Detection Of Disease Activity Research Articles

Calprotectin as a marker of disease activity in spondyloarthritis

Introduction: Spondyloarthritis is a group of arthritic diseases that classically manifest as inflammation of the sacroiliac and limb joints, as well as the axial skeleton. Microscopic bowel inflammation is identifiable in over 50% of these patients. Calprotectin measured in the serum and stool is an emerging surrogate marker of disease activity and/or bowel inflammation in spondyloarthritis. Objective: This article aims to review published literature to determine the accuracy of serum and faecal calprotectin levels for use in monitoring bowel inflammation and spondyloarthritis disease activity. Methods: Boolean operator strategy was used to search two databases (Embase and PubMed) and 52 relevant articles were identified. Duplicate results were eliminated, inclusion and exclusion criteria were applied. Results: Ten studies met the inclusion criteria and were summarized and analyzed. The EBL Validity questionnaire was applied to determine study quality. All research articles found calprotectin to be elevated in spondyloarthritis patients when compared to controls. Faecal calprotectin was superior in detecting disease activity and correlated with disease activity questionnaires, radiology and endoscopy results. Only one study assessed the effect of treatment on calprotectin and its correlation to bowel inflammation. Conclusion: Faecal calprotectin shows promise as a surrogate marker for disease activity and bowel inflammation in spondyloarthritis. Future studies should focus on the effect of treatment on faecal calprotectin and whether some treatments are better at preventing the progression of bowel inflammation in addition to treating spondyloarthritis. Supplementary investigations are needed to identify whether faecal calprotectin is useful as a marker of disease activity in response to treatment.

Ultrasound 7 score and serum chemerin in rheumatoid arthritis: Relation to disease activity

Background and objectives. Chemerin is an adipokine associated with inflammation. Musculoskeletal ultrasonography is now widely used for detecting and monitoring RA due to its availability, practicality, and cost-effectiveness. The aim of the study is to determine how accurate serum chemerin levels are as a biomarker of active rheumatoid arthritis (RA) disease and to find a link between these levels and ultrasonographic findings. Materials and methods. This case-control study included 44 RA cases and 44 matched-age and sex-healthy controls. Seven joints in the hand and foot were assessed by the German-ultrasound 7 (GUS 7) score through greyscale (GSUS) and power Doppler US (PDUS) in all cases. Disease activity score 28 (DAS28) evaluated RA activity, and serum chemerin levels were measured in cases and controls. Both DAS28-CRP and DAS28-ESR were used in the assessment of the disease. Outcomes. The mean age of patients was 43.48 years versus 39.18 years of controls. 86.4% of patients were females; versus 75% of the control group. 47.7% of the studied patients had moderate disease activity, 31.8% had high disease activity, and 20.5% had low disease activity. There was a statistically significant higher median chemerin serum level among RA cases compared to the control group (898.94 versus 247.81 ng/ml, p <0.001). There was a significant difference in median chemerin between inactive and active patients (352.1 versus 1032.6 ng/ml (p <0.001). The serum chemerin can differentiate between RA patients and controls with a higher sensitivity of 81.8%, a specificity of 88.6%, and an accuracy of 85.2%. There was a statistically significant positive association between chemerin and RA activity (p <0.001). The combination of serum chemerin level and tenosynovitis GSUS score and DAS2-ESR to differentiate between active and inactive RA patients was highly significant with the highest sensitivity of 100%. Conclusions. Chemerin can serve as an advantageous marker for assessing RA activity. The US-7 score is a complementary method used in standard assessment to determine synovial inflammation in RA. The combined use of serum chemerin and the US 7 score could be of high value for detecting disease activity in RA.

Peak enhancement in contrast-enhanced ultrasound for assessing endoscopic disease activity in patients with Crohn's disease: a meta-analysis.

Contrast-enhanced ultrasound (CEUS) has been proposed as a valuable tool for detecting disease activity in patients with Crohn's disease (CD). However, previous studies have utilized different parameters, leading to variation in clinical assessment of this technique. To assess the effectiveness of peak enhancement (PE) in CEUS for evaluating endoscopic disease activity in patients with CD. Articles were obtained by searching PubMed, Embase, Web of Science, Wanfang, and CNKI databases. Only studies that investigated the effectiveness of PE in CEUS to discriminate endoscopic disease activity in patients with CD were considered. Pooled sensitivity and specificity were then calculated using a random effects model. Overall, seven studies were included. The endoscopic disease activity of CD was determined based on the simple endoscopic score for Crohn's disease and Crohn's Disease Endoscopic Index of Severity scores. Pooled results showed that a high PE was associated with increased detection efficacy for endoscopic disease activity in CD. Pooled sensitivity, specificity, and positive and negative likelihood ratios were 0.88 (95% confidence interval [CI] = 0.71-0.96), 0.88 (95% CI = 0.81-0.93), 7.60 (95% CI = 4.61-12.53), and 0.14 (95% CI = 0.05-0.35), respectively. The pooled receiver operating characteristic was 0.90 (95% CI = 0.87-0.92), suggesting a good discriminating efficacy of PE in CEUS for endoscopic disease activity of patients with CD. A high PE in CEUS displayed substantial distinguishing accuracy for assessing endoscopic disease activity of patients with CD.

Muscle MRI as a biomarker of disease activity and progression in myotonic dystrophy type 1: a longitudinal study

IntroductionMyotonic dystrophy type 1 (DM1) is an autosomal dominant disease characterized by myotonia and progressive muscular weakness and atrophy. The aim of this study was to investigate the usefulness of longitudinal muscle MRI in detecting disease activity and progression in DM1, and to better characterize muscle edema, fat replacement and atrophy overtime.Materials and methodsThis is a prospective, observational, longitudinal study including 25 DM1 patients that performed at least two muscle MRIs. Demographic and genetic characteristics were recorded. Muscular Impairment Rating Scale (MIRS) and MRC score were performed within 3 months from MRIs at baseline (BL) and at follow-up (FU). We analysed 32 muscles of lower body (LB) and 17 muscles of upper body (UB) by T1 and STIR sequences. T1-, STIR- and atrophy scores and their variations were evaluated. Correlations between MRIs’ scores and demographic, clinical and genetic characteristics were analysed.ResultsEighty (80%) of patients showed fat replacement progression at FU. The median T1 score progression (ΔT1-score) was 1.3% per year in LB and 0.5% per year in UB. The rate of fat replacement progression was not homogenous, stratifying patients from non-progressors to fast progressors (> 3% ΔT1-score per year). Half of the STIR-positive muscles at BL showed T1-score progression at FU. Two patients with normal MRI at baseline only showed STIR-positive muscle at FU, marking the disease activity onset. STIR positivity at baseline correlated with fat replacement progression (ΔT1-score; p < 0.0001) and clinical worsening at FU (ΔMRC-score; p < 0.0001). Sixty-five (65%) of patients showed STIR- and fat replacement-independent muscle atrophy progression, more evident in UB.ConclusionsMuscle MRI represents a sensitive biomarker of disease activity, severity, and progression in DM1. STIR alterations precede fat replacement and identify patients with a higher risk of disease progression, while T1-sequences reveal atrophy and fat replacement progression before clinical worsening.

A novel ultrasound-based score for assessing carotid artery activity in Takayasu's arteritis.

The role of ultrasonography for evaluating vessel wall inflammation in Takayasu's arteritis (TAK) is well-recognised; however, an effective approach for the quantitative assessment of disease activity remains lacking. This study aimed to develop a novel ultrasound-based score for determining TAK activity. TAK patients with carotid artery involvement were prospectively followed-up for 6 months. Our proposed ultrasonographic activity score (ULTRAS, range between 0-12) consisted of wall thickness (TS, range between 0-8) and semi-quantitative echogenicity scores (ES, range between 0-4). The diagnostic performance of ULTRAS for disease activity was evaluated in terms of area under the receiver operating characteristic curve (AUC). Internal validation was subsequently performed. The patients were divided into training and validation groups (n=136 and 30. respectively). In the training group, 83 (61.0%) had active disease. At an optimal cut-off of 7, ULTRAS showed good diagnostic accuracy for active TAK (AUC, 0.88; 95% CI, 82-94). Improved diagnostic performance was achieved when combined with ESR (AUC, 0.91; 95% CI, 86-96) or CRP (AUC, 0.90; 95%CI, 86-95). In the verification group, the AUCs were 0.88, 0.95, and 0.92 for ULTRAS, ESR plus ULTRAS, and CRP plus ULTRAS, respectively. At post-treatment follow-up, the TS, ES, and ULTRAS paralleled the patients' disease remission and symptom recovery. At 3-month follow-up, an improvement in wall thickness of ≥0.3 mm correlated with symptom recovery in 50% of the patients. Our proposed ultrasound-based score carries the potential in the detection of active disease among TAK patients.

Absolute monocyte counts could predict disease activity and secondary loss of response of patients with Crohn's disease treated with anti-TNF-α drug.

Assessing Crohn's disease (CD) activity is critical for monitoring disease progression. In CD, monocytes could release TNF-α. Thus, it is extremely important to study its role in the disease activity and loss of response to anti-TNF-α biologics. In this study, we collected CD patients treated with biologics from January 2017 to May 2022. Indicators associated with disease activity were evaluated by Spearman correlation analysis and Mann-Whitney U test. Specifically, logistic analyses were used to explore the predictors of primary nonresponse (PNR) and secondary loss of response (SLOR) within 1 year of anti-TNF-α agents. In addition, a nomogram was developed for therapeutic effect prediction. 283 patients with CD were identified. Disease activity group, defined as CDAI equal to or greater than 150, had significant elevated absolute monocyte counts than disease remission group based on CDAI score (p = 0.019, Z = -2.354). Logistic analyses showed that absolute monocyte counts could be an independent predictor of 1-year SLOR of anti-TNF-α agents in CD patients (p = 0.013). A nomogram established based on gender, absolute monocyte counts, and hemoglobin could predict SLOR within 1 year of anti-TNF-α agents reliably. The results of this study support the utility of absolute monocyte counts detecting disease activity and anti-TNF-α therapy effect in patients with CD.

How Does Ultrasound Global OMERACT-EULAR Synovitis Score (GLOESS) for Rheumatoid Arthritis (RA) Activity Assessment Perform in Real-Life?

This study aimed to assess the effectiveness of the Global OMERACT-EULAR Synovitis Score (GLOESS) of bilateral second to fifth metacarpophalangeal joints (MCP 2-5) in evaluating rheumatoid arthritis (RA) activity in a real-life setting. This cross-sectional study included consecutive RA patients without hyperalgesia. Clinical data were extracted from electronic medical records. Evaluations were conducted on bilateral MCP 2-5 by two independent experts in musculoskeletal ultrasound (MSUS). Correlation between clinical and ultrasonographic parameters was analyzed, aiming to define a cutoff value for detecting disease activity. Sixty-nine patients were included. The mean DAS28-ESR was 4.3 (±1.4), and the median GLOESS was 7 (13). The correlation between GLOESS and DAS28 was moderate (r = .62; P < .05). A total GLOESS score of ≤3 and all joints with both GS and PD ≤1 showed good sensitivity and specificity for detecting disease activity (remission/low vs moderate/high, P = 0). In a real-life scenario, GLOESS for MCP 2-5 emerges as a valuable measure of RA activity. The optimal cutoff distinguishing remission/low from moderate/high disease activity was determined to be GLOESS ≤3, with all MCP joints exhibiting both GS and PD scores of ≤1.

Correlation between Interleukin-23, Autoantibodies and Thyroid Profile in a Sample of Iraqi Patients with Hashimoto’s Thyroiditis

Hashimoto’s thyroiditis (HT) is the most common autoimmune condition characterized by hypothyroidism and thyroid cell death by leukocytes and antibody-mediated immunological mechanisms. The current paper set out to assess a number of inflammatory and metabolic potential indicators of Hashimoto’s thyroiditis. Fifty-one patients with Hashimoto’s thyroiditis took part in the current investigation. Ages ranged from 20 to 75 for them. Enzyme-linked immunosorbent tests were used to quantify the anti-thyroperoxidase antibody (anti-TPO Ab), anti-thyroglobulin antibody (anti-Tg Ab), T4, T3, and TSH at the beginning and conclusion of the trial. The result showed a significant relationship between the gender according to HT patient’s and healthy groups which showed that female effected more than male and there was no effected according to the age between these groups, In HT patients with greater TSH concentrations compared to healthy individuals, serum FT3 and FT4 concentrations were lower while IL-23 concentrations were higher. TSH levels were positively correlated with IL-23 levels. As a result, IL-23 can be useful for detecting disease activity in HT patients. However, more thorough, extensive, longitudinal investigations are required to evaluate whether IL-23 can be used in therapeutic settings. Our results showed that thyroid profile, serum IL-23, and autoantibodies were strong indicators of Hashimoto’s thyroiditis. To better understand these correlations and underlying pathogenic mechanisms, additional research is necessary.

Cardiopulmonary exercise testing complements both spirometry and nuclear imaging for assessing sarcoidosis stage and for monitoring disease activity.

Pulmonary sarcoidosis is a systemic disease that can confound established follow-up tools. Pulmonary function tests (PFTs) are recommended in initial and follow-up patient evaluations yet are imperfect predictors of disease progression. The cardiopulmonary exercise test (CPET) is another potentially useful monitoring tool, although previous studies report conflicting findings regarding which variables are altered by the disease. Nuclear imaging tests are also employed to assess inflammatory activity and may be predictive of functional deterioration. We asked whether PFTs or CPET are more diagnostic of disease stage, which subsets of functional variables are impacted by the disease, and how these relate to nuclear imaging signs of active inflammation. We collected retrospective data (spirometry, CPET, Gallium-67 scintigraphy, 18F-FDG PET/CT) from 48 patients and 10 controls. Disease severity was assessed following Scadding classification. First, we correlated individual PFTs and CPET parameters to Scadding stage and nuclear imaging data. Next, we performed Principal Component Analysis (PCA) on PFTs and CPET parameters, separated into respiratory, cardiovascular and metabolic subsets. Finally, we constructed multiple regression models to determine which variable subsets were the best predictors of Scadding stage and disease activity. The majority of PFTs and CPET single parameters were significantly correlated with patient stage, while only few correlated with disease activity. Nevertheless, multiple regression models were able to significantly relate PFTs and CPET to both disease stage and activity. Additionally, these analyses highlighted CPET cardiovascular parameters as the best overall predictors of disease stage and activity. Our results display how CPET and spirometry data complement each other for sarcoidosis disease staging, and how these tests are able to detect disease activity. Our findings suggest that CPET, a repeatable and non-invasive functional test, should be more routinely performed and taken into account in sarcoidosis patient follow-up.

Combined gray scale ultrasonography and doppler diagnostic tools with strain elastography in assessment of inflammatory bowel disease in pediatrics patients.

Intestinal changes in inflammatory bowel disease (IBD) are frequently observed on ultrasound. Invasive diagnostic procedures are often employed to differentiate between the main types of IBD and detect complications. Ultrasound Strain Elastography (SE) is a promising non-invasive technique for detecting intestinal changes and assessing inflammatory activity in pediatric IBD. This research aims to evaluate the diagnostic performance of conventional ultrasound, color Doppler, and SE in assessing inflammatory bowel disease in pediatric patients, both separately and in combination as additional tools. Forty patients (18 females and 22 males) initially diagnosed with IBD through clinical and endoscopic biopsy, along with 20 healthy controls, underwent conventional ultrasound, color Doppler, SE, and laboratory evaluations, including CBC, ESR, CRP, Fecal calprotectin, and assessment of IBD activity using PIBDAI. Conventional ultrasound, color Doppler, and SE significantly contributed to detecting disease activity and intestinal changes in IBD (p < 0.001; 95% CI 0.79-1.100), demonstrating better sensitivity in combination compared to each method individually. The combined approach showed 100% sensitivity, 84% specificity, 78.6% precision (Positive Predictive Value), 100% Negative Predictive Value, and an overall accuracy of 92%. The addition of Color Doppler and SE parameters to grayscale ultrasound provides diagnostic value comparable to endoscopy, histopathology, and laboratory markers in detecting inflammatory activity and intestinal changes in IBD. This combined approach can help avoid unnecessary invasive techniques for follow-up.

Serum neurofilament light for detecting disease activity in individual patients in multiple sclerosis: A 48-week prospective single-center study

Background: Serum neurofilament light (sNfL) reflects neuroaxonal damage and is now used as an outcome in treatment trials of relapsing-remitting multiple sclerosis (RRMS). However, the diagnostic properties of sNfL for monitoring disease activity in individual patients warrant further investigations. Method: Patients with suspected relapse and/or contrast-enhancing lesions (CELs) were consecutively included and performed magnetic resonance imaging (MRI) of the brain at baseline and weeks 28 and 48. Serum was obtained at baseline and 2, 4, 8, 16, 24, and 48 weeks. Neurofilament light concentration was measured using Single molecule array technology. Results: We included 44 patients, 40 with RRMS and 4 with clinically isolated syndrome. The median sNfL level peaked at 2 weeks post-baseline (14.6 ng/L, interquartile range (IQR); 9.3–31.6) and reached nadir at 48 weeks (9.1 ng/L, IQR; 5.5–15.0), equivalent to the median sNfL of controls (9.1 ng/L, IQR; 7.4–12). A baseline Z-score of more than 1.1 (area under the curve; 0.78, p < 0.0001) had a sensitivity of 81% and specificity of 70% to detect disease activity. Conclusion: One out of five patients with relapse and/or CELs did not change significantly in post-baseline sNfL levels. The utility of repeated sNfL measurements to monitor disease activity is complementary rather than a substitute for clinical and MRI measures.

P281 The role of transabdominal ultrasound in evaluating Ulcerative Colitis disease activity and predicting treatment response

Abstract Background As a non-invasive, accurate, and feasible examination, transabdominal ultrasound（TAUS） is of great value in disease monitoring of ulcerative colitis. The cut-off for disease activity of 3.0 or 4.0 mm is often debated， and studies lacked assessment of treatment response. This study aimed to assess the diagnostic accuracy of TAUS in detecting disease activity in adult patients with UC using endoscopy as the reference standard and evaluate TAUS for treatment response in a longitudinal cohort. Methods This study prospectively consecutive adult patients with an established diagnosis of UC in the First Affiliated Hospital of Zhengzhou University between June 2022 and September 2023. The patients with moderate-to-severe disease activity at baseline should have follow-up endoscopic assessment after induction, with available corresponding TAUS. The primary outcome of this study was the difference in BWT in the descending colon(DC) or sigmoid colon(SC) for patients with and without segmental endoscopic response after treatment. Endoscopic remission was defined as MES=0-1 or UCEIS=0-1, and Endoscopic response as a decrease of MES ≥1 or a reduction of UCEIS ≥2. Results A total of 315 colon segments in 80 patients were included. 171 (54.3%) colorectal segments had endoscopic remission and 144(45.7%) were in endoscopic activity. The TAUS parameters correlated with the Mayo endoscopic sub-score with significant differences between patients in endoscopic remission and patients in endoscopic activity. And we found 3.45mm(AUROC 0.833;95%CI 0.789-0.878， P＜0.001) to be the cutoff for endoscopic remission with 78% sensitivity and 75% specificity. The multivariable analysis identified BWT， the Colour Doppler Signal(CDS), and the wall layer stratification as independent predictors for endoscopic activity（ P＜0.001、=0.004、=0.001）. BWT in the DC or SC was significantly lower in patients with endoscopic response than those without after treatment. A 28% decrease in BWT from baseline predicted endoscopic response [AUROC 0.743;95%CI 0.589-0.896， P=0.015] with 58% sensitivity and 93% specificity. Multivariable analysis among all the ultrasound parameters considered normal wall layer stratification at baseline as the only independent predictor of endoscopic response at reassessment (odds ratio [OR]23.334, 95% CI 2.257-241.219; p = 0.008). Conclusion TAUS, importantly BWT, CDS, and wall layer stratification as the crucial parameters, is highly accurate in detecting disease activity and treatment response when evaluated against endoscopic outcomes. Normal intestinal wall stratification at baseline predicts long-term endoscopic response.

Interleukin 6 Levels and Disease Activity in Takayasu Arteritis: A Systematic Review With Meta-analysis.

Various studies have suggested interleukin 6 (IL-6) as a potential biomarker for detecting disease activity in Takayasu arteritis. A systematic review and meta-analysis was performed to assess differences in IL-6 levels in patients with active (aTA) and inactive Takayasu arteritis (iTA), as well as healthy controls (HCs), using validated activity scores. Study quality and the risk of bias were assessed using STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) and the Newcastle-Ottawa and Joanna Briggs checklist, respectively. For the meta-analysis, we pooled the raw mean IL-6 levels in each group and then estimated and pooled the crude mean differences between the groups. We applied a random-effects model in all analyses. Of the 93 eligible articles, 10 were included after removing duplicates and studies that met the exclusion criteria. Overall, 1825 patients with a mean age ranging from 24 to 40.6 years were included. The pooled levels of IL-6 increased depending on disease activity as follows: HCs: 3.08 (95% confidence interval [CI], 0.88-5.28), iTA: 7.21 (3.61-10.82), and aTA: 22.67 (12.44-32.91) pg/mL. Patients with aTA had higher IL-6 levels than HCs (21.52 [95% CI, -0.43 to 43.47]) and those with iTA (16.69 [95% CI, 5.32-28.06]), whereas IL-6 levels were not different between HCs and patients with iTA (3.62 [95% CI, -13.18 to 20.42]). Interleukin 6 levels are significantly increased in patients with aTA compared with those with iTA and HCs but not in patients with iTA compared with HCs. More studies are needed to establish the IL-6 cutoff value for assessing disease activity.

Musculoskeletal ultrasonography in rheumatic diseases.

Ultrasonography is an imaging technique based on sound waves used for the evaluation of soft tissues. Sound waves have been used for a long time in nonmedical fields, including military defense systems, radar systems, and detection of icebergs. Technological advances resulted in new techniques becoming available for medical imaging, including ultrasonography, magnetic resonance imaging, and computed tomography. Nowadays, the use of imaging has become a gold standard protocol in the diagnosis of many diseases, and recently developed diagnosis and therapy options provide more efficient treatment of rheumatic diseases. Thus, it has become possible to prevent structural damage and disability in patients with rheumatic disease. Musculoskeletal ultrasonography is becoming a preferred imaging technique for rheumatic diseases, as it has many advantages. Among its advantages are being inexpensive, being radiation-free, having a dynamic image capacity, helping to detect disease activity, and helping with early detection and diagnosis of structural damage. This review summarizes the use of ultrasonography in rheumatic diseases.

Remote Monitoring of Visual Function in Patients with Maculopathy: The Aphelion Study.

Remote monitoring of vision, using tools such as the shape discrimination hyperacuity (SDH) test, can detect disease activity in patients with maculopathy. We determined the in-clinic accuracy and repeatability of three myVisionTrack expanded version (mVTx) tests for self-testing of visual acuity (VA) and contrast sensitivity. Aphelion, a single-arm, prospective study conducted at two sites in the USA, included adults with any maculopathy and a baseline VA of 0.7 log of minimum angle of resolution (logMAR) (Snellen 20/100) or better. Participants completed the mVTx tests (tumblingE, LandoltC, contrast sensitivity, and SDH) and standard clinical tests (near and distance Early Treatment Diabetic Retinopathy Study [ETDRS] charts and the Pelli-Robson contrast sensitivity chart). Test-retest repeatability and agreement between the mVTx tests and the corresponding clinical test were assessed by Bland-Altman analyses. Participants also completed a usability survey. The mean age of the 122 participants was 67years. The most common diagnosis was age-related macular degeneration (42% of patients). The tumblingE test had a test-retest 95% limit of agreement (LoA) of ± 0.18logMAR; the LandoltC test, ± 0.23logMAR; the SDH test, ± 0.24logMAR; and the contrast sensitivity test, ± 0.32 log contrast threshold (logCT). Compared with the distance ETDRS chart, the LoA was ± 0.35logMAR for the tumblingE test (mean difference, - 0.07logMAR) and ± 0.39logMAR for the LandoltC test (mean difference, 0.03logMAR). For the contrast sensitivity test, the LoA compared with the Pelli-Robson chart was ± 0.30 logCT (mean difference, - 0.25logCT). Most participants (85%) reported that they learned the tests quickly. The tumblingE test scored the highest on ease of use. The mVTx tests of VA are accurate and repeatable, supporting their potential use alongside the SDH test to detect disease progression remotely between clinic visits.

Could ultrasound and muscle elastography be associated with clinical assessment, laboratory and nailfold capillaroscopy in juvenile dermatomyositis patients?

BackgroundJuvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children. Imaging exams are useful for muscle assessment, with ultrasonography (US) being a promising tool in detecting disease activity and tissue damage. There are few studies about muscle elastography.ObjectivesOur aim was to associate clinical, laboratory, and nailfold capillaroscopy (NC) assessments with US in JDM patients; and to compare the findings of US and Strain Elastography (SE) from patients and healthy controls.MethodsAn analytic cross-sectional study was performed with JDM patients and healthy controls. Patients underwent clinical exam to access muscle strength and completed questionnaires about global assessment of the disease and functional capacity. Patients were submitted to NC and measurement of muscle enzymes. All subjects underwent US assessment, using gray scale, Power Doppler (PD), and SE.ResultsTwenty-two JDM patients and fourteen controls, aged between 5 and 21 years, matched for age and sex were assessed. In qualitative and semi-quantitative gray scale, we observed a higher frequency of alterations in patients (p < 0.001), while in PD, there was a higher frequency of positivity in patients’ deltoids and anterior tibialis (p < 0.001). Active disease was associated with an important change in the semi-quantitative gray scale in deltoids (p = 0.007), biceps brachii (p = 0.001) and quadriceps femoris (p = 0.005). The SE demonstrated a high negative predictive value of 87.2.ConclusionUS was able, through gray scale, to differentiate JDM patients from controls, while PD achieved such differentiation only for deltoids and anterior tibialis. The semi-quantitative gray scale showed disease activity in proximal muscles. SE was not able to differentiate patients from controls.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): current understanding and challenges.

New diagnostic criteria for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have recently been proposed, distinguishing this syndrome from other inflammatory diseases of the central nervous system. Seropositivity status for MOG-IgG autoantibodies is important for diagnosing MOGAD, but only in the context of robust clinical characterization and cautious interpretation of neuroimaging. Over the last several years, access to cell-based assay (CBA) techniques has improved diagnostic accuracy, yet the positive predictive value of serum MOG-IgG values varies with the prevalence of MOGAD in any given patient population. For this reason, possible alternative diagnoses need to be considered, and low MOG-IgG titers need to be carefully weighted. In this review, cardinal clinical features of MOGAD are discussed. Key challenges to the current understanding of MOGAD are also highlighted, including uncertainty regarding the specificity and pathogenicity of MOG autoantibodies, the need to identify immunopathologic targets for future therapies, the quest to validate biomarkers that facilitate diagnosis and detect disease activity, and the importance of deciphering which patients with MOGAD require long-term immunotherapy.

Manifestation and staging of arthropathy in cystic fibrosis. Defining different stages of cystic fibrosis arthropathy using ultrasound imaging and clinical scoring

BackgroundThe true prevalence of cystic fibrosis arthropathy (CFA) remains unclear and may be significantly higher than previously reported. In recent studies, joint symptoms have been reported in up to 30% of adults with CF. This underlines the importance of CFA as a rising and clinically relevant co-morbidity. A clear definition of CFA is yet missing and its pathogenesis remains unclear. We investigated the clinical manifestation of CFA particularly via ultrasound (US) examination to define and implement a staging for clinical assessment. MethodsIn a prospective cohort study between March 2018 and February 2020 a total of 98 consecutively recruited, adult cystic fibrosis (CF) patients underwent joint-US examination according to a newly developed ultrasound score (US-CFA). A clinical assessment including rheumatological scores (DAS28, HAQ) has been conducted as well as a specially designed questionnaire. Investigation on clinical and microbiological data, as well as a comprehensive laboratory analysis, were carried out. Cluster analysis has been performed to detect patterns defining different CFA stages based on disease activity. ResultsUS imaging has shown a considerable incidence of mild to moderate effusion as sign of joint inflammation/(teno-)synovitis. K-means clustering was used to distinguish 3 different stages of CFA based on the intensity of the detected effusion. These stages showed a significant association with disease activity (DAS28, p = 0.0004) as well as with patient-reported symptoms such as total weeks of CFA per year (p = 0.004), acute CFA (p = 0.015), chronic CFA (p = 0.016), disease burden (p = 0.04). Based on the US-CFA, 16% of patients suffered from severe CFA (II), 51% from intermediate CFA (I) and 33% did not present detectable arthritis. Positive serology for Chlamydophilia pneumoniae (IgA, IgG) and Chlamydia trachomatis (IgA, IgG) significantly correlated with the US-CFA. ConclusionsThe results of this study show that a definition and categorization for the clinical manifestation of CFA can be described through US examination, which is able to detect disease activity concordant with the DAS28 as a validated clinical score on arthritis. Defining these stages will lead to a better understanding of the clinical phenotype of the disease and will optimize diagnosis, therapy and research on CFA in the future.

Biomarkers in autoimmune diseases of the central nervous system.

The autoimmune diseases of the central nervous system (CNS) represent individual heterogeneity with different disease entities. Although clinical and imaging features make it possible to characterize larger patient cohorts, they may not provide sufficient evidence to detect disease activity and response to disease modifying drugs. Biomarkers are becoming a powerful tool due to their objectivity and easy access. Biomarkers may indicate various aspects of biological processes in healthy and/or pathological states, or as a response to drug therapy. According to the clinical features described, biomarkers are usually classified into predictive, diagnostic, monitoring and safety biomarkers. Some nerve injury markers, humoral markers, cytokines and immune cells in serum or cerebrospinal fluid have potential roles in disease severity and prognosis in autoimmune diseases occurring in the CNS, which provides a promising approach for clinicians to early intervention and prevention of future disability. Therefore, this review mainly summarizes the potential biomarkers indicated in autoimmune disorders of the CNS.

Evaluation of serum calcitonin gene related peptide (CGRP) Level in HIV infected patients as an indicator of disease activity

Human immunodeficiency virus (HIV) infection is under global attention due to its rapid spread and high rate of morbidity and mortality. HIV gets an access into the mucosa of genital epithelium through binding to Langerhans cells. While viral load and CD4+ cell count are the main parameters to detect disease activity, new biomarkers are introduced as a potential parameter for monitoring of disease activity in HIV infected patients. Calcitonin Gene Related Peptide (CGRP) is a neuropeptide that is secreted by peripheral neurons at genital epithelia and plays an important role in limitation of HIV transmission and spread to infected CD4+ cells through its effect onto Langerhans cells. This study aimed to evaluate the serum level of CGRP in HIV infected patients and to determine whether CGRP can serve as an indicator of HIV infection activity. The study included 104 HIV patients and 24 normal controls. Patients were divided into four groups. Serum levels of CGRP were measured by ELISA and correlated to viral load and CD4+ cells count for patients in the four groups: primary HIV infection (PHI), chronic HIV infection (CHI) before combinational antiretroviral therapy (cART-naïve), chronic HIV infection after one year of cART-initiation, and chronic HIV infection after two years of cART. Serum levels of CGRP were also measured in sera of controls and compared to patients' groups. Serum levels of CGRP were significantly lower in cART naïve PHI and CHI patients in comparison with normal controls (p < 0.05), Also, serum CGRP levels were positively correlated with CD4+ cells count (p < 0.01), but negatively correlated with viral load (p>0.05). In conclusion, CGRP could be proposed as an indicator of disease activity in HIV patients.