Materiały źródłowe

• #1

  https://link.springer.com/article/10.1007/s00393-022-01294-2

  Adult-onset Stills disease (AOSD) is a rare polygenetic disease with an annual incidence of 0.160.4/100,000. Onset is typically sudden, peaking at 36 years of age with a relatively wide margin including all age groups. Mortality and morbidity are clearly increased. The German Society of Rheumatology (DGRh) therefore commissioned the development of guidelines to inform clinical decision-making regarding diagnosis and pharmacological treatment in AOSD for rheumatologists and specialists in internal medicine. Due to the paucity of international guidelines, the DGRh hereby additionally provides a concise English version in order to render these recommendations more easily accessible, thereby entertaining the hope of contributing to improved AOSD patient care. […] The clinical picture of AOSD has been analyzed in multiple cohort studies, with varying clinical signs and symptoms. In summary, a characteristic clinical pattern emerges, including a combination of very common (50%), common (20%), and less common characteristic findings. In most studies, the exclusion of hemato-oncological diagnoses, alternative rheumatological diseases, and infections is highlighted. In order to diagnose AOSD, the board strongly recommends consideration of this combination of clinical signs and symptoms after exclusion of the conditions mentioned above.

• #2

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

• #3 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #4 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #5 The Great Still-Usion: Unmasking Adult-Onset Still’s Disease Masquerading As Upper Respiratory Tract Infection

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10079803/

  Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder that occurs with an incidence of 0.16-0.4 per 100,000 adults. […] Due to overlapping clinical manifestations and lack of a specific diagnostic test, AOSD is commonly mistaken initially for acute viral syndromes, malignancy, or other autoimmune disorders leading to inappropriate therapy. […] This case emphasizes the importance of considering AOSD in the differential diagnosis of fever of unknown origin and persistent pharyngitis. In such cases, the presence of markedly elevated serum ferritin and the exclusion of other autoimmune, infectious, and malignant etiologies should prompt physicians to consider this diagnosis.

• #6 Adult-onset Still’s disease: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/adult-onset-stills-disease-clinical-manifestations-and-diagnosis

  AOSD is very uncommon. A retrospective observational study in Japan found an incidence of 0.22 and 0.34 cases per 100,000 and a prevalence of 0.73 and 1.47 cases per 100,000 for males and females, respectively. A subsequent Japanese study cited a prevalence of 3.9 cases per 100,000. It is unclear if prevalence is increasing or there has been increased ascertainment or reporting over time. There is a bimodal age distribution, with one peak between the ages of 15 and 25 and the second between the ages of 36 and 46. However, patients older than age 70 have been reported.

• #7 AB1142 Epidemiology and Outcome of Adult-Onset still’s Disease in Northwestern Thrace Region in Turkey | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/74/Suppl_2/1284.1

  Adult-onset Still’s disease (AOSD) is an inflammatory disease characterized by fever, arthritis and typical, salmon-colored rash which is more pronounced and is accompanied by increased fever at nights. Currently, there is limited data on the prevalence of AOSD. […] Studies based on questionnaire data have reported an annual AOSD incidence of 0.22/100,000 in Japan and 0.16/100,000 in France. A study using hospital-based database has found an annual AOSD incidence of 0.4/100,000 in Northern Norway. […] In our study, we aimed to determine the epidemiology, general clinical features and outcomes of AOSD on the basis of hospital-based data in Thrace, the northwestern region of Turkey. […] Over the course of the study, the annual incidence of AOSD was 0.62/100,000 and the overall prevalence was 6.77/100,000. […] In conclusion, in our hospital-based study on AOSD as a condition with very limited epidemiological data, AOSD frequency was significantly higher compared to other series.

• #8

  https://www.clinexprheumatol.org/abstract.asp?a=9001

  Adult-onset Stills disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. Currently, there is limited data on the prevalence of AOSD. Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. […] Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.

• #9

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

• #10 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation of adult-onset Still disease (AOSD). The estimated prevalence of AOSD is more than 1/100,000 population. There is a slightly higher number of affected women than affected men. […] AOSD primarily affects young adults, although older patients have been reported. […] The non-specific clinical features of AOSD make diagnosis difficult. No serological marker is currently available. AOSD is a diagnosis of exclusion. […] The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. […] The overall prognosis is generally good, but acute life-threatening manifestations may occur in rare cases.

• #11 Estimated Prevalence and Incidence of Adult Still’s Disease: Findings by a Nationwide Epidemiological Survey in Japan

  https://www.jstage.jst.go.jp/article/jea1991/7/4/7_4_221/_article

  To estimate prevalence and incidence of adult Still’s disease in Japan, and to describe the epidemiological features of the patients, a nationwide epidemiological survey was conducted in 1994. […] Following major epidemiological findings emerged from the study: (a) The total annual number of patients treated for adult Still’s disease was estimated as 1, 100 in 1993 in Japan. The estimated crude prevalence among those aged 16 years or older were calculated as 0.73 and 1.47 per 100, 000 population for males and females, respectively, with the corresponding crude incidence rate of 0.22 and 0.34. […] (b) The sex ratio (female to male) of the reported patients was 2.1. This female predominance might be specific to Japan, though additional surveys in other countries will be warranted. The mean age of the patients was 38.1 years, and female patients tended to be older than male ones; 50% of the female patients aged 40 years or older, while so did only 28% of the male patients.

• #12 Adult-onset Still’s disease: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/adult-onset-stills-disease-clinical-manifestations-and-diagnosis

  AOSD is very uncommon. A retrospective observational study in Japan found an incidence of 0.22 and 0.34 cases per 100,000 and a prevalence of 0.73 and 1.47 cases per 100,000 for males and females, respectively. A subsequent Japanese study cited a prevalence of 3.9 cases per 100,000. It is unclear if prevalence is increasing or there has been increased ascertainment or reporting over time. There is a bimodal age distribution, with one peak between the ages of 15 and 25 and the second between the ages of 36 and 46. However, patients older than age 70 have been reported.

• #13

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

• #14

  https://www.clinexprheumatol.org/abstract.asp?a=9001

  Adult-onset Stills disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. Currently, there is limited data on the prevalence of AOSD. Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. […] Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.

• #15 Adult-onset Still’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Adult-onset_Still%27s_disease

  Adult-onset Still’s disease is rare and has been described all over the world. The number of new cases per year is estimated to be 1.6 per 1,000,000 population. The number of people currently affected is estimated at 1.5 cases per 100,0001,000,000 population. Onset is most common in two age ranges, between ages 16-25 and between ages of 36-46 years.

• #16 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #17 Adult-onset Still’s disease: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/adult-onset-stills-disease-clinical-manifestations-and-diagnosis

  AOSD is very uncommon. A retrospective observational study in Japan found an incidence of 0.22 and 0.34 cases per 100,000 and a prevalence of 0.73 and 1.47 cases per 100,000 for males and females, respectively. A subsequent Japanese study cited a prevalence of 3.9 cases per 100,000. It is unclear if prevalence is increasing or there has been increased ascertainment or reporting over time. There is a bimodal age distribution, with one peak between the ages of 15 and 25 and the second between the ages of 36 and 46. However, patients older than age 70 have been reported.

• #18 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #19 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #20 Adult-onset Still’s disease: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/adult-onset-stills-disease-clinical-manifestations-and-diagnosis

  AOSD is very uncommon. A retrospective observational study in Japan found an incidence of 0.22 and 0.34 cases per 100,000 and a prevalence of 0.73 and 1.47 cases per 100,000 for males and females, respectively. A subsequent Japanese study cited a prevalence of 3.9 cases per 100,000. It is unclear if prevalence is increasing or there has been increased ascertainment or reporting over time. There is a bimodal age distribution, with one peak between the ages of 15 and 25 and the second between the ages of 36 and 46. However, patients older than age 70 have been reported.

• #21 Clinical characteristics and treatment of elderly onset adult-onset Still’s disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-10932-3

  A recent survey in 2010-2011 showed an elevated mean age of onset (46 years), although the proportion of patients with elderly onset AOSD was unknown. […] Therefore, AOSD is no longer a rare condition in old age. […] In conclusion, the proportion of elderly patients with AOSD gradually increases. The disease activity of patients with elderly onset AOSD was similar to that of patients with younger-onset AOSD, and the frequency of complications, such as DIC and MAS, was high. CMV infections were common in patients with elderly onset AOSD but had been decreasing in recent years with a shift away from GC-dependent treatment.

• #22 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #23 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation of adult-onset Still disease (AOSD). The estimated prevalence of AOSD is more than 1/100,000 population. There is a slightly higher number of affected women than affected men. […] AOSD primarily affects young adults, although older patients have been reported. […] The non-specific clinical features of AOSD make diagnosis difficult. No serological marker is currently available. AOSD is a diagnosis of exclusion. […] The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. […] The overall prognosis is generally good, but acute life-threatening manifestations may occur in rare cases.

• #24 Estimated Prevalence and Incidence of Adult Still’s Disease: Findings by a Nationwide Epidemiological Survey in Japan

  https://www.jstage.jst.go.jp/article/jea1991/7/4/7_4_221/_article

  To estimate prevalence and incidence of adult Still’s disease in Japan, and to describe the epidemiological features of the patients, a nationwide epidemiological survey was conducted in 1994. […] Following major epidemiological findings emerged from the study: (a) The total annual number of patients treated for adult Still’s disease was estimated as 1, 100 in 1993 in Japan. The estimated crude prevalence among those aged 16 years or older were calculated as 0.73 and 1.47 per 100, 000 population for males and females, respectively, with the corresponding crude incidence rate of 0.22 and 0.34. […] (b) The sex ratio (female to male) of the reported patients was 2.1. This female predominance might be specific to Japan, though additional surveys in other countries will be warranted. The mean age of the patients was 38.1 years, and female patients tended to be older than male ones; 50% of the female patients aged 40 years or older, while so did only 28% of the male patients.

• #25 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #26

  https://www.clinexprheumatol.org/abstract.asp?a=9001

  Adult-onset Stills disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. Currently, there is limited data on the prevalence of AOSD. Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. […] Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.

• #27 Adult Still’s Disease

  https://www.arthritis.org/diseases/adult-stills-disease

  Adult-onset Stills disease is a rare type of arthritis that is thought to be autoummune or autoinflammatory. […] The disease affects men and women equally, usually young adults between the ages of 16 and 35. […] Diagnosis is based on review of symptoms, medical history, physical examination and possibly laboratory tests. […] There is no single test that can diagnose adult Stills disease. […] Instead, blood tests are used to rule out other diseases with similar symptoms. […] Other tests, such as X-rays, may be done to check for joint inflammation or damage.

• #28 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Stills Disease is a rare inflammatory disease that exists in two main forms: adult-onset Stills disease (AOSD), and a type of childhood arthritis called systemic juvenile idiopathic arthritis (SJIA). […] As with many rare diseases, its not possible to know for sure how many people have SJIA or AOSD, because of misdiagnosis or late diagnosis. […] Some recent studies suggest that: AOSD affects 0.160.4 per 100,000 adults. […] SJIA affects 3.5 per 100,000 children. […] Approximately the same number of males and females are affected, although some studies suggest that slightly more females are affected than males. […] There is low awareness of Stills disease. […] Low awareness may lead to diagnosis of other conditions first and therefore a treatment not targeting the actual disease. […] Delays in diagnosis are common. Stills disease is often confused with infection.

• #29 Adult-onset Still’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Adult-onset_Still%27s_disease

  Adult-onset Still’s disease is rare and has been described all over the world. The number of new cases per year is estimated to be 1.6 per 1,000,000 population. The number of people currently affected is estimated at 1.5 cases per 100,0001,000,000 population. Onset is most common in two age ranges, between ages 16-25 and between ages of 36-46 years.

• #30 Frontiers | Bibliometrics and Visual Analysis of Adult-onset Still Disease (1976–2020)

  https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2022.884780/full

  From 1976 to 2020, scholars from 86 countries and regions have published AoSD research. Among them, scientists from the United States published the most (397, 18.054%). The Top 10 countries with more than 200 publications included Italy (304, 13.824%), France (212, 9.641%), Japan (210, 9.55%), and Germany (201, 9.141%). […] The research on AoSD focuses on the diagnosis and differential diagnosis of the disease. Targeted therapy will become a research hotspot in the future, and relevant clinical research needs to appropriately expand the sample size and improve the credibility of the conclusions. […] This study analyzed AoSD research published from 1976 to 2020. Furthermore, we summarized the regional distribution, journal category, article citation, main cooperative groups, and keyword clustering related to AoSD research. As an understudied, orphan disease, the mechanisms and pathogenesis of AoSD still need to be elucidated. The development of targeted AoSD therapy is a relatively effective and safe. However, existing clinical research samples sizes are small and prone to Type I or Type II errors. It is necessary to further expand the clinical sample size and clarify the safety and effectiveness of biological agents. This study provides key insights into current AoSD research and can be used as a useful resource for researchers in the field to identify hotspots and trends.

• #31 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #32 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #33 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  The incidence of AOSD has been reported to be 0.16 per 100,000 persons in France, 0.22 per 100,000 persons in Japan, and 0.4 per 100,000 persons in northern Norway. […] However, Asian patients are reported to have a significantly higher in-hospital mortality rate. […] The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. […] Independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.1021.89), hepatomegaly (HR: 5.05, 95% CI: 1.4417.70), infection (HR: 15.56, 95% CI: 5.8841.20), and MAS (HR: 26.82, 95% CI: 7.5295.60). […] In our cohort, a total of 213 (43.4%) patients completed follow-up. The median follow-up time was 7 (310) years [median (25th75th percentile)], and the longest follow-up time was 16 years. During the follow-up period, half of the patients had monocyclic disease (54.0%, 115/213), while the rest had relapsing or chronic erosive patterns (46.0%, 98/213).

• #34 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #35 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Adult-onset Still disease (AOSD) is a rare condition, with incidence rates ranging from 0.16 to 0.4/100,000 patient-years. […] The condition typically affects young adults, with a median age of onset of 36 years, and predominantly women. […] The exact etiology of AOSD is still unknown. […] It is also proposed that a preceding infection may play a role in the etiology of AOSD, leading to an epigenetic effect. […] Different inflammatory mediators, including interleukin 1 (IL-1), IL-6, IL-18, and tumor necrosis factor (TNF)-alpha, likely play a role in the mechanism of the disease and may be targeted by therapeutic interventions. […] The natural history of AOSD can be quite variable. Some patients have a single systemic self-limited episode with symptoms improving within a few months and resolving by 1 year, while other patients have a polycyclic form of the disease, with multiple flares of the systemic and arthritic symptoms and clear periods of remission between the flares.

• #36 Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs | The Journal of Rheumatology

  https://www.jrheum.org/content/51/5/442

  Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology. Annual incidence and point prevalence of AOSD have been estimated as 0.16 to 0.4 per 100,000 and 2.7 to 3.9 per 100,000 people, respectively. The main symptoms are fever, joint pain, and evanescent skin rash that may be accompanied by severe systemic manifestations, including serositis. […] It has been estimated that 30% of patients have monocyclic AOSD, 30% have polycyclic AOSD, and 40% develop chronic patterns. […] Treatment for AOSD includes nonsteroidal antiinflammatory drugs (NSAIDs), corticosteroids (CS), conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), and biologic DMARDs (bDMARDs). […] There have been a number of reviews of bDMARDs for AOSD, including all interleukin-1 inhibitors (IL-1i) or specifically ANK or CNK.

• #37 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  The incidence of AOSD has been reported to be 0.16 per 100,000 persons in France, 0.22 per 100,000 persons in Japan, and 0.4 per 100,000 persons in northern Norway. […] However, Asian patients are reported to have a significantly higher in-hospital mortality rate. […] The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. […] Independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.1021.89), hepatomegaly (HR: 5.05, 95% CI: 1.4417.70), infection (HR: 15.56, 95% CI: 5.8841.20), and MAS (HR: 26.82, 95% CI: 7.5295.60). […] In our cohort, a total of 213 (43.4%) patients completed follow-up. The median follow-up time was 7 (310) years [median (25th75th percentile)], and the longest follow-up time was 16 years. During the follow-up period, half of the patients had monocyclic disease (54.0%, 115/213), while the rest had relapsing or chronic erosive patterns (46.0%, 98/213).

• #38 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Finally, the remaining patients have a form associated with chronic disease, where inflammatory arthritis is the predominant manifestation. […] Polyarthritis and arthritis affecting the large joints (shoulder, hip) at the onset of the disease are associated with an increased risk of developing chronic disease. […] The incidence of AA amyloidosis is low, especially with appropriate therapies to decrease the level of inflammation.

• #39 Treatment of adult-onset Still’s disease: a review | TCRM

  https://www.dovepress.com/treatment-of-adult-onset-stillrsquos-disease-a-review-peer-reviewed-fulltext-article-TCRM

  The epidemiology, diagnostic criteria and classification of AOSD have been reviewed recently. […] Over the last decade, one striking event was the reclassification of AOSD as a polygenic autoinflammatory disorder. […] Data from genetic and immunologic studies, together with the dramatic effect of biologic treatments, have confirmed the major role of these cytokines. […] The renewed comprehension of the disease, along with the availability of new cytokine inhibitors, has led to new therapeutic approaches. […] The general aim of this review is to discuss the optimal management of AOSD. […] It is becoming increasingly apparent that AOSD patients fall into two distinct subsets, ie, those presenting with systemic features and those presenting with prominent arthritis. […] Predictive factors for a prominent articular pattern include female sex, proximal arthritis at disease onset, thrombocytosis, and corticosteroid dependency, whereas high fever, high levels of liver enzymes, or high acute phase reactants are more likely to be associated with a systemic pattern of AOSD.

• #40 Treatment of adult-onset Still’s disease: a review | TCRM

  https://www.dovepress.com/treatment-of-adult-onset-stillrsquos-disease-a-review-peer-reviewed-fulltext-article-TCRM

  The epidemiology, diagnostic criteria and classification of AOSD have been reviewed recently. […] Over the last decade, one striking event was the reclassification of AOSD as a polygenic autoinflammatory disorder. […] Data from genetic and immunologic studies, together with the dramatic effect of biologic treatments, have confirmed the major role of these cytokines. […] The renewed comprehension of the disease, along with the availability of new cytokine inhibitors, has led to new therapeutic approaches. […] The general aim of this review is to discuss the optimal management of AOSD. […] It is becoming increasingly apparent that AOSD patients fall into two distinct subsets, ie, those presenting with systemic features and those presenting with prominent arthritis. […] Predictive factors for a prominent articular pattern include female sex, proximal arthritis at disease onset, thrombocytosis, and corticosteroid dependency, whereas high fever, high levels of liver enzymes, or high acute phase reactants are more likely to be associated with a systemic pattern of AOSD.

• #41 Treatment of adult-onset Still’s disease: a review | TCRM

  https://www.dovepress.com/treatment-of-adult-onset-stillrsquos-disease-a-review-peer-reviewed-fulltext-article-TCRM

  This dichotomy may be of the utmost importance for the management of AOSD patients, as patients falling into one of the two categories should benefit from different treatments. […] In patients with articular refractory AOSD, anti-TNF- should be the preferred treatment. […] Finally, it should be noted that, in an effort to standardize therapeutic management and evaluate comparative effectiveness in an observational setting, the Childhood Arthritis and Rheumatology Research Alliance has developed four consensus treatment plans for systemic-onset JIA. […] As no guidelines are available for AOSD, this consensus plan should be of help for physicians dealing with new-onset AOSD.

• #42 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation of adult-onset Still disease (AOSD). The estimated prevalence of AOSD is more than 1/100,000 population. There is a slightly higher number of affected women than affected men. […] AOSD primarily affects young adults, although older patients have been reported. […] The non-specific clinical features of AOSD make diagnosis difficult. No serological marker is currently available. AOSD is a diagnosis of exclusion. […] The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. […] The overall prognosis is generally good, but acute life-threatening manifestations may occur in rare cases.

• #43 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  The incidence of AOSD has been reported to be 0.16 per 100,000 persons in France, 0.22 per 100,000 persons in Japan, and 0.4 per 100,000 persons in northern Norway. […] However, Asian patients are reported to have a significantly higher in-hospital mortality rate. […] The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. […] Independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.1021.89), hepatomegaly (HR: 5.05, 95% CI: 1.4417.70), infection (HR: 15.56, 95% CI: 5.8841.20), and MAS (HR: 26.82, 95% CI: 7.5295.60). […] In our cohort, a total of 213 (43.4%) patients completed follow-up. The median follow-up time was 7 (310) years [median (25th75th percentile)], and the longest follow-up time was 16 years. During the follow-up period, half of the patients had monocyclic disease (54.0%, 115/213), while the rest had relapsing or chronic erosive patterns (46.0%, 98/213).

• #44 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  Twenty-two patients (10.33%, 22/213) ultimately died. The 5-year survival rate and 10-year survival rate after diagnosis were 92.4% and 86.9%, respectively. […] The results of multivariate analysis showed that the independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.1021.89), hepatomegaly (HR: 5.05, 95% CI: 1.4417.70), infection (HR: 15.56, 95% CI: 5.8841.20) and MAS (HR: 26.82, 95% CI: 7.5295.60).

• #45 Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-021-02688-4

  Twenty-two patients (10.33%, 22/213) ultimately died. The 5-year survival rate and 10-year survival rate after diagnosis were 92.4% and 86.9%, respectively. […] The results of multivariate analysis showed that the independent risk factors for mortality were age at onset 50 (hazard ratio (HR): 6.78, 95% CI: 2.1021.89), hepatomegaly (HR: 5.05, 95% CI: 1.4417.70), infection (HR: 15.56, 95% CI: 5.8841.20) and MAS (HR: 26.82, 95% CI: 7.5295.60).

• #46 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #47 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Conclusion: In hospitalized US AOSD patients, the average age was higher than previously described in cross sectional studies. This may indicate an aging population with a higher number of comorbidities that prompt hospitalization. Mortality increased with age and was higher among women and Asians. To our knowledge, this is the largest epidemiological study of AOSD today in the USA.

• #48 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #49

  https://link.springer.com/article/10.1007/s00393-022-01294-2

  The Yamaguchi classification criteria have shown good diagnostic precision compared to other criteria and diagnostic approaches in cohort studies. Therefore, these criteria can be employed to ascertain the clinical diagnosis of AOSD made by an expert. […] Various risk factors for MAS (synonym acquired hemophagocytic lymphohistiocytosis [HLH] or MAS-HLH) complicating AOSD have been identified in different cohort studies. Evaluation for MAS is advised in case of risk factors. […] Perimyocardial and pulmonary complications in AOSD are of concern and are associated with an unfavorable prognosis and/or treatment resistance. […] In a systematic literature review covering 19712018, the prevalence of AA amyloidosis in AOSD was estimated to be relatively low, at 0.88% (95% confidence interval [CI] 0.491.28). The main risk factor is high clinical disease activity. Due to the adverse prognosis associated with systemic AA amyloidosis, the board recommends excluding this rare complication in AOSD patients in case of persistently active disease.

• #50

  https://link.springer.com/article/10.1007/s00393-022-01294-2

  The Yamaguchi classification criteria have shown good diagnostic precision compared to other criteria and diagnostic approaches in cohort studies. Therefore, these criteria can be employed to ascertain the clinical diagnosis of AOSD made by an expert. […] Various risk factors for MAS (synonym acquired hemophagocytic lymphohistiocytosis [HLH] or MAS-HLH) complicating AOSD have been identified in different cohort studies. Evaluation for MAS is advised in case of risk factors. […] Perimyocardial and pulmonary complications in AOSD are of concern and are associated with an unfavorable prognosis and/or treatment resistance. […] In a systematic literature review covering 19712018, the prevalence of AA amyloidosis in AOSD was estimated to be relatively low, at 0.88% (95% confidence interval [CI] 0.491.28). The main risk factor is high clinical disease activity. Due to the adverse prognosis associated with systemic AA amyloidosis, the board recommends excluding this rare complication in AOSD patients in case of persistently active disease.

• #51 Adult-Onset Still Disease (AOSD) – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.2.

  Finally, the remaining patients have a form associated with chronic disease, where inflammatory arthritis is the predominant manifestation. […] Polyarthritis and arthritis affecting the large joints (shoulder, hip) at the onset of the disease are associated with an increased risk of developing chronic disease. […] The incidence of AA amyloidosis is low, especially with appropriate therapies to decrease the level of inflammation.

• #52 Clinical characteristics and treatment of elderly onset adult-onset Still’s disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-10932-3

  Adult-onset Stills disease (AOSD) a systemic inflammatory disease often occurs at a young age. Recently, elderly onset patient proportion has been increasing; however, data are limited. To evaluate the characteristics of elderly patients with AOSD in a multicenter cohort, we retrospectively analyzed 62 patients with AOSD at five hospitals during April 2008-December 2020. Patients were divided into two groups according to age at disease onset: younger-onset (64 years) and elderly onset (65 years). […] The elderly onset group had a lower frequency of sore throat (53.8% vs. 86.1%), higher frequency of pleuritis (46.2% vs. 16.7%), and higher complication rates of disseminated intravascular coagulation (30.8% vs. 8.3%) and macrophage activation syndrome (19.2% vs. 2.8%) than the younger onset group.

• #53 Clinical characteristics and treatment of elderly onset adult-onset Still’s disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-10932-3

  Elderly AOSD is not uncommon; these patients have different characteristics than younger-onset patients. […] The treatment of AOSD have been progressed recently, and immunosuppressants, including methotrexate or cyclosporine and targeting therapy, such as IL-1 or IL-6 inhibitors, are available in addition to glucocorticoids (GCs). […] Understanding the characteristics of patients with elderly onset AOSD is essential for appropriate management. […] The results showed that elderly patients had different characteristics compared with younger patients, including less frequent sore throat, more frequent pleuritis, a higher frequency of severe complications, such as DIC and MAS, higher levels of inflammatory markers, lower levels of hemoglobin, and higher rates of CMV infections. […] To date, some nationwide epidemiological surveys evaluating the prevalence of AOSD have been conducted in Japan using questionnaires.

• #54

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

• #55 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation of adult-onset Still disease (AOSD). The estimated prevalence of AOSD is more than 1/100,000 population. There is a slightly higher number of affected women than affected men. […] AOSD primarily affects young adults, although older patients have been reported. […] The non-specific clinical features of AOSD make diagnosis difficult. No serological marker is currently available. AOSD is a diagnosis of exclusion. […] The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. […] The overall prognosis is generally good, but acute life-threatening manifestations may occur in rare cases.

• #56 The Great Still-Usion: Unmasking Adult-Onset Still’s Disease Masquerading As Upper Respiratory Tract Infection

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10079803/

  Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder that occurs with an incidence of 0.16-0.4 per 100,000 adults. […] Due to overlapping clinical manifestations and lack of a specific diagnostic test, AOSD is commonly mistaken initially for acute viral syndromes, malignancy, or other autoimmune disorders leading to inappropriate therapy. […] This case emphasizes the importance of considering AOSD in the differential diagnosis of fever of unknown origin and persistent pharyngitis. In such cases, the presence of markedly elevated serum ferritin and the exclusion of other autoimmune, infectious, and malignant etiologies should prompt physicians to consider this diagnosis.

• #57

  https://link.springer.com/article/10.1007/s00393-022-01294-2

  Adult-onset Stills disease (AOSD) is a rare polygenetic disease with an annual incidence of 0.160.4/100,000. Onset is typically sudden, peaking at 36 years of age with a relatively wide margin including all age groups. Mortality and morbidity are clearly increased. The German Society of Rheumatology (DGRh) therefore commissioned the development of guidelines to inform clinical decision-making regarding diagnosis and pharmacological treatment in AOSD for rheumatologists and specialists in internal medicine. Due to the paucity of international guidelines, the DGRh hereby additionally provides a concise English version in order to render these recommendations more easily accessible, thereby entertaining the hope of contributing to improved AOSD patient care. […] The clinical picture of AOSD has been analyzed in multiple cohort studies, with varying clinical signs and symptoms. In summary, a characteristic clinical pattern emerges, including a combination of very common (50%), common (20%), and less common characteristic findings. In most studies, the exclusion of hemato-oncological diagnoses, alternative rheumatological diseases, and infections is highlighted. In order to diagnose AOSD, the board strongly recommends consideration of this combination of clinical signs and symptoms after exclusion of the conditions mentioned above.

• #58 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #59 The Great Still-Usion: Unmasking Adult-Onset Still’s Disease Masquerading As Upper Respiratory Tract Infection

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10079803/

  Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder that occurs with an incidence of 0.16-0.4 per 100,000 adults. […] Due to overlapping clinical manifestations and lack of a specific diagnostic test, AOSD is commonly mistaken initially for acute viral syndromes, malignancy, or other autoimmune disorders leading to inappropriate therapy. […] This case emphasizes the importance of considering AOSD in the differential diagnosis of fever of unknown origin and persistent pharyngitis. In such cases, the presence of markedly elevated serum ferritin and the exclusion of other autoimmune, infectious, and malignant etiologies should prompt physicians to consider this diagnosis.

• #60 Adult-onset Still’s disease: current challenges and future prospec | OARRR

  https://www.dovepress.com/adult-onset-still39s-disease-current-challenges-and-future-prospects-peer-reviewed-fulltext-article-OARRR

  Adult-onset Stills disease (AOSD) is a rare multisystemic inflammatory condition characterized by high fevers, polyarthritis or arthralgias, an evanescent rash, and pharyngitis. […] A recent retrospective series of 57 patients found a mean time to diagnosis of 4 months. […] AOSD and sJIA occur rarely, with incidences estimated at 0.16 cases per 100,000 people per year and 0.6 per 100,000 people per year, respectively. […] The estimated incidence of MAS in AOSD is 12%14%; not only is this higher than in other rheumatologic diseases, but in sJIA 53% of patients had bone marrow biopsies suggestive of MAS. […] The treatment of AOSD has been largely derived from case reports and retrospective data. […] As with many rare medical conditions, progress on AOSD has been hampered by the difficulty of enrolling a sufficient number of patients for large randomized trials.

• #61 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Stills Disease is a rare inflammatory disease that exists in two main forms: adult-onset Stills disease (AOSD), and a type of childhood arthritis called systemic juvenile idiopathic arthritis (SJIA). […] As with many rare diseases, its not possible to know for sure how many people have SJIA or AOSD, because of misdiagnosis or late diagnosis. […] Some recent studies suggest that: AOSD affects 0.160.4 per 100,000 adults. […] SJIA affects 3.5 per 100,000 children. […] Approximately the same number of males and females are affected, although some studies suggest that slightly more females are affected than males. […] There is low awareness of Stills disease. […] Low awareness may lead to diagnosis of other conditions first and therefore a treatment not targeting the actual disease. […] Delays in diagnosis are common. Stills disease is often confused with infection.

• #62

  https://link.springer.com/article/10.1007/s00393-022-01294-2

  The Yamaguchi classification criteria have shown good diagnostic precision compared to other criteria and diagnostic approaches in cohort studies. Therefore, these criteria can be employed to ascertain the clinical diagnosis of AOSD made by an expert. […] Various risk factors for MAS (synonym acquired hemophagocytic lymphohistiocytosis [HLH] or MAS-HLH) complicating AOSD have been identified in different cohort studies. Evaluation for MAS is advised in case of risk factors. […] Perimyocardial and pulmonary complications in AOSD are of concern and are associated with an unfavorable prognosis and/or treatment resistance. […] In a systematic literature review covering 19712018, the prevalence of AA amyloidosis in AOSD was estimated to be relatively low, at 0.88% (95% confidence interval [CI] 0.491.28). The main risk factor is high clinical disease activity. Due to the adverse prognosis associated with systemic AA amyloidosis, the board recommends excluding this rare complication in AOSD patients in case of persistently active disease.

• #63

  https://link.springer.com/article/10.1007/s00393-022-01294-2

  Adult-onset Stills disease (AOSD) is a rare polygenetic disease with an annual incidence of 0.160.4/100,000. Onset is typically sudden, peaking at 36 years of age with a relatively wide margin including all age groups. Mortality and morbidity are clearly increased. The German Society of Rheumatology (DGRh) therefore commissioned the development of guidelines to inform clinical decision-making regarding diagnosis and pharmacological treatment in AOSD for rheumatologists and specialists in internal medicine. Due to the paucity of international guidelines, the DGRh hereby additionally provides a concise English version in order to render these recommendations more easily accessible, thereby entertaining the hope of contributing to improved AOSD patient care. […] The clinical picture of AOSD has been analyzed in multiple cohort studies, with varying clinical signs and symptoms. In summary, a characteristic clinical pattern emerges, including a combination of very common (50%), common (20%), and less common characteristic findings. In most studies, the exclusion of hemato-oncological diagnoses, alternative rheumatological diseases, and infections is highlighted. In order to diagnose AOSD, the board strongly recommends consideration of this combination of clinical signs and symptoms after exclusion of the conditions mentioned above.

• #64 Adult Still’s disease Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/adult-stills-disease

  Adult Still disease (ASD) is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis. […] Fewer than 1 out of 100,000 people develop ASD each year. It affects women more often than men. […] The cause of adult Still disease is unknown. No risk factors for the disease have been identified. […] AOSD can only be diagnosed after many other diseases (such as infections and cancer) are ruled out. You may need many medical tests before a final diagnosis is made. […] There is no known prevention.

• #65 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #66 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #67 Adult-onset Still’s disease masquerading as acute coronary syndrome: a case report and review of the literature | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-024-04799-3

  Adult-onset Stills disease is a rare systemic autoinflammatory disease. […] Adult-onset Stills disease should be considered in a patient with fevers of undetermined origin. […] Due to its multisystemic involvement, adult-onset Stills disease is often a diagnosis arrived at after an extensive cardiac, hematologic, malignant, and infectious workup. […] The hospital course for AOSD remains challenging due to a lack of specific diagnostic testing and limited management guidelines. […] The mainstay of treatment for AOSD is to suppress the immune system with either steroids and/or steroid-sparing biologics in the acute setting, and to ensure that the patient has regular follow-up to monitor disease activity, recurrence, and response to medications. […] Although considered rare, adult-onset Stills disease is not an uncommon cause of fever of undetermined origin. Early identification and initiation of steroid-sparing treatment are critical to faster recovery and prevention of side effects and progression to severe complications.

• #68 Adult-onset Still’s disease: current challenges and future prospec | OARRR

  https://www.dovepress.com/adult-onset-still39s-disease-current-challenges-and-future-prospects-peer-reviewed-fulltext-article-OARRR

  Adult-onset Stills disease (AOSD) is a rare multisystemic inflammatory condition characterized by high fevers, polyarthritis or arthralgias, an evanescent rash, and pharyngitis. […] A recent retrospective series of 57 patients found a mean time to diagnosis of 4 months. […] AOSD and sJIA occur rarely, with incidences estimated at 0.16 cases per 100,000 people per year and 0.6 per 100,000 people per year, respectively. […] The estimated incidence of MAS in AOSD is 12%14%; not only is this higher than in other rheumatologic diseases, but in sJIA 53% of patients had bone marrow biopsies suggestive of MAS. […] The treatment of AOSD has been largely derived from case reports and retrospective data. […] As with many rare medical conditions, progress on AOSD has been hampered by the difficulty of enrolling a sufficient number of patients for large randomized trials.

• #69 Clinical characteristics and treatment of elderly onset adult-onset Still’s disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-10932-3

  A recent survey in 2010-2011 showed an elevated mean age of onset (46 years), although the proportion of patients with elderly onset AOSD was unknown. […] Therefore, AOSD is no longer a rare condition in old age. […] In conclusion, the proportion of elderly patients with AOSD gradually increases. The disease activity of patients with elderly onset AOSD was similar to that of patients with younger-onset AOSD, and the frequency of complications, such as DIC and MAS, was high. CMV infections were common in patients with elderly onset AOSD but had been decreasing in recent years with a shift away from GC-dependent treatment.

• #70 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Abstract Number: 1885 […] Epidemiology of Hospitalized Adult Onset Stills Disease in United States […] Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Stills Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report a majority of patients below the age of 35. Our objective is to describe the demographics, complications and mortality of hospitalized patients with AOSD in USA. […] Results: Between 2009 and 2013, 5,820 AOSD patients were hospitalized. AOSD patients had a mean age of 53.6 (SE 0.6) years and 3817 (70.4%) were females. The racial/ethnic distribution showed that 56% white, 15% African American, 11.7% Hispanic and 3% Asian patients were affected. 37.6% of patients were hospitalized in urban teaching hospitals. The Mid-Atlantic census division had the highest number of patients. 100 (1.7%) developed Macrophage Activating Syndrome (MAS), 66 (1.1%) patients had disseminated intravascular coagulation (DIC) and 25(0.4%) had thrombotic thrombocytopenic purpura (TTP). The mean length of stay was 6.9 (SE- 0.3) days. There were 154 inpatient deaths in 5 years (mortality 2.6%). The patients who died during hospitalization were more likely to be older, mean age of 62.4 (SE- 3.1) years, women (69.2%) and/or Asian (13.9%).

• #71 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Conclusion: In hospitalized US AOSD patients, the average age was higher than previously described in cross sectional studies. This may indicate an aging population with a higher number of comorbidities that prompt hospitalization. Mortality increased with age and was higher among women and Asians. To our knowledge, this is the largest epidemiological study of AOSD today in the USA.

• #72 FRI0595 Epidemiology and complications of hospitalized patients with adult onset still’s disease in united states: a nationwide estimate | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/76/Suppl_2/714.1

  There is a dearth of epidemiological studies in the US and worldwide on Adult Onset Still’s Disease (AOSD). Currently, there is no consensus on its incidence and prevalence in different populations. Most studies report a majority of patients below the age of 35. […] In hospitalized American AOSD patients, the average age was higher than previously thought. This may indicate an aging population with a higher number of comorbidities that justify hospitalization. Mortality increased with age and was higher among women and Asians. To our knowledge, this is the largest epidemiological study of AOSD today in the USA.

• #73 Epidemiology of Hospitalized Adult Onset Still’s Disease in United States – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/epidemiology-of-hospitalized-adult-onset-stills-disease-in-united-states/

  Conclusion: In hospitalized US AOSD patients, the average age was higher than previously described in cross sectional studies. This may indicate an aging population with a higher number of comorbidities that prompt hospitalization. Mortality increased with age and was higher among women and Asians. To our knowledge, this is the largest epidemiological study of AOSD today in the USA.

• #74

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

• #75

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

• #76 Frontiers | Bibliometrics and Visual Analysis of Adult-onset Still Disease (1976–2020)

  https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2022.884780/full

  From 1976 to 2020, scholars from 86 countries and regions have published AoSD research. Among them, scientists from the United States published the most (397, 18.054%). The Top 10 countries with more than 200 publications included Italy (304, 13.824%), France (212, 9.641%), Japan (210, 9.55%), and Germany (201, 9.141%). […] The research on AoSD focuses on the diagnosis and differential diagnosis of the disease. Targeted therapy will become a research hotspot in the future, and relevant clinical research needs to appropriately expand the sample size and improve the credibility of the conclusions. […] This study analyzed AoSD research published from 1976 to 2020. Furthermore, we summarized the regional distribution, journal category, article citation, main cooperative groups, and keyword clustering related to AoSD research. As an understudied, orphan disease, the mechanisms and pathogenesis of AoSD still need to be elucidated. The development of targeted AoSD therapy is a relatively effective and safe. However, existing clinical research samples sizes are small and prone to Type I or Type II errors. It is necessary to further expand the clinical sample size and clarify the safety and effectiveness of biological agents. This study provides key insights into current AoSD research and can be used as a useful resource for researchers in the field to identify hotspots and trends.

• #77 Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs | The Journal of Rheumatology

  https://www.jrheum.org/content/51/5/442

  This systematic review identified 44 studies published in the last 10 years of conventional, biologic, and novel treatments for AOSD, including 3 RCTs of bDMARDs (TCZ, ANK, CNK) and non-RCTs evaluating a number of early and more recent treatments. […] Evidence from case series appears to support the case for bDMARDs for AOSD, particularly TCZ, ANK, and CNK. However, the magnitude of effect and comparative effectiveness of different treatments is still uncertain.

• #78 Adult-onset Still’s Disease – Epidemiology Forecast to 2032

  https://www.researchandmarkets.com/reports/5206755/adult-onset-stills-disease-epidemiology?srsltid=AfmBOop50rIzdZQZRZ4mix_Xr3QhRe0qk8xPCxhDKZsNGT8GCGdswJpQ

  This „Adult-onset Still’s Disease – Epidemiology Forecast to 2032” report delivers an in-depth understanding of the disease, historical and forecasted Adult-onset Still’s Disease epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan. […] The Adult-onset Still’s Disease epidemiology report gives a thorough understanding of the Adult-onset Still’s Disease by including details such as disease definition, symptoms, causes, pathophysiology, and diagnosis. […] The Adult-onset Still’s Disease epidemiology division provides insights about historical and current patient pool and forecasted trend for every seven major countries. […] The Adult-onset Still’s Disease epidemiology segment covers the epidemiology data in the US, EU5 countries (Germany, Spain, Italy, France, and the UK), and Japan from 2019 to 2032.

• #79 Adult-onset Still’s Disease – Epidemiology Forecast to 2032

  https://www.researchandmarkets.com/reports/5206755/adult-onset-stills-disease-epidemiology?srsltid=AfmBOop50rIzdZQZRZ4mix_Xr3QhRe0qk8xPCxhDKZsNGT8GCGdswJpQ

  The Adult-onset Still’s Disease epidemiology covered in the report provides historical as well as forecasted Adult-onset Still’s Disease epidemiology scenario in the 7MM covering the United States, EU5 countries (Germany, Spain, Italy, France, and the United Kingdom), and Japan from 2019 to 2032. […] The report provides insight into the historical and forecasted patient pool of Adult-onset Still’s Disease in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan. […] The report assesses the disease risk and burden and highlights the unmet needs of Adult-onset Still’s Disease. […] The calculated data are presented with relevant tables and graphs to give a clear view of the epidemiology at first sight. […] The report helps recognize the growth opportunities in the 7MM for the patient population. […] The Adult-onset Still’s Disease Epidemiology Model developed by the publisher is easy to navigate, interactive with dashboards, and epidemiology based with transparent and consistent methodologies.

• #80 Still’s disease – AOSD, SJIA | Sobi

  https://www.sobi.com/en/stills-disease

  Stills Disease is a rare inflammatory disease that exists in two main forms: adult-onset Stills disease (AOSD), and a type of childhood arthritis called systemic juvenile idiopathic arthritis (SJIA). […] As with many rare diseases, its not possible to know for sure how many people have SJIA or AOSD, because of misdiagnosis or late diagnosis. […] Some recent studies suggest that: AOSD affects 0.160.4 per 100,000 adults. […] SJIA affects 3.5 per 100,000 children. […] Approximately the same number of males and females are affected, although some studies suggest that slightly more females are affected than males. […] There is low awareness of Stills disease. […] Low awareness may lead to diagnosis of other conditions first and therefore a treatment not targeting the actual disease. […] Delays in diagnosis are common. Stills disease is often confused with infection.

• #81 Still Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538345/

  AOSD is a very uncommon condition, with an estimated annual incidence ranging from 0.1 to 0.4 cases per 100,000 people in Europe. There is a slight predilection for females over males. The age distribution follows a bimodal pattern, with the first peak occurring between 15 and 25 years and the second between 36 and 46. Notably, about three-quarters of patients experience the onset of the disease between 16 and 35. Although unusual, instances of onset beyond the age of 70 have also been documented.[6] […] AOSD is, in part, a diagnosis of exclusion and frequently presents a diagnostic challenge. While a rheumatologist typically leads the care team, collaboration with specialists such as cardiologists, gastroenterologists, and oncologists is crucial for comprehensive management. The disease can cause complications in multiple organ systems, necessitating a multidisciplinary approach.

• #82 The Great Still-Usion: Unmasking Adult-Onset Still’s Disease Masquerading As Upper Respiratory Tract Infection

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10079803/

  Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder that occurs with an incidence of 0.16-0.4 per 100,000 adults. […] Due to overlapping clinical manifestations and lack of a specific diagnostic test, AOSD is commonly mistaken initially for acute viral syndromes, malignancy, or other autoimmune disorders leading to inappropriate therapy. […] This case emphasizes the importance of considering AOSD in the differential diagnosis of fever of unknown origin and persistent pharyngitis. In such cases, the presence of markedly elevated serum ferritin and the exclusion of other autoimmune, infectious, and malignant etiologies should prompt physicians to consider this diagnosis.

• #83 Orphanet: Adult-onset Still disease

  https://www.orpha.net/en/disease/detail/829

  Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation of adult-onset Still disease (AOSD). The estimated prevalence of AOSD is more than 1/100,000 population. There is a slightly higher number of affected women than affected men. […] AOSD primarily affects young adults, although older patients have been reported. […] The non-specific clinical features of AOSD make diagnosis difficult. No serological marker is currently available. AOSD is a diagnosis of exclusion. […] The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. […] The overall prognosis is generally good, but acute life-threatening manifestations may occur in rare cases.

• #84

  https://www.aaem.pl/Adult-onset-Still-s-disease-in-Poland-a-nationwide-population-based-study,132451,0,2.html

  Adult-onset Stills disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, which affects young adults and is associated with multiple organ involvement and life-threatening complications. The aim of the study was to establish the incidence and prevalence of AOSD in Poland, and factors related to this disease among hospitalized patients. […] The average annual incidence rate of AOSD during the 10-year period was established at the level of 0.32/100,000 (95% CI: 0.300.34), and the point prevalence at the end of 2018 was 2.7/100,000. […] AOSD is a rare disease in Poland with gender and territorial differences in incidence rate, and predominance of cardiovascular diseases among comorbidities. The presented data may be useful for comparisons of the Polish population with other geographical regions. Predominance of patients from urban regions and predominance of women may suggest environmental and personal factors in AOSD development; however, further research seems to be necessary.

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