Choroba stilla u dorosłych
Etiologia i przyczyny

Choroba Stilla u dorosłych (Adult-onset Still’s Disease, AOSD) to rzadkie, układowe zapalenie o nieznanej etiologii, charakteryzujące się wysoką gorączką, przemijającą wysypką łososiowo-różową oraz zapaleniem wielostawowym. Patogeneza AOSD jest wieloczynnikowa i obejmuje predyspozycje genetyczne (związane m.in. z HLA-B17, HLA-B18, HLA-B35, HLA-DR2, HLA-DR4, HLA-DrW6), czynniki zakaźne (m.in. Yersinia enterocolitica, Mycoplasma pneumoniae, CMV, EBV, HHV-6, wirusy grypy, wirusy zapalenia wątroby, parvowirus B19) oraz immunopatogenezę z aktywacją makrofagów i neutrofilów prowadzącą do burzy cytokinowej. Kluczowe cytokiny zaangażowane w proces zapalny to IL-1, IL-6, IL-18 oraz TNF-α, a centralną rolę odgrywa aktywacja inflammasomu NLRP3 z nadprodukcją IL-18. Dodatkowo, czynniki hormonalne, środowiskowe, stres, reakcje alergiczne oraz nowotwory mogą predysponować do rozwoju choroby. Epidemiologicznie AOSD występuje z częstością <1/100 000, najczęściej u osób w wieku 16-35 lat, z dwoma szczytami zachorowań (15-25 i 36-46 lat).

Etiologia choroby Stilla u dorosłych

Choroba Stilla u dorosłych (Adult-onset Still’s Disease, AOSD) to rzadkie, układowe schorzenie zapalne, które charakteryzuje się wysoką gorączką, przemijającą wysypką łososiowo-różową, bólami stawów i zapaleniem wielostawowym o nieznanej etiologii. Mimo intensywnych badań, dokładna przyczyna choroby pozostaje niewyjaśniona, choć istnieje kilka dominujących teorii na temat jej patogenezy.123

Czynniki infekcyjne jako potencjalne wyzwalacze choroby

Dominująca hipoteza sugeruje, że AOSD jest zespołem reaktywnym, w którym różne czynniki zakaźne mogą działać jako wyzwalacze u osób z predyspozycją genetyczną. Zarówno infekcje wirusowe, jak i bakteryjne są postrzegane jako potencjalne czynniki przyczyniające się do rozwoju choroby.12

Wśród bakterii podejrzewanych o wywoływanie lub nasilanie AOSD wymieniane są:12

Wśród patogenów wirusowych powiązanych z AOSD w literaturze medycznej wymienia się:12

  • Cytomegalowirus (CMV) – badania wskazują na silny związek między zakażeniem CMV a inicjacją lub wzmocnieniem odpowiedzi zapalnej w AOSD
  • Wirus Epsteina-Barr (EBV)
  • Wirus ludzkiego herpeswirusa 6 (HHV-6)
  • Wirusy grypy
  • Wirusy zapalenia wątroby typu A, B i C
  • Parvowirus B19
  • Wirus różyczki
  • Adenowirusy
  • Echovirus 7
  • Coxsackievirus B4

Sugeruje się, że infekcja wirusowa może wyzwalać początek lub nawrót AOSD. Badanie obejmujące 100 pacjentów z AOSD wykazało obecność przeciwciał przeciwwirusowych, wirusowego obciążenia DNA oraz czujników kwasów nukleinowych (IFI16 i AIM2), co wskazuje na potencjalną rolę wirusów w patogenezie choroby.12

Chociaż nie udowodniono jeszcze jednoznacznego związku przyczynowo-skutkowego między konkretnymi patogenami a AOSD, dane epidemiologiczne sugerują, że infekcje mogą działać jako wyzwalacze u osób podatnych genetycznie.12

Podłoże genetyczne choroby Stilla u dorosłych

Czynniki genetyczne prawdopodobnie odgrywają istotną rolę w rozwoju AOSD, choć siła tych zależności pozostaje niejasna.1 Francuskie badanie obejmujące 62 pacjentów wykazało związek między AOSD a określonymi podtypami ludzkiego antygenu leukocytarnego (HLA):12

  • HLA-B17
  • HLA-B18
  • HLA-B35
  • HLA-DR2
  • HLA-DR4
  • HLA-DrW6

Warto zauważyć, że opisywano również przypadki wystąpienia choroby u bliźniąt, co dodatkowo wspiera hipotezę o komponencie genetycznym.1 Mimo to, związki przyczynowe między genetyką a AOSD pozostają kontrowersyjne. Czynniki genetyczne człowieka najwyraźniej przyczyniają się do rozwoju uogólnionej postaci młodzieńczego idiopatycznego zapalenia stawów (SJIA) u dzieci, natomiast podstawowa genetyczna podatność w postaci dorosłej pozostaje niejasna.1

Immunopatogeneza i rola cytokin w AOSD

Patogeneza AOSD obejmuje podłoże genetyczne, czynniki zakaźne i immunopatogenezę, głównie aktywację makrofagów i neutrofilów, po której następuje burza cytokinowa.1 Choroba Stilla u dorosłych jest uważana za schorzenie autoimmunologiczne lub autozapalne.12

AOSD najczęściej klasyfikuje się jako chorobę autozapalną, co oznacza, że jest to manifestacja kaskady autozapalnej spowodowanej aktywacją komórek odporności wrodzonej. Odróżnia to AOSD od chorób zapalnych mediowanych autoimmunologicznie, w których autoprzeciwciała lub specyficzne dla autoantygenów komórki T i/lub B prowadzą do zapalenia i uszkodzenia tkanek.1

Kluczowe cytokiny zaangażowane w patogenezę AOSD obejmują:123

  • Interleukina-1 (IL-1) – mediator reakcji zapalnych, którego nieprawidłowe poziomy stwierdzono u wielu pacjentów z chorobą Stilla
  • Interleukina-6 (IL-6) – silny czynnik prozapalny
  • Interleukina-18 (IL-18) – odgrywa istotną rolę w aktywacji makrofagów i neutrofilów w chorobie Stilla
  • Czynnik martwicy nowotworu alfa (TNF-α) – kluczowy mediator zapalenia

Ostateczna wspólna ścieżka odpowiedzi zapalnej w AOSD została przypisana aktywacji inflammasomu NLRP3 (NOD-, LRR- i zawierającego domenę pirynu białka 3) z nadprodukcją interleukiny 18 (IL-18).1 Patologiczne wzmocnienie odpowiedzi zapalnej (tzw. „burza cytokinowa”) w AOSD jest prawdopodobnie wieloczynnikowe, być może z powodu czynników zakaźnych i/lub genetycznych.12

Niedobór w rozwiązywaniu stanu zapalnego został zaproponowany jako czynnik odgrywający rolę w patogenezie AOSD. Ten defekt w samoregulacji układu odpornościowego może prowadzić do przewlekłego stanu zapalnego.12

Dodatkowe czynniki etiologiczne

Poza infekcjami i genetyką, w literaturze opisano również inne potencjalne czynniki przyczyniające się do rozwoju AOSD:123

  • Czynniki hormonalne – sugeruje się, że zaburzenia równowagi hormonalnej mogą odgrywać rolę w rozwoju choroby Stilla. Komórki zapalne mogą się zwiększać, gdy hormony organizmu są niezrównoważone, co prowadzi do objawów takich jak gorączka i ból stawów
  • Czynniki środowiskowe – długotrwała ekspozycja na określone toksyny środowiskowe, takie jak emitowane przez przemysł i rolnictwo, może uszkodzić płuca, wywołując odpowiedź zapalną
  • Stres – wysoki poziom stresu w codziennym życiu był również wskazywany jako czynnik predysponujący w AOSD
  • Reakcje alergiczne – alergeny, takie jak pyłki i sierść zwierząt, mogą wywołać odpowiedź zapalną prowadzącą do rozwoju choroby Stilla
  • Nowotwory – zbadano również związek między nowotworami a AOSD i opisano autostan zapalny wywołany nowotworami w raku piersi, raku tarczycy, czerniaku, raku płuc i nowotworach hematologicznych, głównie chłoniakach

Epidemiologia choroby Stilla u dorosłych

AOSD jest chorobą rzadką, z zachorowalnością mniejszą niż 1 na 100 000 osób.12 Choroba zazwyczaj rozpoczyna się między 16 a 35 rokiem życia, choć można wyróżnić dwa szczyty zachorowań: pierwszy między 15 a 25 rokiem życia, drugi między 36 a 46 rokiem życia.123

Niektóre źródła sugerują, że choroba Stilla u dorosłych częściej dotyka kobiet niż mężczyzn, choć inne wskazują na równe ryzyko niezależnie od płci.12

Wyzwania diagnostyczne w chorobie Stilla u dorosłych

Diagnoza AOSD pozostaje jednym z większych wyzwań klinicznych ze względu na brak specyficznych testów diagnostycznych i niespecyficzne objawy.12 Jest to rozpoznanie z wykluczenia, co oznacza, że należy wyeliminować inne potencjalne przyczyny objawów, takie jak:12

Obserwacje kliniczne wskazują, że wysokie poziomy ferrytyny są często spotykane u pacjentów z AOSD, jednak nie jest to znalezisko specyficzne. Skrajnie podwyższone poziomy ferrytyny w surowicy, sięgające nawet 75 500 ng/ml, były raportowane w AOSD. Kilku badaczy zgadza się, że poziomy ferrytyny powyżej 1000 ng/ml sugerują AOSD, a poziomy powyżej 4000 ng/ml są bardzo specyficzne dla tej diagnozy, gdy towarzyszą im odpowiedni obraz kliniczny.12

Podsumowanie

Choroba Stilla u dorosłych pozostaje schorzeniem o złożonej i nie w pełni wyjaśnionej etiologii. Dominujące hipotezy koncentrują się wokół roli czynników infekcyjnych jako wyzwalaczy u osób z genetyczną predyspozycją oraz nieprawidłowej aktywacji układu immunologicznego z następową burzą cytokinową.12

Wspólną cechą różnych teorii na temat przyczyn AOSD jest kompleksowa interakcja między czynnikami genetycznymi, środowiskowymi i immunologicznymi. Trwające badania są ukierunkowane na odkrycie konkretnych czynników genetycznych i środowiskowych, które przyczyniają się do jej rozwoju, co może w przyszłości prowadzić do bardziej ukierunkowanych metod terapeutycznych i lepszego zrozumienia tej rzadkiej, ale poważnej choroby.12

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Still Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK538345/
    Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder characterized by daily fever, inflammatory polyarthritis, and a transient salmon-pink maculopapular rash. […] The etiology of AOSD remains unknown. The prevailing hypothesis suggests that AOSD is a reactive syndrome wherein various infectious agents may act as triggers in individuals with a genetic predisposition. Both genetic factors and various infectious agents, including viruses and bacteria like Yersinia enterocolitica and Mycoplasma pneumoniae, have been proposed as significant contributors to the development of AOSD. […] Uncertainty persists regarding the uniform presence of etiopathogenic factors among all AOSD patients. A French study involving 62 patients demonstrated an association between AOSD and specific human leukocyte antigen (HLA) subtypes (B17, B18, B35, and DR2). […] Specific case reports have highlighted instances of the disease occurring in twins.
  • #1 Adult-onset Still’s disease and the role of dermatological manifestations: A case report and literature review
    https://www.spandidos-publications.com/10.3892/etm.2020.9515
    Adult-onset Still’s disease (AOSD) is a rare inflammatory systemic disease with unknown etiology, characterized by spiking fever, evanescent rash, arthralgia and arthritis, leukocytosis and possible multiorgan involvement. […] The AOSD etiology remains unclear, despite several hypotheses that have been formulated. Viral infections, genetic factors and immune dysfunction, cytokine-mediated inflammation and apoptosis disorder may be involved in the pathogenesis of this disease. […] The involvement of a triggering infectious agent has been suspected, among the incriminated microorganisms being rubella virus, measles, Echovirus 7, Coxsackievirus B4, Cytomegalovirus, Epstein-Barr virus, Human herpes virus 6, Parainfluenza, Influenza A, Adenovirus, Hepatitis B and C, Parvovirus B19, Mycoplasma pneumonia, Chlamydia pneumonia, Yersinia enterocolitica, Brucella abortus, Borrelia burgdorferi, but a cause-effect relation has not been proven yet.
  • #1 Pathogenesis, disease course, and prognosis of adult-onset Still’s disease: an update and review
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6940076/
    Pathogenesis of AOSD involves genetic background, infectious triggers, and immunopathogenesis, mainly the activation of macrophages and neutrophils followed by a cytokine storm. […] The etiology of AOSD is still unclear, while there is evidence that various mechanisms contribute to the pathogenesis of AOSD, mainly including genetic susceptibility, infectious triggers, activation of inflammation, and deficient resolution of inflammation. […] It has long been suspected that infections, especially viral infections, are potential triggers of AOSD due to the similar symptoms between them. […] A study consisting of 100 AOSD patients has investigated the presence of antibodies against virus, virus DNA load, and nucleic acid sensors (IFI16 and AIM2). It shows that CMV infection is strongly related to the initiation or amplification of inflammatory responses in AOSD. It is suggested that viral infection may trigger the initiation or relapse of AOSD. […] The activation and amplification of inflammation, characterized by a cytokine storm, is the hallmark of AOSD. […] Deficiency in resolution of inflammation has been hypothesized to play a role in the pathogenesis of AOSD.
  • #1 Adult-Onset Still’s Disease – DoveMed
    https://www.dovemed.com/diseases-conditions/adult-onset-stills-disease
    There is no known definitive cause for Adult-Onset Stills Disease. […] It is thought to be triggered by a viral or bacterial infection, causing an abnormal immune response in the body. Such infections may include Lyme disease and infectious mononucleosis. […] No particular infection has been found to consistently result in Adult-Onset Stills Disease. […] Some researchers consider it to be an adult form of systemic juvenile inflammatory arthritis (JIA). […] Adult-Onset Stills Disease is linked to many HLA types often linked to autoimmune and autoinflammatory diseases.
  • #1 Adult-onset Still’s disease | Symptoms, treatments and new research
    https://versusarthritis.org/about-arthritis/conditions/adult-onset-still-s-disease/
    Adult-onset Stills disease is an autoimmune condition. This means that the condition is caused by your body’s immune system. […] The cause of AOSD isn’t known, but we do know it generally affects more women than men, and that it mostly starts in people aged between 16 and 35. But adults can get it at any age. […] It’s thought that people who develop AOSD may have genes that make them more likely to get it, but that there also needs to be something in the environment that triggers the condition, such as an infection.
  • #1 Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach
    https://www.mdpi.com/2077-0383/10/4/733
    Adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. The cause of this complex disorder, which usually affects young adults, remains unknown. […] The causal inferences between genetics and AoSD are controversial. Human genetic factors apparently contribute to SoJIA in children, whereas the underlying genomic susceptibility in the adult form is unclear. […] Logically, many investigators focused on identifying infectious triggers and described the occurrence of AoSD after infection with cytomegalovirus, Epstein-Barr, influenza, Mycoplasma, hepatitis, etc. […] Other studies have examined the relationship between cancer and AoSD and reported malignancy-mediated autoinflammation in breast cancer, thyroid cancer, melanoma, lung cancer and haematological malignancies, mostly lymphomas.
  • #1 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 autoimmune or autoinflammatory. […] The cause of adult Stills disease is unknown. Research suggests that it may be triggered by an infection.
  • #1 Adult-onset Still’s disease: Clinical manifestations and diagnosis – UpToDate
    https://www.uptodate.com/contents/adult-onset-stills-disease-clinical-manifestations-and-diagnosis
    Although the specific etiology of AOSD remains unknown, it is likely the manifestation of an autoinflammatory cascade due to innate immune cell activation. This differentiates it from autoimmune mediated inflammatory diseases in which autoantibodies or autoantigen-specific T and/or B cells lead to inflammation and tissue damage. […] The pathologic amplification of the inflammatory response (ie, “cytokine storm”) in AOSD is likely multifactorial, possibly due to infectious and/or genetic triggers. The final common pathway of the inflammatory response has been attributed to NOD-, LRR-, and pyrin domain-containing protein 3 (NLRP3) inflammasome activation with interleukin 18 (IL-18) overproduction.
  • #1 Azthena logo with the word Azthena
    https://www.news-medical.net/health/Stills-Disease-Causes.aspx
    Auto-inflammatory disorder: Auto-inflammatory disorders (different from autoimmune disorders) are characterized by recurrent episodes of inflammation due to inherent abnormality in the immune system. […] Cytokine disorder: Cytokines (chemicals secreted from immune system cells) which play a vital role in normal immunological function are also on researchers radar. Interleukin-1 (IL-1) is a type of cytokine which mediates cell response to inflammation. Abnormal IL-1 levels have been found in several patients with Stills disease. Other cytokines such as tumor necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6) may also play a vital role in immune mediated response associated with Stills disease. The detailed mechanisms and pathways of these chemical mediators, and possible spots therein for therapeutic intervention are being investigated in preclinical and clinical trials.
  • #1 Adult onset Still disease
    https://dermnetnz.org/topics/adult-onset-still-disease
    Adult-onset Still disease is rare, with an incidence of less than one in 100,000 people. It usually begins between 16 and 35 years of age. […] The exact cause of adult-onset Still disease is not known. One theory is that the disease is caused by infection. Another theory is that Still disease is a hypersensitive or autoimmune disorder (this is where the body’s immune system which normally fights off infections or illness suddenly starts to attack the body’s cells and tissues it is suppose to protect).
  • #1 HIE Multimedia – Adult Still disease
    https://adamcertificationdemo.adam.com/content.aspx?productid=117&pid=1&gid=000450
    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. […] There is no known prevention.
  • #1 ADULT ONSET STILL’S DISEASE | Comprehensive Rheumatology
    https://comprehensiverheumatology.com/index.html@p=1348.html
    Adult-onset Stills disease (AOSD) is a rare, systemic inflammatory disorder of unknown etiology. […] AOSD is a challenging diagnosis due to its rarity and nonspecific symptoms. […] Diagnosis also requires that autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus, and infections are ruled out. […] AOSD can be a chronic and relapsing-remitting disease with variable clinical courses.
  • #1 Adult Onset Still’s Disease: A Case Report | British Journal of Medical Practitioners
    https://www.bjmp.org/content/adult-onset-stills-disease-case-report
    Adult Onset Stills disease (AOSD) is an inflammatory disorder of unknown etiology. […] The etiology of adult Stills disease (ASD) is unknown; both genetic factors and a variety of infectious triggers have been suggested as important, but there has been no proof of an infectious etiology, and the evidence supporting a role for genetic factors has been mixed. […] Proposed pathogens have included numerous viruses; suspected bacterial pathogens include Yersinia enterocolitica and Mycoplasma pneumoniae. […] AOSD has been associated with markedly elevated serum ferritin concentrations in as much as 70 percent of patients. […] Indeed, extreme elevation of serum ferritin up to 75,500 ng/mL has been reported in AOSD. […] Several investigators agree that ferritin levels above 1,000 ng/mL are suggestive of AOSD while levels greater than 4,000 ng/mL are very specific for this diagnosis when accompanied by a compatible clinical picture.
  • #1 Adult-Onset Still’s Disease: Causes, Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/adult-onset-stills-disease-aosd
    Adult-onset Stills disease is a rare type of inflammatory arthritis that develops unexpectedly in early adulthood. […] Inflammation in your body causes the symptoms of adult-onset Stills disease. Your immune system usually generates inflammation to fight infections and heal injuries. Its supposed to be a temporary intervention. But in certain diseases, like AOSD, your immune system malfunctions and generates inflammation continuously, even when theres no infection to fight or injury to heal. […] Researchers still dont know exactly why this happens. Like juvenile Stills disease (juvenile idiopathic arthritis), adult Stills disease is idiopathic, which means the cause is unknown. It doesnt appear to be genetic.
  • #1 Still’s disease – AOSD, SJIA | Sobi
    https://www.sobi.com/en/stills-disease
    Stills disease is believed to result from a combination of genetic, environmental, and immunological factors. […] Hormonal and environmental factors are believed to play a part. […] The disease may be triggered by viral infection or by allergic reactions. […] The exact cause or causes of AOSD of SJIA are still not fully understood. […] Ongoing research aims to uncover the specific genetic and environmental factors that contribute to its development. […] Stills disease involves the body’s immune system responding inappropriately or mistakenly, leading to attacks on its own tissues or excessive inflammation. […] The condition is thought to be autoimmune or autoinflammatory.
  • #2 ADULT ONSET STILL’S DISEASE | Comprehensive Rheumatology
    https://comprehensiverheumatology.com/index.html@p=1348.html
    Adult-onset Stills disease (AOSD) is a rare, systemic inflammatory disorder of unknown etiology. […] AOSD is a challenging diagnosis due to its rarity and nonspecific symptoms. […] Diagnosis also requires that autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus, and infections are ruled out. […] AOSD can be a chronic and relapsing-remitting disease with variable clinical courses.
  • #2 Adult Still disease | Health Library | Memorial Health System
    https://www.mhsystem.org/health-library/con-20164392/
    The cause of adult Still disease is not known. Some researchers suspect it might be triggered by a viral or bacterial infection.
  • #2 Still’s disease: Causes, symptoms, and treatment
    https://www.medicalnewstoday.com/articles/stills-disease
    AOSD is a rare auto-inflammatory disease that affects the entire body. The cause of the condition is unknown, but it results in characteristic symptoms that include fever, a pink-colored rash, and joint pain. […] At present, researchers are unsure of the cause of AOSD and refer to the condition as idiopathic. However, they believe that a combination of genetic factors and an infection that acts as a trigger may play a role. […] Some evidence suggests that genes that produce proteins to help mediate inflammation likely play a role in the development of the condition. […] Similarly, certain infectious agents may trigger or exacerbate symptoms of AOSD. These may include: Yersinia enterocolitica, Mycoplasma pneumoniae, cytomegalovirus (CMV). […] As such, AOSD may be a reactive condition in which certain infections act as triggers in a genetically predisposed individual.
  • #2 Orphanet: Adult-onset Still disease
    https://www.orpha.net/en/disease/detail/829
    The etiology of AOSD and its underlying pathogenetic mechanisms are not known. No risk factors for the disease have been identified so far, but environmental factors are suspected. Several infectious conditions have been reported to be associated with onset of the disease (e.g. Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus, Coxsackie virus, hepatitis A, B, and C viruses, and Mycoplasma pneumoniae).
  • #2 Adult-onset Still’s disease and the role of dermatological manifestations: A case report and literature review
    https://www.spandidos-publications.com/10.3892/etm.2020.9515
    Adult-onset Still’s disease (AOSD) is a rare inflammatory systemic disease with unknown etiology, characterized by spiking fever, evanescent rash, arthralgia and arthritis, leukocytosis and possible multiorgan involvement. […] The AOSD etiology remains unclear, despite several hypotheses that have been formulated. Viral infections, genetic factors and immune dysfunction, cytokine-mediated inflammation and apoptosis disorder may be involved in the pathogenesis of this disease. […] The involvement of a triggering infectious agent has been suspected, among the incriminated microorganisms being rubella virus, measles, Echovirus 7, Coxsackievirus B4, Cytomegalovirus, Epstein-Barr virus, Human herpes virus 6, Parainfluenza, Influenza A, Adenovirus, Hepatitis B and C, Parvovirus B19, Mycoplasma pneumonia, Chlamydia pneumonia, Yersinia enterocolitica, Brucella abortus, Borrelia burgdorferi, but a cause-effect relation has not been proven yet.
  • #2 Rare Disease Guide: Adult-Onset Still’s Disease
    https://www.webmd.com/arthritis/adult-onset-stills-disease
    Adult-onset Stills disease is a rare type of arthritis that starts in adulthood. […] Doctors dont know whats behind adult-onset Stills disease. Research suggests that infection or toxic substances in your body can trigger it. Your immune system overreacts to these triggers and sets off the disease. […] Experts consider adult-onset Stills disease an auto-inflammatory condition because the immune system plays a role in its symptoms. […] Research also shows that people with adult-onset Stills disease have higher levels of proteins called cytokines than people who don’t have the disease.
  • #2 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 disease occurring in persons aged 16 years and resembling a systemic form of juvenile idiopathic arthritis. […] The exact etiology of AOSD is still unknown. It has been associated with several different human leukocyte antigens (HLAs), including HLA-Bw35, HLA-DR4, and HLA-DrW6; however, the strengths of these associations remain unclear. […] 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.
  • #2 What is adult-onset Still’s disease (AOSD)? | NRAS
    https://nras.org.uk/resource/what-is-adult-onset-stills-disease-aosd/
    Adult Onset Stills Disease (AOSD) is an auto-inflammatory disease. This means that the inflammation is generated by a disturbance in immune function. […] Occasionally a virus may trigger the illness; however, a sore throat is also a symptom of the illness, and therefore there may be confusion over whether this is the cause or the start of the illness. […] There is much research underway to understand the mechanism behind the auto-inflammatory disease and what generates the inflammation. It is known that high levels of the inflammatory proteins interleukin-1 and interleukin-6 are present.
  • #2 Refractory Cases of Adult Onset Still’s Disease Successfully Treated with TNF-α Blocker
    https://www.jrd.or.kr/journal/view.html?uid=541&vmd=Full
    Adult onset Still’s disease (AOSD) is a systemic inflammatory disorder of unknown etiology characterized by spiking fever, evanescent salmon-colored rash, polyarthritis and leukocytosis. […] Although the etiology and pathogenesis of AOSD is not elucidated clearly, the pathogenetic role of inflammatory cytokines, such as interleukin (IL)-6, IL-8, IL-18 and tumor necrosis factor (TNF)-α were suggested and the correlations of their levels with disease activity were also reported. […] These results raise a possibility that the blocking of these cytokines may provide a therapeutic benefit in controlling disease activity and relieving the symptoms of AOSD.
  • #2 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. […] The etiology of AOSD is not fully characterized in adults, with studies suggesting genetic and infectious triggers. […] The pathophysiology of AOSD has not been fully delineated. However, biomarkers and cytokine signalling pathways in the AOSD disease process have been hypothesized and described. […] Significant overdrive of the innate immune system and excess production of proinflammatory cytokines IL-1, IL-6, and IL-18 are crucial in the pathophysiology. […] One theory for the overproduction of cytokines is retrograde activation of macrophages and neutrophils by IL-1. […] The role of genetics in AOSD remains unclear. […] Currently, research is being conducted to see whether alleles that correlate strongly with systemic-onset juvenile idiopathic play a role in AOSD.
  • #2 Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach
    https://www.mdpi.com/2077-0383/10/4/733
    In short, AoSD is a diagnosis of exclusion. The process of eliminating similar medical conditions is most likely to take a considerable amount of time. […] AoSD belongs to this group of disorders and is thought to be “the archetype of non-familial, or sporadic, systemic autoinflammatory disorders.” […] The exact underlying cause of AoSD is not fully understood. We still do not know what exactly triggers DAMPs and PAMPs. […] The massive release of cytokines in patients with AoSD over a prolonged period of time can be fatal. Deficient resolution of inflammation may be mostly due to failures in immune system self-regulation.
  • #2 Still’s Disease – Symptoms, Causes, and Treatment | Metropolis TruHealth Blog
    https://www.metropolisindia.com/blog/preventive-healthcare/stills-disease-causes-and-treatment
    Stills Disease is a chronic, progressive autoimmune condition. It is caused by an abnormal response of the body’s immune system. […] Although the precise cause of Stills Disease is yet to be determined, it is generally believed that genetic, hormonal, and environmental components are at its core. […] Research suggests that the endocrine system plays a huge role in the development of Still’s. It is believed that inflammatory cells tend to increase when the body’s hormones are imbalanced. This leads to the symptoms of Still’s, like fever and joint pain. […] Heredity also seems to play a role in the development of Still’s. If someone in your family has the disease, your chances of having it also increase. […] Prolonged exposure to certain environmental toxins, such as those emitted by industry and agriculture, can damage the lungs, triggering an inflammatory response. […] Infection by certain viruses, including parvovirus, rubella, and Epstein-Barr virus, can also lead to the development of Still’s. […] It is possible for allergens, such as pollen and pet dander, to trigger an inflammatory response leading to Still’s.
  • #2 HIE Multimedia – Adult Still disease
    https://adamcertificationdemo.adam.com/content.aspx?productid=117&pid=1&gid=000450
    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. […] There is no known prevention.
  • #2 Adult Still’s disease
    https://www.mymlc.com/health-information/diseases-and-conditions/a/adult-stills-disease/
    Adult Still’s disease is a rare type of inflammatory arthritis that features fevers, rash and joint pain. […] It’s not certain what causes adult Still’s disease. Some researchers suspect the condition might be triggered by a viral or bacterial infection. […] Age is the main risk factor for adult Still’s disease, with incidence peaking twice: once from 15 to 25 years and again from 36 to 46 years. Males and females are equally at risk.
  • #2 Best of 2018: 5 Mistakes When Diagnosing Adult-Onset Still’s Disease | RheumNow
    https://rheumnow.com/blog/best-2018-5-mistakes-when-diagnosing-adult-onset-still%25E2%2580%2599s-disease
    Adult-onset Still’s disease is a rare systemic inflammatory disorder of unknown etiology and pathogenesis. […] There are no diagnostic clinical manifestations, serologic tests or histopathologic findings and thus, the diagnosis is one of exclusion. […] Over-emphasis on ferritin levels for diagnosis. While hyperferritinemia may be caused by iron overload, vasculitis or the macrophage activation syndrome, there is nothing specific about this finding and AOSD.
  • #2 A Case Report of Still’s Disease in the Adult | Cardoso | Journal of Medical Cases
    https://www.journalmc.org/index.php/JMC/article/view/2847/2179
    Adult Stills disease (ASD) is a rare systemic inflammatory disorder of unknown etiology, typically characterized by a clinical triad (daily spiking high fevers, evanescent rash, and arthritis) and a biological triad (hyperferritinemia, hyperleucocytosis with neutrophilia and abnormal liver function test). […] There are no specific diagnostic tests for ASD, so the diagnosis of ASD remains one of exclusion and the differential diagnosis may be lengthy. […] The laboratory findings in ASD reflect the systemic inflammation and cytokine cascade present, and none of the findings are specific for ASD. […] There is no association with rheumatoid factor or antinuclear antibody positivity. […] There are no specific diagnostic tests for ASD. The diagnosis of ASD remains one of exclusion and the differential diagnosis may be lengthy. Infectious, neoplastic, autoimmune diseases or drug hypersensitivity reactions can mimic the clinical manifestation of ASD.
  • #2 Unveiling the Mystery of Adult-Onset Still’s Disease: A Compelling Case Report
    https://www.mdpi.com/2075-1729/14/2/195
    Adult-onset Still’s disease (AOSD) is a complex autoimmune inflammatory disease of unknown etiology. […] The etiology of the disease remains unknown, but it is believed that various infectious agents may trigger it in susceptible individuals, such as Rubivirus, Morbillivirus, Mycoplasma Pneumoniae, or Chlamydia Pneumoniae. […] However, no single pathogenic trigger has been clearly identified, suggesting the involvement of multiple factors. […] The key challenge in this clinical case was really distinguishing between infection and autoimmunity. Importantly, both conditions can coexist, and an infection may even trigger autoimmunity. […] Moreover, immune activation from infection is also a common trigger for MAS-HLH both in patients with a genetic predisposition and in sporadic cases with no underlying genetic cause identified. […] AOSD is a rare autoinflammatory disease with diverse clinical presentations and non-specific features that can mimic various conditions. […] Despite the increased understanding of AOSD, it remains a diagnostic and therapeutic challenge with many gaps in knowledge, especially concerning AOSD-MAS.
  • #3 Adult Still’s disease Information | Mount Sinai – New York
    https://www.mountsinai.org/health-library/diseases-conditions/adult-stills-disease
    Adult Still disease is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis. […] The cause of adult Still disease is unknown. No risk factors for the disease have been identified. […] There is no known prevention.
  • #3 Adult-onset Still disease | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/adult-onset-still-disease?lang=us
    Adult-onset Still disease (AOSD) is a rare multisystem inflammatory disorder and was originally thought to be a variant of Still disease, a pediatric arthropathy. […] The cause of adult-onset Still disease is unknown but genetic, environmental and infectious factors may play a role. Elevated levels of various cytokines are seen in adult-onset Still disease. Interleukin-18-induced activation of macrophages and neutrophils appears to be important in Still disease. Interleukin-1- also seems to be important in Still disease causing neutrophil production and diapedesis.
  • #3 Still’s disease – AOSD, SJIA | Sobi
    https://www.sobi.com/en/stills-disease
    Stills disease is believed to result from a combination of genetic, environmental, and immunological factors. […] Hormonal and environmental factors are believed to play a part. […] The disease may be triggered by viral infection or by allergic reactions. […] The exact cause or causes of AOSD of SJIA are still not fully understood. […] Ongoing research aims to uncover the specific genetic and environmental factors that contribute to its development. […] Stills disease involves the body’s immune system responding inappropriately or mistakenly, leading to attacks on its own tissues or excessive inflammation. […] The condition is thought to be autoimmune or autoinflammatory.
  • #3 Juvenile idiopathic arthritis / adult-onset Still’s disease » Global Autoimmune Institute
    https://www.autoimmuneinstitute.org/autoimmune-resources/autoimmune-diseases-list/juvenile-idiopathic-arthritis-stills/
    When diagnosed in adults, JIA is more commonly called adult-onset Stills disease. […] The cause of Stills disease is unknown, though several different treatments are available to help manage symptoms. […] Chronic organ and joint inflammation leads to most complications including joint destruction, heart inflammation, excess fluid around the lungs, and macrophage activation syndrome. […] There are two peaks of age ranges when developing adult Stills disease is most common, 15-25 years and 36-46 years.