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Adult-Onset Still Disease

Adult Onset Still Disease

Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy. The diagnosis rests on clinical criteria and a thorough laboratory workup, with an emphasis on inflammatory markers and ferritin.


Presentation

Adult-onset Still disease (AOSD), commonly referred in the literature as the adult form of systemic-onset juvenile idiopathic arthritis, is a disorder of unknown etiology [1] [2]. The clinical presentation might be quite different from patient to patient, but the following manifestations are included in the diagnostic criteria [1] [3] [4] [5]:

  • Fever - Although not specific, the recurring appearance of very high fever (> 39°C) that lasts for a few hours and mainly happens in the late afternoon or early evening period, is a hallmark of AOSD [3] [6].
  • Joint pain - The involvement of knees, wrists, or ankles is another important manifestation of AOSD [3] [4]. Arthritis usually accompanies fever and has a relatively short duration as well [3].
  • Rash - A salmon-pink or erythematous nonpruritic maculopapular rash, developing together with fever and joint pain, is frequently observed, and is most commonly seen on the trunk and the proximal limbs [5].

Other common manifestations of AOSD are lymphadenopathy, hepatosplenomegaly, muscle aches, and pharyngitis [1] [7]. The clinical course of the disorder can be highly variable, ranging from mild and self-limiting to severely debilitating disease [3]. Furthermore, AOSD may cause a myriad of complications in affected individuals, such as pulmonary hypertension, thrombotic thrombocytopenic purpura (TTP), amyloidosis, and macrophage activation syndrome [1]. An early detection of this disorder could significantly aid in successful treatment and improvement of the overall quality of life.

Splenomegaly
  • Adult-onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon-pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction, and splenomegaly[ncbi.nlm.nih.gov]
  • Arthritis (OR, 8.6; 95% confidence interval [CI], 1.5-49.1; p 0.014), pharyngitis (OR, 6.9; 95% CI, 1.5-30.2; p 0.010), splenomegaly (OR, 5.4; 95% CI, 1.1-26.7; p 0.039), and neutrophilic leukocytosis (OR, 18.1; 95% CI, 3.5-93.6; p 0.001) were significantly[ncbi.nlm.nih.gov]
  • AOSD patients with RHS showed significantly higher frequencies of splenomegaly, hepatomegaly, and lymphadenopathy than did those without RHS.[ncbi.nlm.nih.gov]
  • Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis.[ncbi.nlm.nih.gov]
  • . – Elevated acute-phase reactants: elevated ESR, CRP, white count, and hyperferritinemia (69-97% of patients) -Other findings that may be found include: myalgia, sore throat, hepatomegaly, splenomegaly, lymphadenopathy, serositis, abdominal pain, elevated[utswim.wordpress.com]
Generalized Lymphadenopathy
  • lymphadenopathy Pathophysiology Pathophysiology is yet to be clearly defined Interleukins, macrophage colony stimulating factor, interferon gamma and tumor necrosis factor alpha may play a role May also be due to genetic factors, immune dysfunction and[pathologyoutlines.com]
  • It differs from classical rheumatoid arthritis in that it is more often marked by acute febrile onset, and generalized lymphadenopathy and hepatosplenomegaly are more prominent.[icd10data.com]
  • Other characteristic manifestations include a prodromal sore throat, arthralgias, myalgias, weight loss, serositis, hepatomegaly (often with elevated hepatic enzymes), splenomegaly and generalized lymphadenopathy.[rheumnow.com]
  • B (Minor criteria) comprises (i) TLC or 15000/mm 3 (ii) Still's rash (iii) Pleuritis or pericarditis (iv) Hepatomegaly or splenomegaly or generalized lymphadenopathy. Our patient qualified for all criteria from A and two criteria from B.[ijnm.in]
  • On examination, she looked pale, febrile (temperature 39.2ºC), had non-suppurative pharyngitis and generalized lymphadenopathy. The lymph nodes were slightly tender, small and discrete.[pjms.com.pk]
Fever
  • Arthritis usually accompanies fever and has a relatively short duration as well.[symptoma.com]
  • We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption.[ncbi.nlm.nih.gov]
  • One of the most common presentations of the disease is fever of unknown origin. Early diagnosis can be difficult because fever of unknown origin is more commonly seen with other conditions such as malignancy or infection.[ncbi.nlm.nih.gov]
  • Fevers develop each day (usually in the afternoon or evening), and the rash comes and goes with the fever. Other signs and symptoms may include abdominal pain, pleurisy, swollen lymph nodes, and weight loss.[rarediseases.info.nih.gov]
  • Adult-onset Still disease (AOSD) is a systemic inflammatory disease characterized by fever, arthritis and rash.[ncbi.nlm.nih.gov]
Lymphadenopathy
  • Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy.[symptoma.com]
  • Adult onset Still's disease (AOSD) is a systemic inflammatory disease characterized by high-fevers, articular involvement, maculopapular rash, hepatosplenomegaly, lymphadenopathy, and a neutrophilic leukocytosis.[ncbi.nlm.nih.gov]
  • Adult-onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon-pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction,[ncbi.nlm.nih.gov]
  • Minor criteria include sore throat, lymphadenopathy and/or splenomegaly, abnormal liver function tests, and negative rheumatoid factor and ANA. Elevated ferritin is often found and may assist in diagnosis.[orpha.net]
  • Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy.[ncbi.nlm.nih.gov]
High Fever
  • A 48-year-old woman was admitted because of spiking high fever, sore throat, and jaundice.[ncbi.nlm.nih.gov]
  • Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy.[symptoma.com]
  • Adult onset Still's disease (AOSD) is a systemic inflammatory disease characterized by high-fevers, articular involvement, maculopapular rash, hepatosplenomegaly, lymphadenopathy, and a neutrophilic leukocytosis.[ncbi.nlm.nih.gov]
  • Major diagnostic criteria include arthralgia for more than 2 weeks, intermittent high fever for more than 1 week, characteristic rash, and white blood cell count above 10,000.[orpha.net]
  • Within 1 month, she began spiking high fevers, developed diffuse joint pain, and had marked elevations of ferritin, C-reactive protein, and erythrocyte sedimentation rate, characteristic of AOSD.[ncbi.nlm.nih.gov]
Anemia
  • AOSD patients with RHS showed higher frequencies of leukopenia, anemia, thrombocytopenia, hypoalbuminemia, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, and elevated lactate dehydrogenase levels than did those without RHS.[ncbi.nlm.nih.gov]
  • Abstract Adult-onset Still disease (AOSD) is characterized by fever, skin rash, and lymphadenopathy with leukocytosis and anemia as common laboratory findings.[ncbi.nlm.nih.gov]
  • Autoimmune hemolytic anemia. Nihon Rinsho 2008;66:520–523. 15. Pirofsky B. Clinical aspects of autoimmune hemolytic anemia. Semin Hematol 1976;13:251–265.[synapse.koreamed.org]
  • METHODS: Previously we developed a method to measure the iron content of ferritin; we investigated the usefulness of this method to establish the iron status of patients with anemia of inflammation.[jrheum.org]
  • CC: 6 days of fevers and URI symptoms ID: 67 yo female with pernicious anemia presents with 6 days of fevers and URI symptoms and 3 weeks of nonpruritic, round, scaly rash on her forearm.[uscmedicine.blog]
Swinging Fever
  • In hospital it was found that she had swollen joints, a high swinging fever and blood tests that were consistent with severe inflammation. The Rheumatology specialists were called and a diagnosis of Adult Onset Still’s Disease was made.[nras.org.uk]
Pharyngitis
  • Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy.[symptoma.com]
  • Arthritis (OR, 8.6; 95% confidence interval [CI], 1.5-49.1; p 0.014), pharyngitis (OR, 6.9; 95% CI, 1.5-30.2; p 0.010), splenomegaly (OR, 5.4; 95% CI, 1.1-26.7; p 0.039), and neutrophilic leukocytosis (OR, 18.1; 95% CI, 3.5-93.6; p 0.001) were significantly[ncbi.nlm.nih.gov]
  • The presenting features are variable and may include high fever ( 39 C) with daily spikes, sore throat or pharyngitis, arthralgia or arthritis ( 65% of patients), transient maculopapular rash, and more rarely myalgia, lymphadenopathy, hepatosplenomegaly[orpha.net]
  • 2 Department of Internal Medicine, The University of Chicago Medical Center, Chicago, IL, USA Abstract: Adult-onset Still's disease (AOSD) – a multi-systemic inflammatory condition characterized by high fevers, polyarthritis, an evanescent rash, and pharyngitis[dovepress.com]
  • Pharyngitis Pharyngitis, non-suppurative occurs in approximately 70% of the patients [ 6 ]. Hepatomegaly with or without elevation in liver aminotransferases frequently occurs.[oapublishinglondon.com]
Hepatomegaly
  • Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%.[ncbi.nlm.nih.gov]
  • AOSD patients with RHS showed significantly higher frequencies of splenomegaly, hepatomegaly, and lymphadenopathy than did those without RHS.[ncbi.nlm.nih.gov]
  • Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis.[ncbi.nlm.nih.gov]
  • . – Elevated acute-phase reactants: elevated ESR, CRP, white count, and hyperferritinemia (69-97% of patients) -Other findings that may be found include: myalgia, sore throat, hepatomegaly, splenomegaly, lymphadenopathy, serositis, abdominal pain, elevated[utswim.wordpress.com]
  • Physical examination revealed pallor, hepatomegaly.[ijnm.in]
Hepatosplenomegaly
  • Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy.[symptoma.com]
  • Adult onset Still's disease (AOSD) is a systemic inflammatory disease characterized by high-fevers, articular involvement, maculopapular rash, hepatosplenomegaly, lymphadenopathy, and a neutrophilic leukocytosis.[ncbi.nlm.nih.gov]
  • […] interferon gamma and tumor necrosis factor alpha may play a role May also be due to genetic factors, immune dysfunction and infections Clinical features Systemic inflammatory disease manifesting with spiking fever, sore throat, arthralgia, skin rash and hepatosplenomegaly[pathologyoutlines.com]
  • The presenting features are variable and may include high fever ( 39 C) with daily spikes, sore throat or pharyngitis, arthralgia or arthritis ( 65% of patients), transient maculopapular rash, and more rarely myalgia, lymphadenopathy, hepatosplenomegaly[orpha.net]
  • HLH is also characterized by fever, hepatosplenomegaly, increased ferritin levels all of which are also features of AOSD.[ijpmonline.org]
Retinal Hemorrhage
  • Fundoscopic examination showed multiple areas of retinal whitening, discrete superficial retinal hemorrhages, and few characteristic Purtscher flecken in the left eye. The patient was diagnosed with adult-onset Still disease.[ncbi.nlm.nih.gov]
Eruptions
  • Emerging data also suggest that patients with persistent eruption have a worse prognosis.[ncbi.nlm.nih.gov]
  • We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption.[ncbi.nlm.nih.gov]
  • Therefore, biopsy of atypical eruptions in AOSD patients is recommended because it is likely that the highly distinctive histopathologic features will allow these eruptions to be readily classified. Copyright 2012 Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • KEYWORDS: adult-onset Still disease; evanescent rash; hyperferritinemia; paraneoplastic syndrome; persistent pruritic eruption[ncbi.nlm.nih.gov]
  • A 55-year-old Asian woman presented initially with a "burning" and severely pruritic eruption on her face, hands, and arms, thought clinically to be urticaria.[ncbi.nlm.nih.gov]
Koebner Phenomenon
  • It commonly appears on the trunk, neck, or extremities and may be associated with dermatographism, Koebner phenomenon (lesions arising at sites of trauma/pressure), pruritus, or urticaria. Fixed dermal plaques have been rarely described.[rheumaknowledgy.com]
  • In one-third of patients, the rash is mildly pruritic and develops at sites of pressure or trauma (Koebner’s phenomenon). Arthralgia/Arthritis and Myalgia Intense arthralgias are a universal finding.[stillsdisease.org]
  • Cutaneous manifestations of AOSD: The common ones are Still’s Rash, Koebner phenomenon, Dermatographism. Uncommon: It includes Pruritis, Urticaria, Dermal plaques, Facial Rash, Alopecia, Erythema nodosumd and Raynaud’s phenomenon.[pjms.com.pk]
  • Look for the rash in areas subject to pressure like the beltline and under the breasts or it can be elicited by stroking the skin (Koebner’s phenomenon). The rash is frequently misdiagnosed as a drug reaction. Figure 1.[clinicaladvisor.com]
Exanthema
  • Definition / general Rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema and arthralgia, accompanied frequently by lymphadenopathy Essential features Fever, skin rash, joint pains, lymphadenopathy with paracortical[pathologyoutlines.com]
Arthritis
  • Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease.[ncbi.nlm.nih.gov]
  • Subtype-specific peripheral blood gene expression profiles in recent-onset juvenile idiopathic arthritis. Arthritis Rheum. 2009;60:2102–12. PubMedCentral PubMed CrossRef Google Scholar 41.[doi.org]
  • To evaluate the potential diagnostic value of carpal radiography in suspected cases of AOSD, a retrospective blinded analysis of 48 patients (16 each with AOSD, juvenile chronic arthritis, and adult-onset rheumatoid arthritis) was performed.[ncbi.nlm.nih.gov]
  • From Wikidata Jump to navigation Jump to search arthritis that is a rare from of inflammatory arthritis characterized by fevers, rash, and joint pain adult onset Still's disease adult-onset Still disease Adult still disease edit English adult-onset Still's[wikidata.org]
  • Arthritis Rheum 29 : 415–418 12. Terkeltaub R et al. (1981) HLA-Bw35 and prognosis in adult Still's disease. Arthritis Rheum 24 : 1469–1472 13.[doi.org]
Arthralgia
  • Arthralgia is usually correlated with fever spikes.[orpha.net]
  • Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient.[ncbi.nlm.nih.gov]
  • Adult onset still disease is a rare systemic inflammatory disease which presents with cardinal symptoms of spiking fever, arthralgia, and characteristic non pruritic evanescent salmon pink rash and neutrophilic leukocytosis.[ncbi.nlm.nih.gov]
  • It is characterized by high spiking fevers, arthritis or arthralgia, and an evanescent salmon-coloured rash. Many other systemic manifestations and laboratory test abnormalities may occur.[ncbi.nlm.nih.gov]
  • A 43-year-old man presented with a 3-week history of daily high-spiking fever, diffuse arthralgia and myalgia, sore throat, and a transient nonpruritic maculopapular rash.[ncbi.nlm.nih.gov]
Myalgia
  • Since March 2017, he has had progressive fatigue, myalgias, rash, weight loss, diarrhea, and recurrent low-grade fever. Subsequent bone marrow biopsy and FDG PET/CT demonstrated no active lymphoma.[ncbi.nlm.nih.gov]
  • A 43-year-old man presented with a 3-week history of daily high-spiking fever, diffuse arthralgia and myalgia, sore throat, and a transient nonpruritic maculopapular rash.[ncbi.nlm.nih.gov]
  • Five weeks earlier, sore throat, fever, malaise, and myalgias had developed. Broad-spectrum antibiotic therapy was administered, without improvement. A diagnosis was made.[blogs.nejm.org]
  • Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient.[ncbi.nlm.nih.gov]
  • The presenting features are variable and may include high fever ( 39 C) with daily spikes, sore throat or pharyngitis, arthralgia or arthritis ( 65% of patients), transient maculopapular rash, and more rarely myalgia, lymphadenopathy, hepatosplenomegaly[orpha.net]

Workup

The diagnosis of AOSD is primarily a one of exclusion, as numerous autoimmune, malignant, and infectious diseases induce the nonspecific signs and symptoms seen in this condition [1] [3] [7] [8]. Thus, a comprehensive clinical and laboratory workup is necessary. Firstly, during a history taking, patients should be asked about the course of the disease and the appearance of symptoms, whereas a thorough physical examination has an important role in identifying lymphadenopathy, cutaneous changes, joint inflammation, and hepatosplenomegaly.

A complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum ferritin should be evaluated in laboratory studies [1] [3] [4] [7] [8]. Additionally, rheumatoid factor (RF) and antinuclear antibodies (ANA), both performed to exclude conditions such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), are also recommended in the initial workup [3].

Current diagnostic criteria are divided into major (Fever ≥ 39°C, joint pain, skin rash, pharyngitis, granulocytosis of ≥ 80%, and glycosylated ferritin fraction of ≤ 20%) and minor (leukocytosis of > 10,000/mm3 and a typical skin rash) [3] [4] [7] [8]. AOSD is confirmed if ≥ 4 major criteria or 3 major and 2 minor criteria are met [1] [3] [4] [7].

Elevated Sedimentation Rate
  • The two most commonly associated with AOSD are an elevated sedimentation rate (ESR) and a high blood ferritin level.[thedoctorwillseeyounow.com]
  • The most common laboratory abnormalities include: greatly elevated sedimentation rate leukocytosis (in most cases between 15,000-30,000, mainly neutrophiles) thrombocytosis 400,000 elevated ferritin levels.[stillsdisease.org]
Total Bilirubin Increased
  • Subsequently, the serum level of ferritin decreased, but serum total bilirubin increased to 17.3 mg/dL. Therefore, cyclosporin was administered orally.[ncbi.nlm.nih.gov]
Human Papillomavirus
  • Genital human papillomavirus (HPV) infection has been reported to induce or exacerbate AIIDs, such as systemic lupus erythematosus (SLE). No guidelines are available for the management of genital warts in AOSD. Case report and literature review.[ncbi.nlm.nih.gov]

Treatment

  • Treatment failed in four cases (two with each anti-TNF-α) [ 15 ].[doi.org]
  • We report the successful use of abatacept in the treatment of a patient with AOSD manifested by polyarthritis, rash, fevers, elevated liver function tests, and ferritin levels refractory to treatment with methotrexate and hydroxychloroquine.[ncbi.nlm.nih.gov]
  • Biologic drugs, TNF-α inhibitors, and IL-1 and IL-6 blockers have been used for the treatment of patients with AOSD refractory to conventional treatment or those with life-threatening manifestations aiming for better disease control.[ncbi.nlm.nih.gov]
  • Management and treatment 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.[orpha.net]
  • Haraoui B et al. (2004) Anakinra in the treatment of adult onset Still's disease.[doi.org]

Prognosis

  • Prognosis The overall prognosis is generally good, once acute life-threatening manifestations have been controlled. Some patients with chronic disease and major joint involvement may have significantly altered quality of life.[orpha.net]
  • Systemic manifestations, per se, did not contribute to poor functional prognosis. Chronic articular disease had the worst outcome: 27% evolved to functional class III status, compared with none in the cyclic systemic groups.[ncbi.nlm.nih.gov]
  • Emerging data also suggest that patients with persistent eruption have a worse prognosis.[ncbi.nlm.nih.gov]
  • AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure.[ncbi.nlm.nih.gov]
  • Clinical presentations and prognosis have varied in different studies. OBJECTIVE: The objective of this study was to determine the clinical presentation and the evolution of AOSD at a tertiary referral center in southeast Brazil.[ncbi.nlm.nih.gov]

Etiology

  • The disease is an entity with heterogeneous pathology; and diverse suggested etiologies, clinical manifestations and prognoses.[ncbi.nlm.nih.gov]
  • Adult-onset Still disease (AOSD) is a rare condition disease of unknown etiology, characterized by quotidian or double quotidian spiking fever, with an evanescent pink-salmon rash, arthritis and multi-organ involvement.[ncbi.nlm.nih.gov]
  • Adult-onset Still disease (AOSD) is an inflammatory condition of unknown etiology that responds to glucocorticosteroids and disease-modifying antirheumatic drugs, particularly methotrexate.[ncbi.nlm.nih.gov]
  • Etiology 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.[orpha.net]
  • Adult-onset Still's disease is a rare systemic inflammatory disease of unknown etiology, characterized by daily high, spiking fevers, evanescent rash, and arthritis.[ncbi.nlm.nih.gov]

Epidemiology

  • Our study aims to analyze different aspects of articular manifestations complicating adult onset Still disease to define epidemiological, clinical and evolving characteristics of these complications.[panafrican-med-journal.com]
  • Summary Epidemiology Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation. The estimated prevalence of AOSD is more than 1/100,000 population.[orpha.net]
  • Magadur-Joly G et al (1995) Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54(7):587–590 PubMed CrossRef Google Scholar 4.[doi.org]
  • […] disease accompanied by a triad of spiking fever, maculopapular exanthema and arthralgia, accompanied frequently by lymphadenopathy Essential features Fever, skin rash, joint pains, lymphadenopathy with paracortical hyperplasia and immunoblastic reaction Epidemiology[pathologyoutlines.com]
  • References Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, et al. (1995) Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54: 587.[archivesofmedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD.[ncbi.nlm.nih.gov]
  • More important, novel biologic therapies have offered important clues to AOSD's underlying pathophysiology.[dovepress.com]
  • Pathophysiology is yet to be clearly defined Interleukins, macrophage colony stimulating factor, interferon gamma and tumor necrosis factor alpha may play a role May also be due to genetic factors, immune dysfunction and infections Clinical features[pathologyoutlines.com]
  • Additional research into the pathophysiology of PNGD is also warranted, as this would optimize medical management.[archivesofmedicine.com]
  • Autoinflammatory syndromes and cellular responses to stress: pathophysiology, diagnosis and new treatment perspectives. Best Pract Res Clin Rheumatol. 2012;26:505–33. PubMed CrossRef Google Scholar 52.[doi.org]

Prevention

  • Clinicians need to be aware of this condition so that they can correctly diagnose it and prevent its serious complications.[ncbi.nlm.nih.gov]
  • Treatment aims to control inflammation, and prevent joint damage and other complications.[rarediseases.info.nih.gov]
  • It is important to keep the inflammation under control to prevent more damage to the body. If prednisone is taken for a long time, a doctor may recommend taking calcium and vitamin D to prevent thinning of the bones.[arthritis.org]
  • When steroids are used they will often be given with other drugs to help to prevent side-effects.[nras.org.uk]
  • CTFPHC REFERENCE BOOK Screening for Colorectal Cancer CTFPHC REFERENCE BOOK Screening for Cognitive Impairment CTFPHC REFERENCE BOOK Screening for Depression CTFPHC REFERENCE BOOK Screening for Developmental Delay CTFPHC Review Canadian Task Force on Preventive[qxmd.com]

References

Article

  1. Kadavath S, Efthimiou P. Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med. 2015;47(1):6-14.
  2. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still's disease. Autoimmun Rev. 2014;13(7):708-722.
  3. Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis. 2006;65(5):564-572.
  4. Gopalarathinam R, Orlowsky E, Kesavalu R, Yelaminchili S. Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options. Case Reports in Rheumatology. 2016;2016:6502373.
  5. Mobini M, Ghasemian R, Zameni F. Adult Onset Still's Disease With Different Antibodies: A Case Report and Review of Literature. Acta Med Iran. 2016;54(10):683-685.
  6. Larson EB. Adult Still's disease—recognition of a clinical syndrome and recent experience. West J Med. 1985;142:665–671.
  7. Dudziec E, Pawlak-Buś K, Leszczyński P. Adult-onset Still’s disease as a mask of Hodgkin lymphoma. Reumatologia. 2015;53(2):106-110.
  8. Meijvis SC, Endeman H, Geers AB, ter Borg EJ. Extremely high serum ferritin levels as diagnostic tool in adult-onset Still's disease. Neth J Med. 2007;65(6):212-214.

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Last updated: 2019-07-11 20:34