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.

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 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 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 Hemophagocytic syndrome, a rare variant of Still’s disease

2017 ◽  
Vol 5 (10) ◽  
pp. 4649
Author(s):  
Neha Bhave ◽  
Dinesh Gupta ◽  
Prashant Kashyap
Keyword(s):  
Musculoskeletal Diseases ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Diagnostic Challenge ◽  
Serological Tests ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult onset still’s disease is a rare systemic inflammatory disorder of unknown etiology that is responsible for a significant number of cases of fever of unknown origin (FUO) and musculoskeletal diseases. The diagnosis in adult onset still’s disease is mainly clinical and requires exclusion of other infections. Laboratory tests are nonspecific and treatment mainly comprises of corticosteroids, NSAIDS, immunosuppressive drugs, iv gamma globulin, anti-tumour necrosis factor, anti-interleukin. AOSD (adult onset stills disease) is a diagnostic challenge. Discovery of new serological tests and a specific diagnostic criterion may help the clinician in faster diagnosis and better management of the disease. 

scholarly journals Adult Onset Still’s Disease: A Diagnostic Dilemma

2021 ◽  
Vol 23 (1) ◽  
pp. 83-86
Author(s):  
Dinesh Koirala ◽  
UN Pathak ◽  
P Adhikari ◽  
R Shrestha ◽  
C Neupane
Keyword(s):  
Case Report ◽  
Liver Enzymes ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Rare Clinical Entity ◽  
Adult Onset ◽  
Still’S Disease ◽  
Diagnostic Dilemma ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (ASD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, evanescent rash and other systemic presentations. This case report describes a 19-year-old male who presented with sore throat, fever, arthritis, evanescent rash, raised liver enzymes and hyperferritinemia. It also reveals the diagnostic dilemma faced during the diagnosis of the disease. He was diagnosed to have ASD based on Yamuguchi criteria after the exclusion of other potential diagnoses. Patient was treated with prednisolone and there was a good response with improvement in symptoms and laboratory indices.

scholarly journals A rare clinical presentation: Adult-onset Still’s disease

2020 ◽  
Vol 9 (1) ◽  
pp. 56-59
Author(s):  
Prakash Banjade ◽  
Prakash Poudel Jaishi ◽  
Jonas Malla
Keyword(s):  
Clinical Manifestations ◽  
Differential Diagnoses ◽  
Treatment Modalities ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult -onset Still’s disease (AOSD) is a rare, idiopathic, inflammatory disorder of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO )exclusion characterized by generalized migratory joint ache, blanchable rash, fever and other systemic manifestations. We report an interesting case of a 23 year old Nepalese lady from Okhaldunga who presented with one-month duration of FUO along with sore throat, fever, vomiting, generalized joint ache, erythematous blanchable rash, headache and visual impairment. On examination there was hepatomegaly and investigations showed raised liver enzymes, serum ferritin and fibrinogen.After extensive workup, potential differential diagnoses were ruled out She was diagnosed to have Adult -onset Still’s disease based on Yamuguchi criteria after exclusion of other potential differentials.The patient partially responded to prednisolone and later methotrexate was prescribed which improved her symptoms. 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 Adult onset Still’s disease: a diagnostic challenge

2018 ◽  
Vol 4 (1) ◽  
pp. 87
Author(s):  
Rahul Jain ◽  
V. K. Joglekar ◽  
Kriti Jain
Keyword(s):  
Disease Diagnosis ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Diagnostic Challenge ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
Adult Onset Still's Disease

<p class="abstract"><span lang="EN-IN">Adult Still’s disease (ASD) is a systemic inflammatory disorder of unknown etiology, typically characterized by a clinical triad of daily spiking high fevers, evanescent rash, and arthritis. This report described a 26-year-old male who presented with these symptoms along with raised liver enzymes and hyperferritinemia. After ruling out systemic infections, localized infections, malignancies and other rheumatological diseases, Adult onset Still’s disease diagnosis was made according to Yamaguchi criteria (having 4 major features and 3 minor features). AOSD is a heterogeneous and rare disease and the lack of serologic markers as a true gold standard makes diagnosis difficult.</span></p>

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.

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