scholarly journals Elevated serum levels of free interleukin-18 in adult-onset Still’s disease

Rheumatology ◽  
2016 ◽  
Vol 55 (12) ◽  
pp. 2237-2247 ◽  
Author(s):  
Charlotte Girard ◽  
Jürgen Rech ◽  
Michael Brown ◽  
Danièle Allali ◽  
Pascale Roux-Lombard ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Serum Levels ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Interleukin 18 ◽  
Still's Disease ◽  
Adult Onset Still's Disease

scholarly journals Case Report: Intracranial hypertension in an adult-onset Still’s disease patient initially presented with prolonged fever

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 1050
Author(s):  
Gerasimos Eleftheriotis ◽  
Elias Skopelitis
Keyword(s):  
Intracranial Hypertension ◽  
Elevated Serum ◽  
Disease Patient ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Prolonged Fever ◽  
Still's Disease ◽  
Serum Igg4 ◽  
Adult Onset Still's Disease

This article describes the case of a 19-year-old woman who presented with prolonged fever, positive antinuclear antibodies (ANA) and splenomegaly. Pulmonary infiltrates were discovered and the patient was treated for community-acquired pneumonia, with no clinical amelioration. A more thorough evaluation was subsequently made, revealing elevated serum IgE and IgG4 levels and negative ANA tested by the hospital’s laboratory with two methods. During hospitalization thrombocytopenia, liver function test impairment, and evanescent rash during some febrile episodes developed. Vomiting also presented without any concomitant symptoms or signs; a funduscopic examination was consequently ordered, showing bilateral papilledema. Brain imaging was totally normal but a lumbar puncture revealed elevated opening pressure and lymphocytic pleocytosis along with low cerebrospinal fluid lactate dehydrogenase (CSF LDH). The patient was empirically treated with antimicrobials, dexamethasone, and acetazolamide and had immediate clinical and laboratory improvement. Diagnostic workup, however, was negative for an infectious agent; antimicrobials were ceased but the patient continued to improve. Adult-onset Still’s disease (AOSD) was considered as the working diagnosis because the patient fulfilled Yamaguchi criteria, responded to corticosteroids, and an alternative diagnosis was lacking. Nevertheless, because of the patient’s atypical features a trial to discontinue dexamethasone was undertaken, leading to immediate recurrence; the possibility of a self-limiting viral illness was excluded. Thrombocytopenia was attributed to hemophagocytic lymphohistiocytosis (HLH) that complicated AOSD. Corticosteroid reinitiation combined with methotrexate fully controlled all clinical and laboratory parameters. One month later papilledema had disappeared and the patient remained symptom-free even without acetazolamide. To our knowledge, this is the first report in the literature of an AOSD case presenting intracranial hypertension without cerebral imaging abnormalities and neurological or meningeal symptoms and signs, as well as with the initial observation of serum IgG4 elevation. A classic regimen combined with acetazolamide led to a positive outcome.

scholarly journals The Role of Interleukin 18/Interleukin 18-Binding Protein in Adult-Onset Still’s Disease and Systemic Juvenile Idiopathic Arthritis

2022 ◽  
Vol 11 (2) ◽  
pp. 430
Author(s):  
Charlotte Girard-Guyonvarc’h ◽  
Mathilde Harel ◽  
Cem Gabay
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Binding Protein ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Free Form ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Interleukin 18 ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Interleukin 18 (IL-18) is a pro-inflammatory cytokine of the IL-1 family, whose activity is tightly controlled at the level of production, as well as signalization. Notably, it is buffered by its natural inhibitor, IL-18 binding protein (IL-18BP), which is massively present in circulation in normal and in most pathological conditions, thus preventing harmful pro-inflammatory systemic effects of IL-18. IL-18 has long been considered to be involved in the pathophysiology of various inflammatory diseases. However, a first clinical trial using recombinant IL-18BP for the treatment of rheumatoid arthritis and psoriasis gave disappointing results. Direct measurements of unbound, bioactive, free form of circulating IL-18 demonstrated that IL-18 was more specifically involved in adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA) but also in their most severe complication, macrophage activation syndrome (MAS). More importantly, administration of recombinant IL-18BP to patients with AOSD, and sJIA with MAS, showed promising results. This review summarizes available data regarding IL-18 and IL-18BP in AOSD and sJIA in mouse models and humans and shows the importance of IL-18/IL-18BP imbalance in these conditions, leading to the conclusion that IL-18, particularly free IL-18, may be a useful biomarker in these diseases and an interesting therapeutic target.

scholarly journals Association between adult-onset Still's disease and interleukin-18 gene polymorphisms

2002 ◽  
Vol 3 (7) ◽  
pp. 394-399 ◽  
Author(s):  
T Sugiura ◽  
Y Kawaguchi ◽  
M Harigai ◽  
H Terajima-Ichida ◽  
Y Kitamura ◽  
...  
Keyword(s):  
Gene Polymorphisms ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Interleukin 18 ◽  
Still's Disease ◽  
Adult Onset Still's Disease

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.

Serum S100A8/A9, But Not Follistatin-like Protein 1 and Interleukin 18, May Be a Useful Biomarker of Disease Activity in Adult-onset Still’s Disease

2012 ◽  
Vol 39 (7) ◽  
pp. 1399-1406 ◽  
Author(s):  
HYOUN-AH KIM ◽  
JEONG-MI AN ◽  
JIN-YOUNG NAM ◽  
JA-YOUNG JEON ◽  
CHANG-HEE SUH
Keyword(s):  
Disease Activity ◽  
Systemic Disease ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Disease Score ◽  
Still’S Disease ◽  
Interleukin 18 ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Objective.S100A8/A9, follistatin-like protein 1, and interleukin 18 (IL-18) have been suggested as biomarkers of disease activity in patients with systemic juvenile idiopathic arthritis or adult-onset Still’s disease (AOSD). We investigated the clinical significance of these factors in AOSD.Methods.Blood samples were collected from 36 patients with AOSD, 40 patients with rheumatoid arthritis (RA), and 33 healthy controls. Of the patients with AOSD, followup samples were collected from 16 patients after resolution of disease activity.Results.Serum levels of S100A8/A9 (11.77 ± 8.84 μg/ml) in AOSD patients were higher than those in RA patients (3.53 ± 3.43 μg/ml; p < 0.001) and controls (2.49 ± 1.83 μg/ml; p < 0.001). Follistatin-like protein 1 levels in AOSD were not different from those in RA and controls. IL-18 levels in AOSD (7560.3 ± 7577.6 pg/ml) were higher than those in RA (217.7 ± 292.1 pg/ml; p < 0.001) and controls (139.2 ± 86.2 pg/ml; p < 0.001). The sensitivity and specificity of IL-18 for diagnosing AOSD was highest with a cutoff value of 366.1 pg/ml. Serum S100A8/A9 correlated with leukocyte count, erythrocyte sedimentation rate, C-reactive protein, ferritin, and systemic disease score; however, IL-18 correlated only with ferritin and systemic disease score. S100A8/A9 was decreased after disease activity was resolved in followup of AOSD patients (9.96 ± 7.35 μg/ml in active AOSD vs 3.6 ± 4.77 μg/ml in resolved cases; p = 0.001). The change of S100A8/A9 was well correlated with that of systemic disease score.Conclusion.The data suggest that serum S100A8/A9 may be a useful biomarker for evaluating disease activity in patients with AOSD.

scholarly journals Elevated Troponin Serum Levels in Adult Onset Still’s Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Carlo Umberto Manzini ◽  
Lucio Brugioni ◽  
Michele Colaci ◽  
Maurizio Tognetti ◽  
Amelia Spinella ◽  
...  
Keyword(s):  
Systemic Disease ◽  
Clinical History ◽  
Serum Levels ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Recent Onset ◽  
Still's Disease ◽  
Cardiac Assessment ◽  
Adult Onset Still's Disease

Adult onset Still’s disease (AOSD) is a rare inflammatory systemic disease that occasionally may affect myocardium. Diagnosis is based on typical AOSD symptoms after the exclusion of well-known infectious, neoplastic, or autoimmune/autoinflammatory disorders. In the case of abrupt, recent onset AOSD, it could be particularly difficult to make the differential diagnosis and in particular to early detect the possible heart involvement. This latter event is suggested by the clinical history of the four patients described here, incidentally observed at our emergency room. All cases were referred because of acute illness (high fever, malaise, polyarthralgias, skin rash, and sore throat), successively classified as AOSD, and they presented abnormally high levels of serum troponin without overt symptoms of cardiac involvement. The timely treatment with steroids (3 cases) or ibuprofen (1 case) leads to the remission of clinicoserological manifestations within few weeks. These observations suggest that early myocardial injury might be underestimated or entirely overlooked in patients with AOSD; routine cardiac assessment including troponin evaluation should be mandatory in all patients with suspected AOSD.

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