Adult Still's Disease

1983 ◽  
Vol 28 (3) ◽  
pp. 255-258 ◽  
Author(s):  
J. G. Larkin ◽  
R. D. Sturrock
Keyword(s):  
Unknown Origin ◽  
Bed Rest ◽  
Sufficient Evidence ◽  
Still’S Disease ◽  
Pyrexia Of Unknown Origin ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
Long Term Follow Up ◽  
Nosological Entity ◽  
Toxic Drugs

Adult Still's disease is a recently recognised syndrome which is an important and often unrecognised cause of pyrexia of unknown origin. A knowledge of the clinical features of this disease may save patients with undiagnosed fever from prolonged and invasive investigations. The condition usually responds to conservative therapy with non-steroidal anti-inflammatory agents and bed rest, although in a minority of patients more toxic drugs may be required. The prognosis is usually good although long-term follow-up studies of these patients are small in number, and the most recent review sounds a warning as to the incidence of chronicity. Sufficient evidence now exists to identify Still's disease as a separate nosological entity in adults. It should be added to the ever-expanding list of causes of pyrexia of unknown origin.

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.

scholarly journals Non-purulent pharyngitis: adult still’s disease

2020 ◽  
Vol 8 (9) ◽  
pp. 3362
Author(s):  
Nagabhushanarao B. V. ◽  
Narmada Vatti ◽  
Naveen Kumar Dannana ◽  
Surendra Konathala
Keyword(s):  
Serum Ferritin ◽  
Severe Disease ◽  
Elevated Serum ◽  
Unknown Origin ◽  
High Dose ◽  
Still’S Disease ◽  
Delay In Diagnosis ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
Cell Counts

 Adult Still’s disease is rare and may present as pyrexia of unknown origin. Due to lack of expertise, diagnosis may be delayed inadvertently. The patient usually presents with spiked fever, polyarthralgia or arthritis, evanescent skin rash, non-purulent pharyngitis, lymphadenopathy and hepatosplenomegaly. Leukocytosis, predominantly of neutrophils, elevated erythrocyte sedimentation rate and C-reactive protein without obvious infection are the hallmarks of the disease. Delay in diagnosis may expose the patient to the side effects of antibiotics as they are repeatedly prescribed in view of elevated leukocytes. The majority of patients report pain in the throat without evidence of infection. This was an important clue to our diagnosis of this patient. Grossly elevated serum ferritin is diagnostic of adult onset still's disease. As the white cell counts are grossly elevated, a bone marrow examination to rule out hematological malignancy may be mandatory. Serum ferritin value has prognostic value too. Minor illness may respond to non-steroidal anti-inflammatory drugs (NSAIDs), but steroids are the mainstay of the treatment.  Methotrexate is of additional value for those presenting predominantly with arthritis. Anakinra, Infliximab and Tocilizumab are other options. Those patients presenting with severe disease and organ involvement require high dose intravenous steroids followed by high dose oral steroids.

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 AB0660 COVID-19 SHARES CLINICAL FEATURES WITH ANTI-MELANOMA DIFFERENTIATION ASSOCIATED PROTEIN 5 POSITIVE DERMATOMYOSITIS AND ADULT STILL’S DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1362.1-1362
Author(s):  
Y. Kondo ◽  
Y. Kaneko ◽  
H. Takei ◽  
H. Tamai ◽  
T. Takeuchi
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features ◽  
Serum Ferritin ◽  
Rheumatic Diseases ◽  
Macrophage Activation ◽  
Cytokine Storm ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Still's Disease

Background:The coronavirus disease 2019 (COVID-19), caused by a novel corona virus named SARS-CoV-2, has emerged as a global pandemic. Severe inflammatory process is one of main pathogenesis of COVID-19 and this involves cytokine storm along with overactivation of macrophage. On another front, cytokine storm with macrophage activation is frequently observed in various connective tissue diseases including dermatomyositis with positive antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies and adult Still’s disease. Macrophage activation during inflammatory states is partially characterized by an increased serum ferritin levels and hyperferritinaemia and characteristics shared by the three diseases are a topic of interest to rheumatologists, however, no study has evaluated anti-MDA5-positive dermatomyositis and adult Still’s disease in comparison to COVID-19.Objectives:The aim of this study was to highlight the homology and heterogeneity of COVID-19, anti-MDA5 dermatomyositis, and adult Still’s disease by comparing clinical pictures of each disease in order to discuss their respective pathogeneses.Methods:We reviewed consecutive, newly diagnosed, untreated patients with COVID-19, anti-MDA5 dermatomyositis, or adult Still’s disease. We compared their clinical, laboratory, and radiological characteristics, including the prevalence of macrophage activation syndrome and lung involvement in each disease.Results:The numbers of patients with COVID-19, anti-MDA5 dermatomyositis, and adult-onset Still’s disease with hyperferritinaemia (serum ferritin ≥ 500ng/dL) who were included for main analysis were 22, 14, and 59, respectively. COVID-19 and adult Still’s disease both featured hyperinflammatory status, such as high fever and elevated serum C-reactive protein, whereas COVID-19 and anti-MDA5 dermatomyositis both presented with severe interstitial lung disease and hypoxaemia. While two-thirds of the patients in each group met the criteria for macrophage-activated syndrome that is used in systemic juvenile idiopathic arthritis, the HScore, an indicator of haemophagocytic lymphohistiocytosis, was low in anti-MDA5 dermatomyositis and COVID-19 even in severe or critical cases. The findings of chest computed tomography were similar between COVID-19 and anti-MDA5 dermatomyositis (Figure 1).Conclusion:COVID-19 shared clinical features with rheumatic diseases characterised by hyperferritinaemia, including anti-MDA5 dermatomyositis and adult Still’s disease. These findings should be investigated further in order to shed light on the pathogenesis of not only COVID-19 but also the aforementioned rheumatic diseases.References:[1]Mehta P, McAuley DF, Brown M, et al. COVID-19: consider cytokine storm syndromes and immunosuppression. Lancet. (2020) 395: 1033-4.[2]Gono T, Sato S, Kawaguchi Y, et al. Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford). 2012; 51(9):1563-70.Figure 1.Imaging characteristics of chest CT scans in patients with COVID-19, anti-MDA5 dermatomyositis, and adult Still’s disease A)Bilateral ground-glass and consolidative opacities with peripheral distribution in COVID-19. B)Bilateral ground-glass opacities with peripheral consolidations in anti-MDA5 dermatomyositis. C)Pleural effusion with pleural thickening on the left side in adult Still’s disease.Disclosure of Interests:Yasushi Kondo: None declared., Yuko Kaneko: None declared., Hisoshi Takei: None declared., Hiroya Tamai: None declared., Tsutomu Takeuchi Grant/research support from: received research grants outside the submitted work from Abbvie, Astra Zeneca, Bristol Myers Squibb, Chugai Pharmaceutical, Eisai Pharmaceutical, Janssen Pharmaceutical, Mitsubishi Tanabe Pharma Corporation, Novartis, Takeda Pharmaceutical, Abbott Japan Co., Ltd., Astellas Pharma, Ltd., Daiichi Sankyo, Pfizer, Sanofi–Aventis, Santen Pharmaceutical, Teijin Pharma Ltd., Asahikasei Pharma Corp., SymBio Pharmaceuticals Ltd., Celtrion, Nipponkayaku Co. Ltd., Eli Lilly Japan, and Taisho Toyama Pharmaceutical.

scholarly journals FRI0505 TOCILIZUMAB DISCONTINUATION AFTER REMISSION ACHIEVEMENT IN PATIENTS WITH ADULT STILL’S DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 851.2-851
Author(s):  
H. Tamai ◽  
Y. Kaneko ◽  
T. Takeuchi
Keyword(s):  
Laboratory Data ◽  
Good Control ◽  
Still’S Disease ◽  
Median Dose ◽  
Chugai Pharmaceutical ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
The Mean ◽  
Recurrence Group ◽  
Observation Period

Background:The efficacy of tocilizumab, an interleukin (IL)-6 receptor inhibitor, has been proved in patients with adult Still’s disease on suppressing systemic inflammation and decreasing glucocorticoid dose. However, whether tocilizumab can be discontinued after remission achievement is unclear.Objectives:To clarify the possibility of tocilizumab discontinuation in patients with adult Still’s disease who achieved remission with tocilizumab.Methods:Consecutive patients with adult Still’s disease diagnosed according to the Yamaguchi’s criteria in our hospital from April 2012 until September 2019 were retrospectively reviewed. Patients who were in good control with tocilizumab were included in the analysis, and their clinical courses were collected from their medical charts. Patients were divided according to the presence of recurrence after tocilizumab discontinuation and compared.Results:Among 42 patients with adult Still’s disease who had a history of intravenous tocilizumab of 8mg/kg use, 13 patients discontinued tocilizumab following a good disease control. During the mean observation period of 26.4 months, six patients (46%) remained in remission while seven patients (54%) developed recurrence after tocilizumab discontinuation. The sex and the mean observation period were not different between the patients with recurrence and those without (71% vs 50%, p=0.43; 27.3 months vs 25.4 months, p=0.93, respectively), but the age at tocilizumab discontinuation tended to be higher in the recurrence group than the non-recurrence group (64.0 years vs 46.5 years, p=0.08). The disease activity including swollen joint counts and laboratory data at tocilizumab discontinuation were comparable between the two groups (serum ferritin levels, 88 ng/mL vs 122 ng/mL, p=0.67). While the duration of tocilizumab use was not different between the two groups (29.4 months vs 39.5 months, p=0.40), the mean interval of tocilizumab infusion at tocilizumab discontinuation in the recurrence group was 3.6 weeks, shorter than the 6.7 weeks in the non-recurrence group (p=0.03). The median dose of prednisolone at tocilizumab discontinuation was 5.0 mg/day in the recurrence group and 0.0 mg/day in the non-recurrence group (p=0.06). In the recurrence group, the duration from the last tocilizumab administration to recurrence was 7.8 months, and the median dose of prednisolone at recurrence was 5.0 mg/day.Conclusion:Patients with adult Still’s disease remaining in remission with a longer interval of tocilizumab administration and a lower dose of prednisolone was likely to succeed in withdrawal of tocilizumab.Disclosure of Interests:Hiroya Tamai: None declared, Yuko Kaneko Speakers bureau: Dr. Kaneko reports personal fees from AbbVie, personal fees from Astellas, personal fees from Ayumi, personal fees from Bristol-Myers Squibb, personal fees from Chugai, personal fees from Eisai, personal fees from Eli Lilly, personal fees from Hisamitsu, personal fees from Jansen, personal fees from Kissei, personal fees from Pfizer, personal fees from Sanofi, personal fees from Takeda, personal fees from Tanabe-Mitsubishi, personal fees from UCB, Tsutomu Takeuchi Grant/research support from: Eisai Co., Ltd, Astellas Pharma Inc., AbbVie GK, Asahi Kasei Pharma Corporation, Nippon Kayaku Co., Ltd, Takeda Pharmaceutical Company Ltd, UCB Pharma, Shionogi & Co., Ltd., Mitsubishi-Tanabe Pharma Corp., Daiichi Sankyo Co., Ltd., Chugai Pharmaceutical Co. Ltd., Consultant of: Chugai Pharmaceutical Co Ltd, Astellas Pharma Inc., Eli Lilly Japan KK, Speakers bureau: AbbVie GK, Eisai Co., Ltd, Mitsubishi-Tanabe Pharma Corporation, Chugai Pharmaceutical Co Ltd, Bristol-Myers Squibb Company, AYUMI Pharmaceutical Corp., Eisai Co., Ltd, Daiichi Sankyo Co., Ltd., Gilead Sciences, Inc., Novartis Pharma K.K., Pfizer Japan Inc., Sanofi K.K., Dainippon Sumitomo Co., Ltd.

Adult Still's Disease presenting as PUO; a Not Uncommon Disorder

Rheumatology ◽  
1987 ◽  
Vol 26 (1) ◽  
pp. 65-66 ◽  
Author(s):  
N. COHEN ◽  
J. WEISSGARTEN ◽  
A. GOLIK ◽  
A. PIK ◽  
E. YONA ◽  
...  
Keyword(s):  
Still’S Disease ◽  
Still's Disease ◽  
Adult Still's Disease
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