ADULT ONSET STILL’S DISEASE PRESENTING AS PYREXIA OF UNKNOWN ORIGIN

2021 ◽  
pp. 43-44
Author(s):  
Naim M Kadri ◽  
Yash S Patel ◽  
Pratvi V Vaishnav ◽  
Smit R Patel
Keyword(s):  
Young Male ◽  
Unknown Origin ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Pyrexia Of Unknown Origin ◽  
Still's Disease ◽  
Sustained Effect ◽  
Yamaguchi Criteria ◽  
Adult Onset Still's Disease

Adult onset Still’s disease (AOSD) is a rare inammatory disease characterized by fever, arthralgia, rash, sore throat, lymphadenopathy, hepatomegaly and/or splenomegaly. It is a rare cause of pyrexia of unknown origin (PUO) and should be considered once the more common infectious, rheumatological and malignant causes have been excluded. This is usually a diagnosis of exclusion. Yamaguchi criteria can be used for diagnosis. Patients of AOSD show an excellent response to Non-steroidal anti-inammatory drugs (NSAIDs), however some may not have a sustained effect, in which case glucocorticoids have to be considered. Here’s a case of a young male patient who was evaluated for the cause of pyrexia of unknown origin.

Aetiology of pyrexia of unknown origin in north India

2020 ◽  
pp. 004947552094790
Author(s):  
Ashok Kumar Pannu ◽  
Rithvik Golla ◽  
Savita Kumari ◽  
Vikas Suri ◽  
Pankaj Gupta ◽  
...  
Keyword(s):  
Extrapulmonary Tuberculosis ◽  
Age Groups ◽  
Unknown Origin ◽  
North India ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Pyrexia Of Unknown Origin ◽  
Still's Disease ◽  
Adult Onset Still's Disease

The aetiology of pyrexia of unknown origin (PUO) varies dramatically according to epidemiology. We studied the cause and spectrum of PUO in Indian adults. A total of 152 patients (112 prospectively and 40 retrospectively) met Petersdorf and Beeson's criteria. The diagnostic evaluation was guided by potentially diagnostic clues, based on a ‘step-wise’ approach. The five main categories, i.e. infectious, neoplastic, non-infectious inflammatory, miscellaneous and undiagnosed comprised 43.4%, 21.5%, 19.7%, 2.0% and 12.5%, respectively. The top three causes were tuberculosis (n = 43, 28.3%), lymphoma (n = 19, 12.5%) and adult-onset Still's disease (n = 12, 7.9%). Tuberculosis predominated in all age groups, and about 70% of cases had the extrapulmonary form, the most common being gastrointestinal. Hodgkin and non-Hodgkin lymphomas were equally distributed, but solid malignancies were uncommon. Adult-onset Still's disease was the second commonest cause in adults aged ≤ 40 years. Fever resolved spontaneously in 12/19 cases of undiagnosed cause. Extrapulmonary tuberculosis remains the most prevalent PUO in India.

scholarly journals Adult-onset Still’s disease, the most internistic of the rheumatic diseases: current concepts and a report of six cases

2013 ◽  
pp. 259-264
Author(s):  
Roberto Boni ◽  
Pier G. Rabitti
Keyword(s):  
Serum Ferritin ◽  
Hemophagocytic Syndrome ◽  
Unknown Origin ◽  
Essential Elements ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown origin. It is characterized by spiking fever, evanescent rash, arthralgia/arthritis, and leukocytosis. The differential diagnosis includes a number of other conditions, and management is complicated by the lack of course predictors and the risks associated with complications and treatments. This report examines recent advances in our understanding of adult-onset Still’s disease (pathogenesis, diagnosis, complications, treatment). Current research in this field is focused on the significance of serum ferritin in AOSD, mechanisms underlying the hemophagocytic syndrome, and use of biologic therapies in patients who are refractory to conventional treatment. Six cases of AOSD diagnosed by our staff between 2002 and 2009 are also analyzed and compared with other cases reported in the literature. This analysis showed that Still’s rash and serum ferritin levels were not essential elements for diagnosis. In addition, the course of the disease showed little relation to the severity / characteristics of the presenting picture, but the evolution worsened with the age of the patient at diagnosis.

Interleukin 18: A Biomarker for Differential Diagnosis Between Adult-onset Still’s Disease and Sepsis

2014 ◽  
Vol 41 (6) ◽  
pp. 1118-1123 ◽  
Author(s):  
Roberta Priori ◽  
Serena Colafrancesco ◽  
Cristiano Alessandri ◽  
Antonina Minniti ◽  
Carlo Perricone ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Disease Activity ◽  
Serum Levels ◽  
Inactive Disease ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Yamaguchi Criteria ◽  
Adult Onset Still's Disease

Objective.The differential diagnosis between rheumatic diseases and infectious conditions is a great challenge in clinical practice. Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory syndrome that shares several clinical and laboratory variables with sepsis. Interleukin (IL)-18 is overexpressed in AOSD, suggesting a possible role as a disease biomarker. The aim of our study was to detect IL-18 serum levels in a cohort of patients with AOSD and sepsis and to address its possible role as a biomarker for differential diagnosis.Methods.A group of unselected patients with AOSD diagnosed according to the Yamaguchi criteria and consecutive patients with sepsis diagnosed according to the American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference criteria were enrolled. The clinical and laboratory data were collected. In the AOSD group, disease activity was assessed by Pouchot’s and Rau’s criteria. IL-18 serum levels were detected by ELISA.Results.Thirty-nine patients with AOSD and 18 patients with sepsis were enrolled. Two out of 18 patients with sepsis (11.1%) also fulfilled the Yamaguchi criteria. A significant difference was found in IL-18 serum levels between patients with active and inactive disease (p < 0.001), and it positively correlated with disease activity (p = 0.0003), ferritin serum level (p = 0.016), and erythrocyte sedimentation rate (p = 0.041). IL-18 was significantly increased in patients with AOSD when compared with sepsis (p = 0.014). For a cutoff of 148.9 pg/ml, this test had a specificity of 78.3% and a sensitivity of 88.6%.Conclusion.We have demonstrated that IL-18 can be a biomarker for differential diagnosis between AOSD and sepsis.

scholarly journals Adult-onset Still’s disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review

2019 ◽  
Vol 40 (1) ◽  
pp. 145-152 ◽  
Author(s):  
Ken Goda ◽  
Tsuneaki Kenzaka ◽  
Masahiko Hoshijima ◽  
Akihiro Yachie ◽  
Hozuka Akita
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Yamaguchi Criteria ◽  
Adult Onset Still's Disease ◽  
Cytokine Profiling ◽  
Activation Syndrome

Abstract Adult-onset Still’s disease (AOSD) is a relatively rare systemic inflammatory disorder and is diagnosed using various sets of classification criteria, with the Yamaguchi criteria as the most widely used criteria. Herein, we present the case of a 21-year-old woman admitted with a high fever, lasting for over 1 month, who did not fulfill the Yamaguchi criteria. However, by analyzing the inflammatory cytokine profile, we defined this case as AOSD based on a greatly elevated serum interleukin-18 level. In addition, we predicted the occurrence of macrophage activation syndrome by a characteristic increase in the soluble tumor necrosis factor receptor II level, which allowed a timely intervention for this malicious complication. Therefore, we suggest that cytokine profiling will be useful for the diagnosis and management of AOSD.

scholarly journals AB1016 RATE OF PREDEFINING DISEASE ACTIVITY, REMISSION AND RELAPSE OF ADULT-ONSET STILL’S DISEASE: SYSTEMATIC LITERATURE REVIEW

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1801.2-1801
Author(s):  
E. Bilgin ◽  
U. Kalyoncu
Keyword(s):  
Disease Activity ◽  
Observational Studies ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Genetic Studies ◽  
Still's Disease ◽  
Definition Of ◽  
Yamaguchi Criteria ◽  
Adult Onset Still's Disease

Background:Adult-onset Still’s disease (AOSD) is a rare, multisystem and auto-inflammatory disorder. Although several scoring systems are proposed to describe AOSD disease activity, there is no agreement on the definition of disease activity, remission or relapse of AOSD.Objectives:Aim of this literature review was to determine the rates of defining ‘’disease activity’’, ‘’remission’’ and ‘’relapse’’ of AOSD.Methods:We performed a systematic review (May 2019) on Pubmed by using the MeSh word ‘’ Adult-onset Still’s disease’’; results were restricted to human studies and English language. We excluded case reports, reviews and studies including less than 10 patients; 215 articles were recruited for final assessment. Of this 215 article, we reached the full-texts of the 181 articles. Final assessment was done with 181 articles. In these articles, we searched whether the definitions of disease activity, remission or relaps were made or not.Results:Total of 181 articles were included to analysis. Mean age was 37.2±6.6 and 73.6% of patients was female. Articles were devided to 4 main groups according to main focus related to AOSD: Observational studies, laboratory studies, genetic studies and clinical trials. Diagnosis of AOSD was according to Yamaguchi criteria in 159 (87.8%) of articles and Yamaguchi criteria was the most commonly used criteria set. Disease activity was defined in 96 (54.1%) of articles. Remission and relapse were defined in 52 (28.7%) and 18 (10%) of articles, respectively. Most commonly used definition of disease activity was the ‘’systemic score’’ which was defined by Pouchet et al. Rates of defining ‘’disease activity’’, ‘’remission’’ and ‘’relapse’’ according to 4 main groups were given in Table 1.Table 1.Rates of disease activity, remission and relapse reporting based on 4 main article groupsArticle groupn (%)Disease activity (n,%)pRemission (n,%)pRelapse (n,%)pObservational Studies70 (38.7)18 (25.7)<0.00117 (24.3)<0.0018 (11.4)<0.001Laboratory Studies72 (39.8)46 (63.9)15 (20.8)2 (2.8)Genetic Studies17 (9.4)5 (29.4)2 (11.8)0 (0)ClinicalTrial22 (12.2)14 (63.6)18 (81.8)8 (36.4)Conclusion:In recent years, new treatment options become available for AOSD. However, data about how to measure the AOSD activity in largely missing. Definitions of disease activity, remission or relapse are missing in the most of the studies, including clinical trials, in current literature. Future studies are needed for this issue.References:NoneDisclosure of Interests:Emre Bilgin: None declared, Umut Kalyoncu Consultant of: Abbvie, Amgen, Janssen, Lilly, Novartis, UCB

Adult-onset Still’s disease in a 73-year-old Maltese man

2020 ◽  
Vol 13 (8) ◽  
pp. e234752
Author(s):  
Janice Borg ◽  
Mary Louise Camilleri ◽  
Paul John Cassar
Keyword(s):  
Disease Onset ◽  
Virus Detection ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Barr Virus ◽  
Still's Disease ◽  
Epstein Barr ◽  
Yamaguchi Criteria ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory condition diagnosed by Yamaguchi criteria. We report an atypical presentation of a 73-year-old man, who was admitted with fever, sore throat and pleurisy that were unresponsive to three courses of antibiotics. Fever persisted in a quotidian pattern and the typical salmon-coloured rash consistent with AOSD appeared at 4 weeks from symptoms onset. These features in addition to neutrophilia and hyperferritinaemia in the absence of concurrent infectious and neoplastic causes satisfied Yamaguchi criteria for a diagnosis of AOSD. Epstein-Barr antigen was initially detected at moderate titre levels, but was undetectable after 1 week. Complete resolution of symptoms was reported on initiation of steroid treatment. A relationship between disease onset and viral syndromes has been documented to occur and few similar cases preceded by Epstein-Barr virus detection have been reported.

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