Non-purulent pharyngitis: adult still’s disease

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.

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1031 ◽

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.

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

Italian Journal of Medicine ◽

10.4081/itjm.2010.259 ◽

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.

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Elevated serum caeruloplasmin level in a patient with adult Still's disease

British Journal of Dermatology ◽

10.1046/j.1365-2133.2002.04517.x ◽

2002 ◽

Vol 146 (3) ◽

pp. 508-510 ◽

Cited By ~ 1

Author(s):

L. Kemeny ◽

M. Mehravaran ◽

A. Dobozy

Keyword(s):

Elevated Serum ◽

Still’S Disease ◽

Still's Disease ◽

Adult Still's Disease

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Adult Still's Disease

Scottish Medical Journal ◽

10.1177/003693308302800308 ◽

1983 ◽

Vol 28 (3) ◽

pp. 255-258 ◽

Cited By ~ 3

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.

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Elevated serum levels of checkpoint molecules in patients with adult Still’s disease

10.21203/rs.3.rs-26391/v2 ◽

2020 ◽

Author(s):

Yuya Fujita ◽

Tomoyuki Asano ◽

Haruki Matsumoto ◽

Naoki Matsuoka ◽

Jumpei Temmoku ◽

...

Keyword(s):

Disease Activity ◽

Immunosuppressive Treatment ◽

Elevated Serum ◽

Serum Levels ◽

Chronic Arthritis ◽

Enzyme Linked Immunosorbent Assay ◽

Still’S Disease ◽

Serum Samples ◽

Still's Disease ◽

Adult Still's Disease

Abstract Background: The interaction between galectin-9 (Gal-9) and its ligand, T cell immunoglobulin and mucin-containing-molecule-3 (TIM-3), one of the coinhibitory receptors, transduce the inhibitory signaling to regulate immune responses. The dysregulated expression of checkpoint molecules has been reported under various inflammatory or autoimmune conditions. The aim of this study is to investigate the levels of these checkpoint molecules and their associations between proinflammatory markers in patients with adult Still’s disease (ASD). Methods: Serum samples were collected from 47 patients with active ASD, 116 patients with rheumatoid arthritis (RA), and 37 healthy controls (HCs). Serum levels of Gal-9, soluble TIM-3 (sTIM-3), and IL-18 were determined using enzyme-linked immunosorbent assay (ELISA). Results were compared with clinical features of ASD. Results: Serum Gal-9 levels in patients with ASD (median: 21.57 ng/ml, interquartile range IQR [11.41–39.72]) were significantly higher compared to those in patients with RA (7.58 ng/ml, IQR [5.57–10.20] p < 0.001) as well as those in HCs (4.51 ng/ml, [IQR; 3.58–5.45], p < 0.001). Similarly, serum sTIM-3 levels in patients with ASD were significantly higher than those in patients with RA and HCs. Serum levels of Gal-9 or sTIM-3 showed positive correlations with IL-18 levels (Gal-9; r = 0.90, p< 0.001, sTIM-3; r = 0.78, p< 0.001) in patients with ASD. Serum levels of Gal-9 or sTIM-3 correlated with serum ferritin (Gal-9; r = 0.77, p< 0.001, sTIM-3; r = 0.71, p< 0.001) and ASD disease activity score (Pouchot’s score, Gal-9; r = 0.66, p< 0.001, sTIM-3; r = 0.59, p< 0.001). Whereas there was no significant correlation between serum Gal-9 or sTIM-3 and CRP. ASD patients with chronic arthritis phenotype had a significantly higher Gal-9/ferritin and sTIM-3/ferritin ratio than those without this phenotype. After immunosuppressive treatment, Gal-9 and sTIM-3 levels showed a significant decline in parallel to the disease activity scores. Conclusions: Serum levels of the coinhibitory checkpoint molecules were elevated and correlated with disease activity in patients with ASD. These coinhibitory checkpoint molecule may be implicated in the autoinflammatory process seen in ASD.

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