clinical manifestations

  http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0121-81232022000400310

  Although the SS can occur at any age, most patients are between the third and fifth decades of life, with a higher prevalence between 65 and 69 years of age. […] The data are to some extent discordant between one registry and another, and will often depend on the diagnostic criteria used to classify the patients. […] The global incidence rates range between 3 and 11 cases per 100,000 patients, while the prevalence is around 0.01-0.72%. […] Most likely these figures are underestimated, since many asymptomatic patients will never be diagnosed. […] In Colombia, Santos et al. carried out a study whose main objective was to describe the prevalence of rheumatic diseases and associated factors in the Colombian population over 18 years of age, for which they included data from 6 major cities and used the Copcord (Community Oriented Program for the Control of Rheumatic Diseases) questionnaire adapted for Colombia.

• #14 Sjögren’s syndrome: Epidemiology and clinical manifestations

  http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0121-81232022000400310

  Sjgren’s syndrome is an entity of rheumatic origin, with complex autoimmune characteristics, in which the salivary and lacrimal glands are mainly compromised. […] Population studies that have attempted to describe the incidence and prevalence of Sjgren’s syndrome in various countries throughout the world are to some extent discordant between one registry and another. […] The global incidence of SS is estimated at approximately 7 per 100,000 person-years; however, the estimates of its incidence and prevalence vary widely depending on the specific classification criteria, the study design, and the population examined. […] According to the studies, the highest incidence rates were reported in Europe and Asia. […] The researchers calculated a prevalence in people over 18 years of age of 0.12%, of whom 82% were women, with a female:male ratio of 4.6:1.

• #14 Sjögren’s syndrome: Epidemiology and clinical manifestations

  http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0121-81232022000400310

  The researchers concluded that the prevalence of SS was 0.08%. […] The female population predominated, with a ratio of 4.63:1 (woman:man), which resulted in a prevalence of 0.31% in women and 0.07% in men. […] SS is considered a frequent connective tissue disease in which data on the global incidence and prevalence rates are underestimated, and it is clearly much more frequent in women.

• #15 Ethnicity Matters in Sjogren’s Syndrome | MedPage Today

  https://www.medpagetoday.com/rheumatology/generalrheumatology/74334

  The surveillance period was 2007 to 2009, and data were collected from rheumatologists, other clinicians, hospitals, and death registries. […] During the years 2007 to 2009, there were 138 incident cases of physician-diagnosed Sjogren’s syndrome among adult residents of Manhattan, with 84 cases having been diagnosed by a rheumatologist, for overall age-adjusted incidence rates of 3.4 (95% CI 2.9-4) and 3.5 (95% CI 2.9-4.1) per 100,000 person-years, respectively. […] Among women, the age-adjusted incidence rate was 5.7 (95% CI 4.7-6.7) per 100,000, while among men the rate was 1 (95% CI 0.6-1.5) per 100,000 (P<0.001). [...] The capture-recapture analysis added 167 incident cases, suggesting that at least 17% of cases had been missed. This raised the adjusted incidence rate to 4.1 (95% CI 2.5-5.8) per 100,000 person-years.

• #15 Ethnicity Matters in Sjogren’s Syndrome | MedPage Today

  https://www.medpagetoday.com/rheumatology/generalrheumatology/74334

  Among the ethnically diverse residents of Manhattan, certain groups had markedly different incidence rates of primary Sjogren’s syndrome, a retrospective study found. […] Women were more often affected, with age-adjusted incidence rates being highest for Asian women, at 10.5 (95% CI 6.6-15.7) per 100,000 person-years, according to Peter M. Izmirly, MD, of New York University School of Medicine in New York City, and colleagues. […] For white women, the age-adjusted incidence rate was 6.2 (95% CI 4.7-7.9) per 100,000, while for black women the rate was 3.3 (95% CI 1.5-6.3) per 100,000, and for Latinas it was 3.2 (95% CI 1.8-5.2) per 100,000, the researchers reported online in Arthritis Care & Research. […] Few data have been available estimating the incidence and prevalence of the disease, especially among minority groups.

• #15 Ethnicity Matters in Sjogren’s Syndrome | MedPage Today

  https://www.medpagetoday.com/rheumatology/generalrheumatology/74334

  For prevalence, the overall age-adjusted rate was 13.1 (95% CI 11.1-15.1) per 100,000 person-years. […] As with incidence, prevalence was approximately six times higher among women, while the prevalence rates according to ethnicity were 23.8 per 100,000 among white women, 23.7 for Asians, 16.1 for blacks, and 15 among Latinas. […] Capture-recapture added 24 cases, suggesting that 13% of cases might have been missed. […] While the analysis specifically provided rate estimates for Manhattan, the city’s ethnic diversity „also provided epidemiologic estimates for the major racial/ethnic populations in the U.S.,” Izmirly and colleagues observed. […] „Given the already limited number of published studies on primary Sjogren’s syndrome among Asians and Latinos, additional work is needed to better describe and understand the epidemiology of primary Sjogren’s syndrome among specific ethnic subpopulations,” the authors wrote.

• #16 Manifestations and management of Sjögren’s disease | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-024-03262-4

  Sjgrens disease is a heterogeneous autoimmune disorder that may be associated with systemic manifestations such as pulmonary or articular involvement. […] The estimated incidence and prevalence of Sjgrens disease vary depending on the classification criteria used. In a systematic review of published literature, the incidence of Sjgrens disease was estimated to be 6.9 per 100,000 person-years and the prevalence to be 60.8 cases per 100,000. […] This disease has a strong female predominance, with a reported female-to-male ratio ranging from 9:1 to 28:1. […] The prevalence of secondary Sjgrens disease (Sjgrens disease with overlap) varies depending on the associated autoimmune disease. Among 300 patients at a tertiary care center, the prevalence of Sjgrens disease with overlap was estimated to be 15% in patients with systemic lupus erythematosus, 20% in patients with rheumatoid arthritis, and 30% in patients with systemic sclerosis.

• #16 Manifestations and management of Sjögren’s disease | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-024-03262-4

  Systemic manifestations of Sjgrens disease are common. In a multi-center study of 395 patients with Sjgrens disease, 30% had systemic manifestations at the time and 39% had experienced them in the past. […] The EULAR Sjgrens Syndrome Disease Activity Index (ESSDAI) score classifies systemic disease activity from low to high based on 12 domains (cutaneous, renal, articular, muscular, peripheral nervous system, central nervous system, hematological, glandular, constitutional, lymphadenopathic, pulmonary, biological). […] Among 921 patients in a Spanish registry, only 8% of patients with Sjgrens disease had no systemic disease activity based on ESSDAI score over a mean follow-up of 6 years. […] Patients with Sjgrens disease may also develop systemic manifestations that are not included in the ESSDAI such as cardiovascular manifestations and Raynauds phenomenon.

• #16 Manifestations and management of Sjögren’s disease | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-024-03262-4

  Analyses of the Big Data Sjgren Project Consortium registry found that hypocomplementemia and cryoglobulinemia correlated with high systemic activity based on ESSDAI score and had a greater influence on phenotype than the presence of Ro/La autoantibodies and ANA. […] Sjgrens disease often follows a largely benign course, but patients prognosis can be markedly worsened by systemic manifestations. […] A systematic review that analyzed data from 14 studies found a 1.5-fold increase in mortality in patients with Sjgrens disease compared with the general population. […] Among 1580 patients in a Spanish registry, 13% were classified as presenting with a life-threatening systemic disease, defined as high systemic ESSDAI activity in at least one organ domain. […] The most common of these presentations were lymphoma, neurological involvement, and pulmonary involvement.

• #16 Manifestations and management of Sjögren’s disease | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-024-03262-4

  Mortality over 10 years was 33% in patients with high activity in more than one organ domain compared to 20% in the overall cohort. […] Pulmonary involvement also impacts survival. A US population-based cohort found that the development of ILD in patients with Sjgrens disease was associated with poorer survival, with a hazard ratio of 2.16 over 9 years of follow-up.

• #17 Diagnosis and classification of Sjögren’s disease – UpToDate

  https://www.uptodate.com/contents/diagnosis-and-classification-of-sjogrens-disease

  Sjögren’s disease (SjD) is a chronic autoimmune inflammatory disorder characterized by impaired lacrimal and salivary gland function with resultant dryness of the eyes and mouth. […] SjD is most prevalent in female adults in their 50s and 60s but can affect adolescents and young adults, as well as males. […] This topic will review the epidemiology, diagnosis, differential diagnosis, and classification of SjD.

• #18 Sjögren’s Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/sjogrens-syndrome-pro

  The global prevalence calculated for primary Sjgrens syndrome is 61 per 100,000 inhabitants, with the highest prevalence in Europe. […] Women develop Sjgrens syndrome significantly more frequently than men. The sex difference ranges between 9:1 to 19:1. […] The mean age at time of first diagnosis of primary Sjgrens syndrome is 56 years, with another peak occurring between 20 and 40 years. However, symptoms may occur years before diagnosis.

• #19 Epidemiology of Sjögren’s: A Systematic Literature Review | springermedizin.de

  https://www.springermedizin.de/epidemiology-of-sjoegren-s-a-systematic-literature-review/26290796

  The SLR found that incidence and prevalence of primary Sjgrens was highest in females and in older age groups (65 years), while the average age at onset and diagnosis of primary Sjgrens ranged between 3457 years and 4067 years, respectively. […] The current SLR identified a prevalence range of 12.413.1 per 100,000 person-years or 22.0770.0 per 100,000 persons (once metrics were scaled to 100,000 persons). […] The difference between average ages of onset (3457 years) and diagnosis (4067 years) identified by this SLR reflects the known diagnostic delays in Sjgrens. […] Overall, the findings of this SLR provide key insights into the demographics and epidemiology of primary Sjgrens.

• #20 Sjögren’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease

  Despite the lower risk for men, primary SS in men tends to represent a more severe form of the disease. […] The role of race and ethnicity in the prevalence of the disease is unknown. […] Although Sjgren’s disease occurs in all age groups, the average age of onset is between ages 40 and 60, although as many as half of all cases may be left undiagnosed or unreported. […] The prevalence of SS generally increases with age. […] Sjgren’s disease is reported in 30-50% of people with rheumatoid arthritis and in 10-25% with systemic lupus erythematosus.

• #21 Sjögren Syndrome in Systemic Lupus Erythematosus: A Subset Characterized by a Systemic Inflammatory State | The Journal of Rheumatology

  https://www.jrheum.org/content/47/6/865

  SLE-sSS, as defined by AECC, occurred in 23% of the patients with SLE. […] The frequency of SLE-sSS increased with age and affected roughly one-quarter of all patients with SLE. […] It has become increasingly clear that different subsets of the SLE population exist. […] The diagnosis of SS is a clinical entity, based on dryness of eyes and mouth due to destructive inflammation in the exocrine glands, especially tear and salivary glands. […] The clinical SLE-sSS phenotype has been described as a mild version of SLE with dominance of skin and joint manifestations and with less severe internal organ involvement, and in particular, less nephritis. […] In our present study, subjective and objective symptoms of sSS, defined according to AECC, from a large and well-characterized cohort of consecutive SLE patients and matched controls are presented.

• #21 Sjögren Syndrome in Systemic Lupus Erythematosus: A Subset Characterized by a Systemic Inflammatory State | The Journal of Rheumatology

  https://www.jrheum.org/content/47/6/865

  The occurrence of SLE-sSS in our study (23%) is higher than that reported by most previous studies (6-14%). […] The frequencies of SLE-sSS increased with age. […] Our investigations of the SLE-sSS subset demonstrate that it affects roughly one-quarter of patients with SLE, and the frequency increases with age.

• #22 Characterizing clinical findings of Sjögren’s Disease patients in community practices using matched electronic dental-health record data | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0289335

  Established classifications exist to confirm Sjgrens Disease (SD) (previously referred as Sjgrens Syndrome) and recruit patients for research. […] Late awareness or delayed diagnosis occur due to the presentation of nonspecific symptoms such as fatigue, muscle pain, slow progression of the disease and lack of consensus in the diagnostic criteria to be applied in clinical settings. […] Studies during the past decade have utilized International Classification of Disease (ICD) codes for SD from the electronic health record (EHR) and administrative claims data to determine prevalence in the US (United States), Canada and Taiwan. […] In the first US population-based study using EHR data, the authors assessed the prevalence of physician-diagnosed primary SS in Olmsted County, Minnesota by using the diagnostic codes for SS (referred as Sjogrens disease (SD) in this paper), sicca syndrome, and keratoconjunctivitis sicca (KCS) and then ascertaining the presence of symptoms and diagnostic test results.

• #23 Disease phenotype and diagnostic delay in Saudi patients with primary Sjögren’s syndrome | Saudi Medical Journal

  https://smj.org.sa/content/42/4/405

  Objectives: To describe primary Sjgrens syndrome (pSS) cohort in Saudi Arabiain view in of clinical/serological/histopathological phentotype, and, diagnostic delay. […] The mean diagnostic delay was 2.22.4 (range 1-11) years. […] The diagnostic delay is variable in our cohort. […] Despite being one of the most important challenges, diagnostic delay has not been well explored in pSS. […] The diagnostic delay in our cohort was variable, reaching 11 years in one patient; however, the mean delay was shorter than what has been reported, barely exceeding 2 years. […] Measures that can facilitate early diagnosis should be a priority and can be implemented by specialties that receive patients with a suspected diagnosis of pSS, including family medicine, ophthalmologists, dentists, neurologists, and pulmonologists.

• #24 Prevalence of Physician-Diagnosed versus Clinically Confirmed Primary Sjögren’s Syndrome (SS) Among Adults in the United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/prevalence-of-physician-diagnosed-versus-clinically-confirmed-primary-sjogrens-syndrome-ss-among-adults-in-the-united-states/

  Prevalence of Physician-Diagnosed versus Clinically Confirmed Primary Sjgrens Syndrome (SS) Among Adults in the United States […] Keywords: Epidemiology, Sjgren’s syndrome […] This study compared the projected prevalence of primary SS in the United States (US) using two case definitions: physician-diagnosed (PD) and the subset of clinically confirmed (CC) cases. […] The literature review yielded four studies that provided relevant population-based rates (Hall 2015, Maciel et al. 2017, Izmirly et al. 2019, Felix Gomez et al. 2023). […] When age-specific prevalence rates were applied to the 2024 population, current prevalence of PD cases was 10.55 per 10,000; however, prevalence of CC cases was only 1.98 per 10,000. […] This study estimated the 2024 prevalence (per 10,000) of PD cases of primary SS at 10.55, but prevalence was only 1.98 for clinically confirmed cases even though patients were diagnosed by rheumatologists in almost all cases. […] Prevalence is projected to increase over the next decade by 7%.

• #25 Clinical and laboratory evaluation of sicca complaints: distinctive aspects of primary, secondary and non-Sjogren syndrome | Advances in Rheumatology | Full Text

  https://advancesinrheumatology.biomedcentral.com/articles/10.1186/s42358-022-00255-2

  Sjgren Syndrome (SS) is a systemic autoimmune disease with a wide spectrum of manifestations that can lead to misdiagnosis. […] The diagnosis of SS remains a challenge behind dryness. Up to 31% of the suspected cases had other conditions associated to the symptoms. […] The use of different criteria, the lack of a multidisciplinary team, and the limited access to laboratory tests can reduce the validity of SS diagnosis and explain the variable prevalence of the disease worldwide. […] A previous Brazilian study revealed a prevalence of 0.17%, comparable to other series of pSS around the world. […] The 2002 American European Consensus Group (AECG) criteria, the 2016 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) classification criteria have been useful in clinical practice and research, allowing comparisons between studies.

• #26 Prevalence of Extraglandular Manifestations in Patients With Primary Sjögren’s Syndrome in Southern Norway: A Comparison to the Literature | Volume 30 – Issue 3 – September 2015 | Archives of Rheumatology

  https://tjr.org.tr/full-text/720

  Mean prevalence rate of EGM was 42.1% with a range from 0 to 92.5% compared to the range of 0.8 to 94% in the PubMed reports on EGM. […] Epidemiological studies involving large groups of patients tend to yield lower prevalence of EGM than studies based on a smaller sample size. […] The prevalence of pSS in this study was within the range of that reported in the literature. […] Due to methodological issues, we were unable to compare reports of prevalence of EGM in pSS. […] Our findings suggest that studies including larger sample sizes may be subjected to underreporting. […] A prevalence ranging from 0.04 to 0.66% has been reported for primary Sjgrens syndrome (pSS) depending on the investigated populations and the classification criteria used. […] The use of different classification criteria and lack of control groups in some studies should be taken into consideration regarding the data from the studies included in this article. […] Also, it should be kept in mind that epidemiological studies involving large groups of patients tend to yield lower prevalence of EGM than studies based on a smaller sample size.

• #27 Epidemiology of neurological manifestations in Sjögren’s syndrome: data from the French ASSESS Cohort | RMD Open

  https://rmdopen.bmj.com/content/2/1/e000179

  Neurological manifestations seem common in primary Sjgren’s syndrome (pSS) but their reported prevalences vary. […] We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort of patients with pSS, the Assessment of Systemic Signs and Evolution in Sjgren’s syndrome (ASSESS) cohort. […] Neurological manifestations were present in 74/392 (18.9%) patients, including 63 (16%) with PNS manifestations and 14 (3.6%) with CNS manifestations. […] Prevalences of peripheral and central neurological manifestations in pSS are about 15% and 5%, respectively. […] Neurological manifestations are associated with greater pSS activity. […] New neurological manifestations are more common in patients with prior neurological involvement.

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