scholarly journals Catastrophic adult-onset Still’s disease as a distinct life-threatening clinical subset: case–control study with dimension reduction analysis

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Anaïs Wahbi ◽  
Benoît Tessoulin ◽  
Cédric Bretonnière ◽  
Julien Boileau ◽  
Dorothée Carpentier ◽  
...  
Keyword(s):  
Dimension Reduction ◽  
Organ Failure ◽  
Disease Duration ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Younger Age ◽  
Life Threatening ◽  
Adult Onset Still's Disease

Abstract Objectives Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder. Diagnosing AOSD can be challenging, as disease presentation and clinical course are highly heterogeneous. For unclear reasons, a few patients develop life-threatening complications. Our objective was to determine whether these cases resulted from therapeutic delay or could represent a peculiar AOSD subset. Methods We conducted a multicentre retrospective study of 20 AOSD patients with organ failure requiring intensive care unit admission and 41 control AOSD patients without organ failure. Clinico-biological data at hospital admission were explored using supervised analyses and unsupervised dimension reduction analysis (factor analysis of mixed data, FAMD). Results Disease duration before admission was shorter in patients with life-threatening AOSD (median, 10 vs 20 days, p = 0.007). Disease duration before AOSD therapy initiation also tended to be shorter (median, 24 vs 32 days, p = 0.068). Despite this shorter disease duration, FAMD, hierarchical clustering and univariate analyses showed that these patients exhibited distinctive characteristics at first presentation, including younger age; higher frequency of splenomegaly, liver, cardiac and/or lung involvement; less frequent arthralgia; and higher ferritin level. In multivariate analysis, 3 parameters predicted life-threatening complications: lack of arthralgia, younger age and shorter time between fever onset and hospitalisation. Conclusion This study suggests that life-threatening complications of AOSD occur very early, in a peculiar subset, which we propose to name catastrophic adult-onset Still’s disease (CAOSD). Its exact burden may be underestimated and remains to be clarified through large multicentre cohorts. Further studies are needed to identify red flags and define the optimal therapeutic strategy.

Multi-organ failure in adult onset Still’s disease: a septic disguise

2008 ◽  
Vol 28 (S1) ◽  
pp. 3-6 ◽  
Author(s):  
Paul R. J. Ames ◽  
Emily Walker ◽  
Darren Aw ◽  
David Marshall ◽  
Francois de Villiers ◽  
...  
Keyword(s):  
Organ Failure ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Multi Organ Failure ◽  
Adult Onset Still's Disease

Adult onset still's disease: experience in 23 patients and literature review with emphasis on organ failure

1987 ◽  
Vol 17 (1) ◽  
pp. 39-57 ◽  
Author(s):  
Antonio J. Reginato ◽  
H. Ralph Schumacher ◽  
Daniel G. Baker ◽  
Carolyn R. O'Connor ◽  
Jose Ferreiros
Keyword(s):  
Literature Review ◽  
Organ Failure ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Disease Experience ◽  
Adult Onset Still's Disease

scholarly journals A Diagnosis of Adult-Onset Still’s Disease after Multiple Urgent Care Visits

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Kami M. Hu ◽  
Adam C. Richardson ◽  
Kelly M. Blosser ◽  
Semhar Z. Tewelde
Keyword(s):  
Fold Increase ◽  
Community Acquired Pneumonia ◽  
Urgent Care ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Autoinflammatory Disorder ◽  
Still's Disease ◽  
Life Threatening ◽  
Adult Onset Still's Disease

A 34-year-old man with recent treatment and resolution of community-acquired pneumonia presents to the emergency department with protracted fever, rash, and sore throat. Sustained fever and greater than two-fold increase in leukocytosis despite appropriate antibiotic therapy prompted hospital admission for infectious disease and rheumatologic evaluations which ultimately revealed adult-onset Still’s disease, a rare autoinflammatory disorder with potentially life-threatening complications.

scholarly journals POS1344 EVALUATING THE MULTIVISCERAL INVOLVEMENT ON ADULT-ONSET STILL’S DISEASE TO RETRIEVE IMAGING-BASED DIFFERENCES IN PATIENTS WITH AND WITHOUT MACROPHAGE ACTIVATION SYNDROME; RESULTS FROM A SINGLE-CENTRE OBSERVATIONAL STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 954.2-955
Author(s):  
I. DI Cola ◽  
F. Bruno ◽  
O. Berardicurti ◽  
R. Monti ◽  
A. Conforti ◽  
...  
Keyword(s):  
Ct Scan ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Life Threatening ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

Background:Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder usually affecting young adults, burdened by life-threatening complications, mainly macrophage activation syndrome (MAS), a secondary form of hemophagocytic lymphohistiocytosis [1]. In this context, the importance of an accurate assessment of AOSD is suggested to promptly recognise the multivisceral involvement of the disease which is associated with life-threatening complications. The assessment of the most aggressive subsets of the disease could guide the clinicians when to apply additional resources but avoiding unnecessary expenditures in patients with a less severe clinical picture.Objectives:In this study, we aimed at describing the multivisceral involvement of the disease to retrieve imaging-based differences in AOSD patients with and without MAS.Methods:The present evaluation has been designed as a cross-sectional study to descriptively compare the multivisceral involvement in AOSD patients with and without MAS. Patients admitted to our Institution, who underwent a total body CT scan, were selected from our historical cohort and assessed. Clinical and CT scan characteristics of AOSD patients with and without MAS were compared. Clinical and CT scan characteristics of AOSD patients with and without MAS were analysed by parametric or non-parametric t tests for all continuous variables, and chi squared test was used for categorical ones, as appropriate. Furthermore, possible correlations among radiological outcomes with laboratory markers and systemic score were estimated by using a point-biserial coefficient correlation.Results:This study evaluated 39 AOSD patients (men 64.1%), mean age of 48.8±16.6 years). Out of those, 14 patients (35.9%) were complicated by MAS. These patients showed higher values of ferritin [AOSD: 770.0 (1306.5) ng/mL vs MAS: 2926.3 (4918.5) ng/mL p=0.003] and systemic score (AOSD: 4.6±1.4 vs MAS: 6.9±1.7, p<0.0001). AOSD patients with MAS presented a higher prevalence of lung disease than others (AOSD: 56.0% vs MAS 85.7% p=0.048). Lung disease correlated with the systemic score (coefficient 0.491, p=0.003). AOSD patients with MAS were more frequently characterised by hepatomegaly (AOSD: 12.0% vs MAS: 50.0% p=0.019) and splenomegaly (AOSD: 16.0% vs MAS 50.0% p=0.033), respectively, than others. Hepatomegaly correlated with CRP (coefficient 0.421, p=0.016), ferritin (coefficient 0.397, p=0.020), and systemic score (coefficient 0.391, p=0.022). Furthermore, the presence of splenomegaly correlated with the systemic score (coefficient 0.439, p=0.009). CT scan features of abdominal effusions were more frequently observed in AOSD patients with MAS than those without this complication (AOSD: 12.0% vs 57.1% p=0.007). Finally, a higher percentage of AOSD patients with MAS showed a significant lymph node enlargement, either mediastinal or abdominal, than others on CT scan (AOSD: 36.0% vs MAS 71.4% p=0.048). The presence of lymphadenomegaly correlated with the systemic score (coefficient 0.368, p=0.032).Conclusion:Our findings showed a higher prevalence of multiorgan involvement in AOSD patients with MAS, suggesting imaging-based differences, although other studies are needed to fully assess this issue. Pulmonary disease, hepatomegaly, splenomegaly, lymph nodes enlargement, and abdominal effusions were associated with these more aggressive patients.References:[1]Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun. 2018 Sep;93:24-36.Disclosure of Interests:None declared

scholarly journals SAT0544 USE OF BIOLOGICAL DMARDS IN PATIENTS WITH ADULT-ONSET STILL’S DISEASE: RESULTS FROM TURKBIO REGISTRY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1229.1-1229
Author(s):  
A. Yazici ◽  
E. Dalkiliç ◽  
M. Birlik ◽  
M. A. Öztürk ◽  
S. Akar ◽  
...  
Keyword(s):  
Disease Duration ◽  
Biological Agents ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Switching Rate ◽  
Drug Survival ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Background:Adult-onset Still’s disease (AOSD) is a rare multisystemic inflammatory disorder, and is diagnosed by exclusion. AOSD is generally treated with nonsteroidal antiinflammatory drugs, corticosteroids, and conventional disease modifiying antirheumatic drugs (cDMARDs). Biological disease modifiying antirheumatic drug (bDMARD) therapy are recommended in AOSD patients who are refractory to tradional therapy, and bDMARDs is becoming increasingly important in AOSD treatment.Objectives:To evaluate the use of bDMARDs and drug survival in AOSD patients.Methods:TURKBIO registry is the Turkish version of Danish DANBIO rheumatological database which has been established in 2011. All patients with AOSD who received biological agents registered in TURKBIO registry between dates of October 2011 and October 2019 were included in this study. The demographic data, response of therapy, frequency of using and switching biological agents were collected.Results:As of October, 21 AOSD patients were recruited. Mean age of patients was 34.6±7.3 (min-max: 24-49) years, mean disease duration was 9.3±7.4 (min-max: 1-22) years, and 57.1% of patients was female. Mean duration from onset to start of bDMARDs was 7±6.1 (min-max: 0.5-21) years. It was observed that 13 patients (61.9%) received tocilizumab (TCZ), 6 patients (28.6%) received IL-1 inhibitors (5 anakinra and one canakinumab), 2 patients (9.5%) received certolizumab and one patient (4.8%) received etanercept as a first-line bDMARDs. The most frequently used biological agents in current treatment were as follows: 52.4% of patients received TCZ and 33.3% received IL-1 inhibitors (4 anakinra, 3 canakinumab), and the most frequently used concomitant drugs were methotrexate (47.6%) and hydroxychloroquine (14.3%). The switching rate was 33.3%, and in half of them the reason of switching was adverse events. The median drug survival for bDMARDs was 28.6 months (Table).Table.Demographic, laboratory features and management of AOSD(median;25-75)n=21Age (year)34.7 (28.3-40.6)Gender (Female) n(%)12 (57.1)Disease duration (year)8 (2-17)Duration from onset to start of bDMARs (year)6 (1.5-10)ESR (on onset)44 (21-66)CRP (on onset)65 (3.1-108)Current bDMARDs n(%) Tocilizumab11(52.4) IL-1 inhibitors7 (33.3) Etanercept1 (4.8) Certolizumab2 (9.5)Concomitant cDMARD n(%) Methotrexate10 (47.6) Leflunomide4 (19) Sulfasalazine1 (4.8) Hydroxychloroquine3 (14.3)bDMARDs Survival (months)28.6 (5.5-75)Switching Rate n(%)7 (33.3)Adverse Event n(%)3 (14.3)Conclusion:This is the first evaluation of AOSD patients who used biological agents from TURKBIO registry. According our data, TCZ and anti-IL1 agents were the most frequent biological choices. The limitation of this study was the low number of the patients with AOSD who used biological agents.References:[1].Zhou S, Qiao J, Bai J, Wu Y, Fang H. Biological therapy of traditional therapy-resistant adult-onset Still’s disease: an evidence-based review. Ther Clin Risk Manag 2018;14:167-71.Acknowledgments:NoneDisclosure of Interests:None declared

scholarly journals AB1278 A CASE REPORT OF TOCILIZUMAB INDUCE RAPID REMISSION OF ADULT-ONSET STILL’S DISEASE WITH LIFE-THREATENING INTERSTITIAL LUNG DISEASE AND MACROPHAGE ACTIVATION SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1930.1-1930
Author(s):  
G. Zhang ◽  
W. Liu ◽  
T. Xu ◽  
X. Zhang
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Life Threatening ◽  
Adult Onset Still's Disease

Background:Adult-onset still’s disease (AOSD) is a systemic inflammatory condition characterized by fevers, polyarthritis, skin rashes and increased leukocyte and neutrophil counts [1]. Sometimes life-threatening complications such as macrophage activation syndrome (MAS), disseminated intravascular coagulopathy (DIC) can be occurred without response to glucocorticoid and disease-modifying antirheumatic drugs (DMARDs).Objectives:To describe an AOSD patient with life-threatening interstitial lung disease (ILD) and MAS response to tocilizumab.Methods:This is a case report at our medical practice.Results:A 43-year-old man was hospitalized for a 2-week history of fever accompanying skin rashes, polyarthritis, sore throat, leukocytosis (the maximum leukocyte count 37.25×109/L with 89.9% neutrophils), elevated levels of C-reactive protein (190.9mg/L) and ferritin level was very high (>15000ng/ml), Several antibiotics were consequently used, but fever was on going. Chest CT displayed diffuse interstitial lung disease. Infectious diseases and lymphoma were excluded and AOSD with ILD was diagnosed. However, MAS occurred the 20 days later after the pulse of intravenous MP (MP 500mg×3days) following MP 40 mg/day, which cannot stop the situation deteriorating. The minimum total white blood cells, hemoglobin and platelets went down to 0.25×109/l, 53g/l and 18×109/l. Finally, tocilizumab rescued this patient from desperation.Conclusion:Tocilizumab was effective in AOSD with life-threatening ILD and MAS.References:[1]E. Feist, S. Mitrovic, and B. Fautrel, Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nat Rev Rheumatol 14 (2018) 603-618.Disclosure of Interests:None declared

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