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

2021 ◽  
Author(s):  
Yuko Kaneko

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.

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Rajesh Gopalarathinam ◽  
Eric Orlowsky ◽  
Ramesh Kesavalu ◽  
Sreeteja Yelaminchili

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.


2021 ◽  
Vol 22 (23) ◽  
pp. 13038
Author(s):  
Ji-Won Kim ◽  
Mi-Hyun Ahn ◽  
Ju-Yang Jung ◽  
Chang-Hee Suh ◽  
Hyoun-Ah Kim

Neutrophils are innate immune phagocytes that play a key role in immune defense against invading pathogens. The main offensive mechanisms of neutrophils are the phagocytosis of pathogens, release of granules, and production of cytokines. The formation of neutrophil extracellular traps (NETs) has been described as a novel defense mechanism in the literature. NETs are a network of fibers assembled from chromatin deoxyribonucleic acid, histones, and neutrophil granule proteins that have the ability to kill pathogens, while they can also cause toxic effects in hosts. Activated neutrophils with NET formation stimulate autoimmune responses related to a wide range of inflammatory autoimmune diseases by exposing autoantigens in susceptible individuals. The association between increased NET formation and autoimmunity was first reported in antineutrophil cytoplasmic antibody-related vasculitis, and the role of NETs in various diseases, including systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, has since been elucidated in research. Herein, we discuss the mechanistic role of neutrophils, including NETs, in the pathogenesis of systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still’s disease (AOSD), and provide their clinical values as biomarkers for monitoring and prognosis.


2020 ◽  
Vol 10 (1) ◽  
pp. 49-54
Author(s):  
Manal Yooussef ◽  

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.


2017 ◽  
Vol 11 ◽  
Author(s):  
Harpreet Singh ◽  
Deepak Jain ◽  
Saroj Dhankhar ◽  
Rekha Mathur

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.


2015 ◽  
Vol 42 (3) ◽  
pp. 277-280 ◽  
Author(s):  
Pedro Carrilho-Ferreira ◽  
Doroteia Silva ◽  
Maria de Jesus Silva ◽  
Rui André ◽  
Manuel Gato Varela ◽  
...  

Adult-onset Still's disease is a rare disorder with potentially severe clinical features, including cardiac involvement. This systemic inflammatory disease of unknown origin should be considered in the differential diagnosis of pericarditis, with or without pericardial effusion. Cardiac tamponade is a very rare sequela that requires an invasive approach, such as percutaneous or surgical pericardial drainage, in addition to the usual conservative therapy. The authors describe a case of adult-onset Still's disease rendered more difficult by pericarditis and cardiac tamponade, and they briefly review the literature on this entity.


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