scholarly journals Adult-Onset Still’s Disease: Novel Biomarkers of Specific Subsets, Disease Activity, and Relapsing Forms

2021 ◽  
Vol 22 (24) ◽  
pp. 13320
Author(s):  
Beatrice Maranini ◽  
Giovanni Ciancio ◽  
Marcello Govoni
Keyword(s):  
Clinical Symptoms ◽  
Interferon Γ ◽  
Immunosuppressive Drugs ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Systemic Inflammatory Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease of unknown etiology. Recent studies have demonstrated that the hallmark of AOSD is a cytokine storm, which is characterized by the excessive production of interleukin (IL)-1, IL-6, IL-18, tumor necrosis factor-α (TNF-α), and interferon-γ (IFN-γ), suggesting how pro-inflammatory cytokines play an important role in the pathogenesis of this disease. Actually, a certain proportion of patients (around 17–32%) with severe clinical symptoms achieves only partial remission or is resistant to both first-line corticosteroids and second-line DMARDs. These patients are defined as refractory AOSD patients, requiring higher dosage glucocorticoids, longer treatment duration, or the simultaneous introduction of immunosuppressive drugs, further leading to AOSD relapses. In this narrative review, we will analyze the latest literature data to unravel potential pathogenetic factors associated with specific patterns of AOSD disease or relapses in order to identify biomarkers that may guide clinical decisions, eventually leading to new therapeutic options.

scholarly journals Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Rajesh Gopalarathinam ◽  
Eric Orlowsky ◽  
Ramesh Kesavalu ◽  
Sreeteja Yelaminchili
Keyword(s):  
Diagnostic Workup ◽  
Differential Diagnoses ◽  
Treatment Modalities ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Systemic Inflammatory Disease ◽  
Adult Onset Still's Disease

Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 33-year-old African-American male who presented with one-month duration of FUO along with skin rash, sore throat, and arthralgia. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.

scholarly journals Interluekin-6 inhibitors for the treatment of adult-onset Still’s disease

2021 ◽  
Author(s):  
Yuko Kaneko
Keyword(s):  
Proinflammatory Cytokines ◽  
Cytotoxic T Cells ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Systemic Inflammatory Disease ◽  
Adult Onset Still's Disease ◽  
Multifunctional Cytokine

ABSTRACT Adult-onset Still’s disease is a systemic inflammatory disease characterized by high spiking fever, arthritis, evanescent skin rash, leukocytosis, and hyperferritinemia. The pathogenesis of adult-onset Still’s disease has not been fully understood yet; however, multiple proinflammatory cytokines, such as IL-1β and IL-6, play important roles in the development of adult-onset Still’s disease. IL-6 is a multifunctional cytokine that accelerates the differentiation of macrophages and cytotoxic T-cells and chemotaxis of neutrophils and macrophages. Serum concentrations of IL-6 well correlate with disease activity of adult-onset Still’s disease, and blockade of IL-6 has been proven to be effective in active adult-onset Still’s disease. This review will focus on the recent understanding of the role of proinflammatory cytokines of adult-onset Still’s disease and the efficacy of IL-6 inhibitors for the treatment of adult-onset Still’s disease.

scholarly journals Therapy with canakinumab for adult-onset still's disease

2019 ◽  
Vol 56 ◽  
pp. 35-40
Author(s):  
E. L. Nasonov
Keyword(s):  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Adult Onset ◽  
Still’S Disease ◽  
First Line ◽  
Disease Modifying Drugs ◽  
Still's Disease ◽  
First Line Therapy ◽  
Genetically Determined ◽  
Adult Onset Still's Disease

Still's disease in children (systemic-onset juvenile idiopathic arthritis, SoJIA) and in adults (adult-onset Still's disease) are considered as non-familial systemic autoinflammatory diseases of unknown etiology driven by similar immunopathogenetic mechanisms. The adult-onset Still's disease pathogenesis is based on genetically determined innate immunity disturbances and molecular basis of immunopathogenesis consists of NLRP3 inflammasomedependent mechanisms of inflammation characterized by hyperproduction of proinflammatory cytokines interleukin (IL) 1 and IL18. Nonsteroidal anti-inflammatory drugs, glucocorticoids, methotrexate and other disease modifying drugs are considered as «first line» medications for the treatment of adult-onset Still's disease and if they fail biologicals are recommended. A review of the literature data concerning anti-IL1 monoclonal antibodies administration in adult-onset Still's disease is presented, indicating good prospects for the use of canakinumab not only in case of resistance to standard therapy, but also as a «first-line» therapy in the onset of the disease.

scholarly journals COVID-19 and Adult-onset Still’s Disease as part of Hyperferritinemic Syndromes

2021 ◽  
Author(s):  
Tatsuya Kawasaki ◽  
Seido Ooka ◽  
Machiko Mizushima ◽  
Yuta Nakamura ◽  
Hiroki Ikeda ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Disease Onset ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Clinical Markers ◽  
Still’S Disease ◽  
Low Oxygen ◽  
Still's Disease ◽  
Appropriate Treatment ◽  
Adult Onset Still's Disease

Abstract The coronavirus disease (COVID-19) is known to cause hyperferritinemia and hemophagocytic lymphohistiocytosis (HLH). Including this laboratory parameter, clinical symptoms similar to COVID-19 have been observed in adult-onset Still’s disease (AOSD), catastrophic antiphospholipid syndrome (CAPS), macrophage activation syndrome (MAS), and septic shock, which has led to the proposal of a concept called ‘hyperferritinemic syndromes.’ Additionally, high levels of some clinical markers in both COVID-19 and AOSD make them difficult to differentiate. While the efficacy of ciclesonide had been expected for mild pneumonia with COVID-19, the efficacy of tocilizumab, which is a known treatment for AOSD, was not established. Here, we report the first known occurrence of COVID-19, diagnosed in March 2020, preceded by the diagnosis of AOSD, in April 2019, in a 65-year-old, otherwise healthy man. Following the diagnosis of the latter, the patient was first given prednisolone and then tocilizumab, which led to remission. With the recurrence of joint pain and rash in March 2020, accompanied by low oxygen saturation levels (90%), and ground-glass appearance on chest CT, PCR test revealed COVID-19 infection. Ciclesonide was started on day 7 of the disease onset, which led to improved inflammatory markers by day 21. Thus, we infer that while tocilizumab is theoretically useful for COVID-19 due to its inhibition of interleukin 6 (IL-6), additional ciclesonide therapy might be required to prevent worsening of the condition. AOSD and COVID-19 must, therefore, be differentiated by levels of ferritin which differ between the two, and appropriate treatment must be allocated.

scholarly journals Effect of Ultrasound Therapy on the Adult-Onset Still’s Disease: A Case Study

2020 ◽  
Vol 10 (1) ◽  
pp. 49-54
Author(s):  
Manal Yooussef ◽  
Keyword(s):  
Short Form ◽  
Unknown Origin ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Timed Up And Go ◽  
Still's Disease ◽  
Muscle Testing ◽  
Systemic Inflammatory Disease ◽  
Adult Onset Still's Disease

Adult Onset Still’s Disease (AOSD) is a rare systemic inflammatory disease of unrecognized etiology and pathogenesis that presents in 5% to 10% of patients as Fever of Unknown Origin (FUO) accompanied by systemic manifestations. We reported a compelling case of a 39-year-old African male who presented with a one-month duration FUO along with skin rash, sore throat, and arthralgia. After an extensive workup, potential differential diagnoses were ruled out, and the patient was diagnosed with AOSD based on the Yamaguchi criteria. After the application of Ultrasound (US) and exercises on both ankles as well as left knee, left elbow, and left wrist, there was an improvement of Visual Analogue Scale by 66.6%, Five Repetition Sit to Stand Test (FRSTS) improved 50%, Timed Up and Go (TUG) test improved 87.5%, and there was also an improvement in the 36-Item Short Form Survey (SF-36), and muscle testing. This result was due to the anti-inflammatory effect of the US, leading to decrease pain and improve function in the individuals.

scholarly journals Adult Onset Still's Disease and Rocky Mountain Spotted Fever

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Paul Persad ◽  
Rajendrakumar Patel ◽  
Niki Patel
Keyword(s):  
Spotted Fever ◽  
Rocky Mountain ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Infectious Agents ◽  
Adult Onset ◽  
Still’S Disease ◽  
Rocky Mountain Spotted Fever ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult Still's Disease was first described in 1971 by Bywaters in fourteen adult female patients who presented with symptoms indistinguishable from that of classic childhood Still's Disease (Bywaters, 1971). George Still in 1896 first recognized this triad of quotidian (daily) fevers, evanescent rash, and arthritis in children with what later became known as juvenile inflammatory arthritis (Still, 1990). Adult Onset Still's Disease (AOSD) is an inflammatory condition of unknown etiology characterized by an evanescent rash, quotidian fevers, and arthralgias. Numerous infectious agents have been associated with its presentation. This case is to our knowledge the first presentation of AOSD in the setting of Rocky Mountain Spotted Fever. Although numerous infectious agents have been suggested, the etiology of this disorder remains elusive. Nevertheless, infection may in fact play a role in triggering the onset of symptoms in those with this disorder. Our case presentation is, to our knowledge, the first case of Adult Onset Still's Disease associated with Rocky Mountain spotted fever (RMSF).

scholarly journals Adult onset Still’s disease presenting as fever of unknown origin: a case report with review of literature

2017 ◽  
Vol 11 ◽  
Author(s):  
Harpreet Singh ◽  
Deepak Jain ◽  
Saroj Dhankhar ◽  
Rekha Mathur
Keyword(s):  
Joint Pain ◽  
Fever Of Unknown Origin ◽  
Unknown Origin ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Rheumatologic Diseases ◽  
Systemic Inflammatory Disease ◽  
Adult Onset Still's Disease

Adult onset Still’s disease (AOSD) is a systemic inflammatory disease with exact etiology and pathogenesis yet to be discovered. AOSD, being an important cause of fever of unknown origin, is diagnosed after ruling out infections, malignancy and other rheumatologic diseases. It may present with fever without typical rash although typical triad is of fever, joint pain and rash. A 35-year old previously healthy man was referred to our hospital with 6 months of fever, joint pain and weight loss. Examination and investigations revealed anaemia, leukocytosis (predominant neutrophilia), lymphadenopathy, hepatomegaly, arthritis and evidence of interstitial lung disease with raised serum ferritin levels. The hematological disorders, infections and other rheumatologic diseases were excluded. The diagnosis of adult onset Still’s disease can be very difficult as there are no specific tests and diagnosis is based on symptom complex. AOSD presenting as fever of unknown origin could be a challenge for the physician to diagnose and manage timely.

scholarly journals Adult Onset Still’s Disease: A Case Report with a Rare Clinical Manifestation and Pathophysiological Correlations

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Katerina M. Antoniou ◽  
George A. Margaritopoulos ◽  
Ioannis Giannarakis ◽  
Christianna Choulaki ◽  
Nikos Fountoulakis ◽  
...  
Keyword(s):  
Pleural Effusion ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Inflammasome Activation ◽  
Adult Onset ◽  
Still’S Disease ◽  
Pleural Effusions ◽  
Still's Disease ◽  
Multisystemic Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease is an inflammatory multisystemic disease of unknown etiology. Pleuritis is the most common pulmonary manifestation and pleural effusions are usually exudates with a predominance of neutrophils. We report a case of an eosinophilic pleural effusion as a novel and hitherto unrecognized manifestation of active adult-onset Still’s disease. We also observed a marked NLRP3 inflammasome activation with increased production of IL-1βwhich coincided with the development and resolved upon remission of the pleural effusion suggesting a possible novel pathogenetic pathway for the development of pleural effusions in the context of the auto-inflammatory disorders.

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