scholarly journals Juvenile Dermatomyositis Magnetic Resonance Imaging Score (JIS) does not correlate with criteria for clinically inactive disease: a single-centre retrospective evaluation

2021 ◽  
Author(s):  
Kapil Gargh ◽  
Eslam Al-Abadi ◽  
Samantha Low ◽  
Kathryn Harrison ◽  
William Coles ◽  
...  
Keyword(s):  
Disease Activity ◽  
Juvenile Dermatomyositis ◽  
Treatment Decision ◽  
Clinical Decision ◽  
Inactive Disease ◽  
Mri Findings ◽  
Clinical Criteria ◽  
Physician Decision Making ◽  
Core Set

AbstractThe Paediatric Rheumatology International Trials Organisation (PRINTO) criteria for clinically inactive disease (CID) and their proposal for glucocorticoid tapering do not consider MRI findings, despite the growing use of MRI and development of reliable MRI scoring tools. We aim to evaluate how CID correlates with MRI scores and physician decision making. We retrospectively used the Juvenile Dermatomyositis Imaging Score (JIS) to score MRIs of all children with JDM over a 10-year period. Demographic, diagnosis, treatment and core set measures data were collected. Correlation between CID and JIS was assessed as well as correlation with the physician treatment decision. There were 25 patients with 59 follow-up episodes to analyse correlation between physician treatment decision and JIS; and 50 episodes for the CID category and JIS correlation. JIS was not significantly associated with the CID category but did correlate with the physician decision. No significant association was found between clinical decision and CID category. The JIS area under the ROC curve (AUC) was 0.80 (95% CI 0.62–0.99) with a score ≥ 8 to predict an escalation. JIS sensitivity and specificity were both 78% with accuracy of 78%, compared to only 67%, 46% and 49%, respectively, for the CID criteria. Clinical criteria alone are not sufficient to assess disease activity status. Clinical decision trends correlated to MRI findings but not PRINTO CID criteria. Multi centre prospective studies are needed to replicate our findings and establish how to best use MRI as a biomarker of disease activity.

The Patient Activity Scale-II Is a Generic Indicator of Active Disease in Patients with Rheumatic Disorders

2010 ◽  
Vol 37 (9) ◽  
pp. 1932-1934 ◽  
Author(s):  
KETNA PAREKH ◽  
WILLIAM J. TAYLOR
Keyword(s):  
Disease Activity ◽  
Diagnostic Category ◽  
Treatment Decision ◽  
Disease State ◽  
Self Report ◽  
Inactive Disease ◽  
Treatment Intensity ◽  
Activity Scale ◽  
The Relationship ◽  
Active Disease

Objective.To determine whether the Patient Activity Scale-II (PAS-II) is a generic measure of disease activity by assessing whether the relationship of PAS-II with treatment decision (indicating disease activity) is invariant across disease.Methods.The Health Assessment Questionnaire-II (HAQ-II), a 10 cm visual analog scale for “pain,” and another for “patient global assessment” were recorded from 1000 consecutive patients attending rheumatology outpatient clinics. Active disease was defined as treatment intensity increased and inactive disease was defined as treatment intensity unchanged or decreased. A logistic regression analysis was conducted with active disease as the dependent variable and the predictor variables were PAS-II, diagnostic category, and the interaction between diagnostic category and PAS-II.Results.PAS-II had a weak but statistically significant association with active disease that was independent of diagnosis. An increase of 1 point in PAS-II increased the odds of being in the active disease state by 1.19 (95% CI 1.10 to 1.37). The relationship between active disease state and PAS was not affected by diagnostic category.Conclusion.PAS-II can be used as a generic self-report indicator of active disease across different rheumatic disorders, and not just in rheumatoid arthritis. The strength of the relationship with disease activity is weak and physician-derived indicators remain very important.

Challenges of achieving clinical remission in a national cohort of juvenile-onset systemic lupus erythematosus patients

Lupus ◽  
2019 ◽  
Vol 28 (5) ◽  
pp. 667-674 ◽  
Author(s):  
N Abdelrahman ◽  
M W Beresford ◽  
V Leone ◽  
Keyword(s):  
Disease Activity ◽  
Lupus Erythematosus ◽  
Clinical Remission ◽  
Immunosuppressive Treatment ◽  
Inactive Disease ◽  
Juvenile Onset ◽  
Time To Diagnosis ◽  
National Cohort ◽  
Onset Systemic Lupus Erythematosus

Background and objectives The multisystem involvement and variable course of juvenile-onset systemic lupus erythematosus (JSLE) make it difficult to assess disease activity over time. International consensus definitions of inactive disease and clinical remission have been proposed. The aim of this study was to determine the proportion of patients meeting these criteria in a large national cohort of JSLE patients and the association between achieving inactive disease and clinical remission with disease activity at presentation and time to diagnosis. Methods Patients diagnosed with JSLE aged ≤17 years with a minimum of 12 months follow-up participating in the UK JSLE Cohort Study were assessed against these criteria at baseline, 1 year and final clinic visit. Results A total of 218 patients with mean follow-up duration of 4.7 years were included and analyzed at baseline visit, of which 93 and 209 were available for analysis at the 1-year and the last follow-up visits, respectively. Eighty-five percent at 1 year and 62% at final follow-up still had active disease while only 6% and 9%, respectively, achieved inactive disease according to the proposed criteria. The majority of patients continued to require immunosuppressive treatment despite their prolonged follow-up with only two patients achieving clinical remission on medication and none off medication. A large number of patients did not meet the criteria for inactive disease due to isolated laboratory abnormalities such as reduced lymphocyte count. Isolated low lymphocyte count was the reason for not fulfilling the inactive disease criteria in 20/79 (25%) patients at 1 year and 14/130 (11%) patients at final follow-up visit. No statistically significant differences in relation to time to diagnosis and disease activity at presentation were found between patients achieving inactive disease compared to those who did not, at 1 year and final follow-up. Conclusion The majority of patients failed to achieve the proposed criteria for inactive disease and continued to require immunosuppressive treatment. This reflects the high burden of disease in JSLE despite immunosuppressive therapy. A significant proportion of patients had isolated laboratory abnormalities of potentially limited clinical significance, suggesting that some modifications of the proposed criteria may be required.

scholarly journals Cell-Free Osteochondral Scaffold for the Treatment of Focal Articular Cartilage Defects in Early Knee OA: 5 Years’ Follow-Up Results

2019 ◽  
Vol 8 (11) ◽  
pp. 1978 ◽  
Author(s):  
Andrea Sessa ◽  
Luca Andriolo ◽  
Alessandro Di Martino ◽  
Iacopo Romandini ◽  
Roberto De Filippis ◽  
...  
Keyword(s):  
Articular Cartilage ◽  
International Knee Documentation Committee ◽  
Clinical Results ◽  
Activity Level ◽  
Cartilage Defects ◽  
Mri Findings ◽  
Clinical Criteria ◽  
Osteochondral Scaffold ◽  
Articular Cartilage Defects

The purpose of this study was to investigate the clinical results at five years’ follow-up of a tri-layered nanostructured biomimetic osteochondral scaffold used for focal articular cartilage defects in patients meeting the criteria of early osteoarthritis (EOA). The study population comprised 22 patients (mean age: 39 years), prospectively assessed before surgery, at 24 and 60 months’ follow-up. Inclusion criteria were: at least two episodes of knee pain for more than 10 days in the last year, Kellgren-Lawrence OA grade 0, I or II and arthroscopic or MRI findings according to the European Society of Sports Traumatology, Knee Surgery & Arthroscopy (ESSKA) criteria. Clinical results demonstrated significant improvement in International Knee Documentation Committee (IKDC) subjective and objective scores and in Tegner score, although activity level never reached the pre-injury level. The complication rate of this study was 8.3%. Two patients underwent re-operation (8.3%), while a comprehensive definition of failure (including both surgical and clinical criteria) identified four failed patients (16.6%) at this mid-term follow-up evaluation. The use of a free-cell osteochondral scaffold represented a safe and valid alternative for the treatment of focal articular cartilage defects in the setting of an EOA, and was able to permit a significant clinical improvement and stable outcome with low complication and failure rates.

Outcome assessment in the idiopathic inflammatory myopathies

2018 ◽  
Author(s):  
Lisa G. Rider ◽  
Frederick W. Miller
Keyword(s):  
Disease Activity ◽  
Endothelial Activation ◽  
Inactive Disease ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Muscle Enzyme ◽  
Activation Markers ◽  
Core Set ◽  
Trial Endpoints ◽  
Muscle Ultrasound

Due to their rarity, heterogeneity, and multispecialty nature, the myositis syndromes have limited data-driven consensus on appropriate outcome measures. Recently, two international, multispecialty consortia developed new tools and consensus on core set measures of myositis disease activity and damage, as well as response criteria that are now recommended for use as clinical trial endpoints but will also be useful in clinical practice. Magnetic resonance imaging, muscle ultrasound, selected laboratory tests, and immunological biomarkers—including cytokines, chemokines, lymphocyte flow cytometry, and endothelial activation markers—can all be helpful adjuncts to serum muscle enzyme levels in assessing disease activity and damage, but these have not yet been fully validated. Definitions of clinically inactive disease, complete clinical response, and remission have also been proposed but require further validation. These advances should enhance the development of therapies by standardizing our ability to demonstrate their efficacy in treating the idiopathic inflammatory myopathies.

scholarly journals P549 A virtual clinic enhances the quality use of anti-TNF dose intensification and rates of treatment success using a treat-to-target approach compared with standard outpatient care in Crohn’s disease

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S467-S467
Author(s):  
A Srinivasan ◽  
D R van Langenberg ◽  
R Little ◽  
M P Sparrow ◽  
P De Cruz ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Disease Activity ◽  
Outpatient Care ◽  
Inactive Disease ◽  
Treat To Target ◽  
Model Of Care ◽  
Dose Intensification ◽  
Trough Levels

Abstract Background Virtual clinics (VC) represent a novel model-of-care which promote quality use of biologics and facilitate a treat-to-target (T2T) approach. We aimed to evaluate the clinical and process-driven outcomes of intensified anti-TNF therapy for secondary loss of response (SLoR) in a VC compared with standard outpatient care (SoC). Methods A retrospective multicenter, parallel observational cohort study of patients with Crohn’s disease (CD) who underwent anti-TNF dose intensification to address SLoR with up to 2-years of follow-up was undertaken. Objective assessments of disease activity and anti-TNF trough levels at SLoR then during subsequent 6-month semesters were compared longitudinally between VC and SoC groups. ‘Anti-TNF escalation success’ was the primary endpoint, defined as achieved when two consecutive 6-month semesters of objective assessment demonstrated inactive disease. Dose intensification was defined as ‘appropriate’ if there was both objective evidence of disease activity, plus anti-TNF trough levels at or below the lower limit of the therapeutic range prior to intensification. ‘Treatment escalation failure’ was defined by the occurrence of one or more of biologic switching, discontinuation or further intensification; recommencement of corticosteroids, or IBD-related surgery. Results 149 patients (69 VC vs. 80 SoC) underwent infliximab (n = 94) or adalimumab (n = 55) dose intensification with similar baseline patient, disease and treatment characteristics between cohorts. The median duration of follow-up post dose-intensification was 1.9 and 1.5 years for the SoC and VC cohorts respectively (p = 0.37). There were higher rates of appropriate dose intensification (82.6 vs. 40.0%, p < 0.01) across the VC cohort. Higher proportions of anti-TNF escalation success (60.9 v 35.0%, p < 0.01), biomarker remission (i.e., C-Reactive Protein (<5mg/l) and faecal calprotectin (<150 μg/ml) normalisation, log-rank tests, p = 0.06 and p < 0.01 respectively), tight disease monitoring (84.1 vs. 28.8%, p < 0.01) and de-escalation of intensified therapy (21.3 vs. 10.0%, p = 0.03) were also achieved by the VC cohort following dose-intensification. Conclusion This study favoured a VC-led model of care over standard outpatient-based IBD care in facilitating an effective T2T approach to CD management. A VC model of care improved quality use of intensified anti-TNF therapy through processes that promoted appropriate dose intensification and encouraged more frequent anti-TNF dose de-escalation. Moreover, tight semester-based monitoring using a T2T-based strategy facilitated by the VC was associated with improved treatment outcomes.

CLO19-026: Determining the Treatment Decision of Tumors With Mixed Response

2019 ◽  
Vol 17 (3.5) ◽  
pp. CLO19-026
Author(s):  
Candice Baldeo ◽  
Tasneem Kaleem ◽  
Ricardo Paz-Fumagalli ◽  
John Copland ◽  
Michael Menefee
Keyword(s):  
Tumor Response ◽  
Treatment Decision ◽  
Clinical Decision ◽  
Hematologic Malignancies ◽  
Current Treatment ◽  
Response To Treatment ◽  
Mixed Response ◽  
Line Of Therapy ◽  
Additional Agent

Introduction: Individuals receiving systemic anticancer therapies for advanced solid tumors routinely undergo imaging studies to assess the efficacy of the treatment. Mixed response (MR) to cancer therapy is a common but poorly described phenomenon. There is a paucity of data regarding both the incidence and possible mechanisms of this clinical quandary. Potential etiologies include tumor heterogeneity, differences in tumor microenvironment, and discrepancies in drug delivery to different tumor deposits. It is also possible that MR simply reflects differences in the rate of resistance emerging. MR represents a therapeutic dilemma for the clinician. Methods: Mixed tumor response was defined as: One tumor decreasing in size; one tumor increasing in size (classified as RECIST response/progressions), One tumor stable; another tumor progressing, One tumor stable; another tumor responding, New tumor; another tumor responding or remaining stable. Between 2015 and 2017, 120 restaging CT scans were reviewed of patients who had received at least 1 line of therapy for advanced cancer diagnosis which showed MR; hematologic malignancies were excluded. Charts were reviewed to determine the clinical decision that was made at the time of the MR. Results: A total of 120 scans with MR were reviewed from various solid tumor diagnoses. 38 scans were excluded due to loss of follow-up or death. Of the remaining 82 scans, therapy was switched in 30, the same therapy was continued in 50, and an additional agent was added to the current treatment in 2 cases (Table). Of the patients in which treatment was switched, 20% (6/30) showed response to treatment on the following scan. Of the cases that were kept on current treatment, none showed response on the following restaging scan which was done 6–8 weeks later. There were 4 (10%) deaths prior to the next scan in the group that had treatment switched and similarly 5 deaths (10%) prior to the next scan in the group in which treatment remained the same. Conclusion: MR is associated with a poor prognosis, irrespective of treatment decisions. These data are retrospective and our sample size is small, so definitive conclusions cannot be drawn. However, changing therapy when a MR is observed may be of benefit to some patients. A prospective evaluation to more accurately describe and understand the MR phenomenon is warranted.

scholarly journals Multi-modal single-cell sequencing identifies cellular immunophenotypes associated with juvenile dermatomyositis disease activity

2021 ◽  
Author(s):  
Jessica Neely ◽  
George Hartoularos ◽  
Daniel Bunis ◽  
Yang Sun ◽  
David Lee ◽  
...  
Keyword(s):  
Disease Activity ◽  
Single Cell ◽  
Juvenile Dermatomyositis ◽  
Cell Types ◽  
Gene Signature ◽  
Inactive Disease ◽  
Cell Type ◽  
Gene Score ◽  
Single Cell Sequencing ◽  
Inflammatory Monocytes

Juvenile dermatomyositis (JDM) is a rare autoimmune condition with insufficient biomarkers and treatments, in part, due to incomplete knowledge of the cell types mediating disease. We investigated immunophenotypes and cell-specific genes associated with disease activity using multiplexed RNA and protein single-cell sequencing applied to PBMCs from 4 treatment-naive JDM (TN-JDM) subjects at baseline, 2, 4, and 6 months and 4 subjects with inactive disease. Analysis of 55,564 cells revealed separate clustering of TN-JDM cells within monocyte, NK, CD8+ effector T and naive B populations. The proportion of CD16+ monocytes was reduced in TN-JDM, and naive B cells were expanded. Cell-type differential gene expression analysis and hierarchical clustering identified a pan-cell-type IFN gene signature over-expressed in TN-JDM in all cell types and correlated with disease activity. TN-JDM monocytes displayed an inflammatory state: CD16+ monocytes expressed the highest IFN gene score and differential protein expression of adhesion molecules, CD49d and CD56, compared to CD14+ inflammatory monocytes. A transitional B cell population expressing higher CD24 and CD5 proteins and an IFN-hi naive B population were associated with TN-JDM and exhibited less CD39, an immunoregulatory protein. This data provides new insights into JDM immune dysregulation at cellular resolution and novel resource for myositis investigators.

scholarly journals Serum Calprotectin (S100A8/A9): A Promising Biomarker in Diagnosis and Follow-up in Different Subgroups of Juvenile Idiopathic Arthritis

2021 ◽  
Author(s):  
Céline La ◽  
Phu Quoc Lê ◽  
Alina Ferster ◽  
Laurence Goffin ◽  
Delphine Spruyt ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Added Value ◽  
Response To Treatment ◽  
Inactive Disease ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Controls ◽  
Minimal Disease ◽  
Active Disease

Abstract IntroductionIn the management of juvenile idiopathic arthritis (JIA), there is a lack of diagnostic and prognostic biomarkers. This study assesses the use of serum calprotectin (sCal) as a marker to monitor disease activity, and as a classification and prognosis tool of response to treatment or risk of flares in patients with JIA. MethodsEighty-one patients with JIA from the CAP48 multicentric cohort were included in this study, as well as 11 non-pediatric healthy controls. An enzyme-linked immunosorbent assay (ELISA) method was used to quantify sCal with a commercial kit.ResultsPatients with an active disease compared to healthy controls and to patients with inactive disease showed an 8-fold and a 2-fold increased level of sCal respectively. sCal was found to be correlated with the CRP and even more strongly with the ESR. Evolution of DAS28 scores correlated well with evolution of sCal, as opposed to evolution of CRP. With regard to CRP, sCal could differentiate forms with active oligoarthritis from polyarthritis and systemic forms. However, sCal brought an added value compared to the CRP as a prognosis marker. Indeed, patients with active disease and reaching minimal disease activity (according to JADAS) at 6 months following the test had higher sCal levels, while patients with inactive disease had higher sCal levels if a flare was observed up to 3 to 9 months following the test.ConclusionsThis study confirms the potential uses of serum calprotectin as a biomarker in the diagnosis and follow-up of JIA.

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