OBJECTIVES: The main objective was to generate a GLobal OMERACT Ultrasound DActylitis Score (GLOUDAS) in psoriatic arthritis and to test its reliability. To this end, we assessed the validity, feasibility and applicability of ultrasound assessment of finger entheses to incorporate them into the scoring system. METHODS: The study consisted of a stepwise process. First, in cadaveric specimens, we identified enthesis sites of the fingers by ultrasound and gross anatomy, and then verified presence of entheseal tissue in histological samples. We then selected the entheses to be incorporated into a dactylitis scoring system through a Delphi consensus process among international experts. Next, we established and defined the ultrasound components of dactylitis and their scoring systems using Delphi methodology. Finally, we tested the interobserver and intraobserver reliability of the consensus- based scoring systemin patients with psoriatic dactylitis. RESULTS: 32 entheses were identified in cadaveric fingers. The presence of entheseal tissues was confirmed in all cadaveric samples. Of these, following the consensus process, 12 entheses were selected for inclusion in GLOUDAS. Ultrasound components of GLOUDAS agreed on through the Delphi process were synovitis, tenosynovitis, enthesitis, subcutaneous tissue inflammation and periextensor tendon inflammation. The scoring system for each component was also agreed on. Interobserver reliability was fair to good (κ 0.39-0.71) and intraobserver reliability good to excellent (κ 0.80-0.88) for dactylitis components. Interobserver and intraobserver agreement for the total B-mode and Doppler mode scores (sum of the scores of the individual abnormalities) were excellent (interobserver intraclass correlation coefficient (ICC) 0.98 for B-mode and 0.99 for Doppler mode; intraobserver ICC 0.98 for both modes). CONCLUSIONS: We have produced a consensus-driven ultrasound dactylitis scoring system that has shown acceptable interobserver reliability and excellent intraobserver reliability. Through anatomical knowledge, small entheses of the fingers were identified and histologically validated.

• MeSH
• Delphi Technique MeSH
• Adult MeSH
• Enthesopathy diagnostic imaging   MeSH
• Finger Joint * diagnostic imaging   pathology   MeSH
• Middle Aged MeSH
• Humans MeSH
• Cadaver MeSH
• Observer Variation MeSH
• Fingers diagnostic imaging   pathology   MeSH
• Arthritis, Psoriatic * diagnostic imaging   MeSH
• Reproducibility of Results MeSH
• Aged MeSH
• Feasibility Studies MeSH
• Severity of Illness Index * MeSH
• Synovitis diagnostic imaging   pathology   MeSH
• Tenosynovitis diagnostic imaging   MeSH
• Ultrasonography * methods   MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Validation Study MeSH

The aim of this study was to describe the clinical and molecular genetic findings in seven individuals from three unrelated families with Blau syndrome. A complex ophthalmic and general health examination including diagnostic imaging was performed. The NOD2 mutational hot spot located in exon 4 was Sanger sequenced in all three probands. Two individuals also underwent autoinflammatory disorder gene panel screening, and in one subject, exome sequencing was performed. Blau syndrome presenting as uveitis, skin rush or arthritis was diagnosed in four cases from three families. In two individuals from one family, only camptodactyly was noted, while another member had camptodactyly in combination with non-active uveitis and angioid streaks. One proband developed two attacks of meningoencephalitis attributed to presumed neurosarcoidosis, which is a rare finding in Blau syndrome. The probands from families 1 and 2 carried pathogenic variants in NOD2 (NM_022162.3): c.1001G>A p.(Arg334Gln) and c.1000C>T p.(Arg334Trp), respectively. In family 3, two variants of unknown significance in a heterozygous state were found: c.1412G>T p.(Arg471Leu) in NOD2 and c.928C>T p.(Arg310*) in NLRC4 (NM_001199139.1). In conclusion, Blau syndrome is a phenotypically highly variable, and there is a need to raise awareness about all clinical manifestations, including neurosarcoidosis. Variants of unknown significance pose a significant challenge regarding their contribution to etiopathogenesis of autoinflammatory diseases.

• MeSH
• Arthritis * genetics   diagnosis   MeSH
• Hereditary Autoinflammatory Diseases MeSH
• Humans MeSH
• Lymphedema genetics   diagnosis   MeSH
• Mutation * MeSH
• Central Nervous System Diseases MeSH
• Arthropathy, Neurogenic genetics   diagnosis   MeSH
• Pedigree * MeSH
• Sarcoidosis * genetics   diagnosis   MeSH
• Exome Sequencing MeSH
• Nod2 Signaling Adaptor Protein * genetics   MeSH
• Synovitis * genetics   diagnosis   MeSH
• Uveitis * genetics   diagnosis   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH

OBJECTIVES: The aim of the present study was to propose a methodologically innovative sonographic approach for optimal evaluation of synovial tissues (starting from histopathology). METHODS: Using high-frequency ultrasound probes and high-level ultrasound machines, we matched the histological microarchitecture of synovial tissues with multiple sonographic patterns in physiological and pathological conditions. Likewise, high-sensitive color/power Doppler assessments have also been performed to evaluate the microcirculation. RESULTS: Modern equipment allows for a macroscopic classification of synovial pathologies recognizing different morphological patterns; however, intimal and subintimal layers of the synovium cannot be distinguished from each other on ultrasound. High-sensitive Doppler imaging clearly defines the microvascular pattern, especially in patients with hypertrophic synovial pathologies. CONCLUSIONS: In clinical practice, using adequate technological equipment i.e. high-frequency B-mode and high-sensitive Doppler imaging, detailed sonographic assessment of synovial tissues can be performed - defining the main sono-histological patterns.

• MeSH
• Humans MeSH
• Arthritis, Rheumatoid * pathology   MeSH
• Muscles pathology   MeSH
• Synovial Membrane diagnostic imaging   blood supply   pathology   MeSH
• Synovitis * diagnostic imaging   pathology   MeSH
• Ultrasonography MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH

• MeSH
• COVID-19 * prevention & control   MeSH
• Knee Joint MeSH
• Humans MeSH
• Synovitis * diagnosis   MeSH
• Vaccination adverse effects   MeSH
• COVID-19 Vaccines adverse effects   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Comment MeSH

OBJECTIVES: In the ULTIMATE study with an open label extension, we assessed the long-term effect of secukinumab at tissue level on synovitis and enthesitis, and across all psoriatic arthritis (PsA) manifestations, using both clinical evaluations and power Doppler ultrasonography (PDUS). METHODS: This randomised, placebo-controlled, Phase 3 study (ULTIMATE) included biologic-naïve patients with PsA with active PDUS synovitis and clinical enthesitis, and inadequate response to conventional synthetic disease-modifying antirheumatic drugs. The study consisted of 3 treatment periods; in the first period (baseline to week 12) patients were randomised to receive subcutaneous secukinumab (150 mg or 300 mg according to severity of skin psoriasis) or placebo every week until week 4 and once every 4 weeks up to week 12. In the second period (weeks 12-24) all patients received open-label secukinumab with placebo patients switching to secukinumab (150 mg or 300 mg). The third period (weeks 24-52) was an extended open-label treatment period. The long-term responsiveness of the Global EULAR-OMERACT Synovitis Score (GLOESS), clinical enthesitis and global PDUS-detected enthesitis score (using two candidate definitions of activity) at patient level, together with clinical efficacy across key manifestations of PsA and safety were assessed. RESULTS: Of the 166 patients enrolled, 144 completed week 52. A significant reduction in GLOESS was demonstrated in the secukinumab group vs placebo at week 12, followed by a stable reduction of synovitis until week 52 in the secukinumab group while placebo switchers from week 12 reached a similar level of reduction at week 24 with stability thereafter. Likewise, a significant reduction in the Spondyloarthritis Research Consortium of Canada (SPARCC) enthesitis index was shown in the secukinumab group vs placebo at week 12 with sustained improvement to week 52. Global OMERACT PDUS enthesitis scores were numerically lower in secukinumab vs placebo switchers in the first two treatment periods, with some stability in the third period in both groups. Improvements in clinical responses were also observed across all key domains of PsA up to week 52 in both treatment groups with no new or unexpected safety signals. CONCLUSIONS: ULTIMATE showed consistent improvements in clinically and ultrasound-assessed synovitis and enthesitis and sustained clinical efficacy through week 52 in patients with PsA treated with secukinumab and placebo switched to secukinumab.

• MeSH
• Antirheumatic Agents * adverse effects   MeSH
• Double-Blind Method MeSH
• Enthesopathy * diagnostic imaging   drug therapy   MeSH
• Antibodies, Monoclonal, Humanized adverse effects   MeSH
• Humans MeSH
• Arthritis, Psoriatic * complications   diagnostic imaging   drug therapy   MeSH
• Synovitis * diagnostic imaging   drug therapy   chemically induced   MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Clinical Trial, Phase III MeSH
• Randomized Controlled Trial MeSH

OBJECTIVES: Assessment of active synovitis is crucial for the management of juvenile idiopathic arthritis (JIA). We aimed to investigate the correlation of musculoskeletal ultrasound (MSUS) and clinical examination results and relate them to arthritis relapse rate. METHODS: JIA patients with questionable presence of active arthritis (Q-joints) and controls (JIA and healthy children) were recruited. MSUS of Q-joints, active joints and their inactive counterparts was performed at study entry. Standard disease activity parameters were prospectively recorded. RESULTS: Of 481 joints of 138 JIA patients, 99 joints (20.6%) of 58 patients had one or more Q-joints with 54/99 (54.5%) having MSUS features of active disease. Clinically inactive joints had lower proportion of MSUS synovitis (78/253, 30.8%) while MSUS activity was present in 114/129 (88.4%) of clinically active joints and in 2/105 (1.9%) joints of 36 healthy controls. Within the 15-month follow-up 23/99 (22%) Q-joints and 31/253 (12%) clinically inactive joints relapsed. Joints with subclinical synovitis relapsed more frequently than MSUS inactive ones (p<0.001). The relapse rate was higher in MSUS-active Q-joints (19/23, 82%) than in clinically inactive ones (16/31, 52%) with MSUS synovial hypertrophy as the main relapse predictor in multivariate analysis. Ankle and knee joints relapsed most frequently. CONCLUSIONS: Acknowledgement of joints with questionable synovitis may contribute to the assessment of disease activity in JIA. Presence of MSUS synovitis carries a clinically meaningful risk of disease recurrence in these joints. In clinical practice, our findings encourage timely MSUS assessment of the joints in question, especially in patients without any other features of active disease.

• MeSH
• Child MeSH
• Arthritis, Juvenile * diagnosis   MeSH
• Humans MeSH
• Uncertainty MeSH
• Recurrence MeSH
• Synovitis * diagnosis   MeSH
• Ultrasonography methods   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Publication type
• Journal Article MeSH

• MeSH
• Child MeSH
• Conservative Treatment MeSH
• Hip Joint * diagnostic imaging   pathology   MeSH
• Humans MeSH
• Femur Head Necrosis MeSH
• Legg-Calve-Perthes Disease diagnosis   etiology   physiopathology   therapy   MeSH
• Slipped Capital Femoral Epiphyses surgery   diagnosis   etiology   physiopathology   MeSH
• Synovitis diagnosis   etiology   physiopathology   therapy   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Publication type
• Review MeSH

Hypotéza: Muskuloskeletální ultrasonografie (MSUS) dětí s juvenilní idiopatickou artritidou (JIA) umožní identifikaci subklinické synovitidy i strukturálního poškození kloubů a významně přispěje k monitoraci onemocnění a optimalizaci terapie při menší zátěži pacienta i zdravotnického systému. Senzitivita MSUS pro synovitidu může mít srovnatelnou výpovědní hodnotu s magnetickou rezonancí (MR). Hlavní cíle: Zavedení a standardizace MSUS kloubů u dětí s JIA. Stanovení spektra fyziologických nálezů na MSUS. Stanovení klinické významnosti MSUS změn, standardizace hodnocení patologických nálezů. Definice sonografických parametrů kloubní remise. Metody: MSUS bude prováděna dvěma školenými operátory: u pacientů bez kloubního onemocnění v 6 věkových kategoriích a u pacientů s jakýmkoli podtypem aktivní JIA. Srovnání MSUS nálezů na klinicky aktivních kloubech dětí s JIA a na zdravých kloubech kontrol stejného věku. Porovnání klinického kloubního nálezu s MSUS a MR, korelace se standardizovanými parametry aktivity JIA, funkčního omezení a kvality života (JADAS, JAMAR, PedsQL, CHAQ).; Hypothesis: The identification of subclinical synovitis and structural damage in the joints by musculoskeletal ultrasonography (MSUS) in children with juvenile idiopathic arthritis (JIA) will contribute to the monitoring and optimizing of therapy with less burden for the patient and healthcare system. MSUS sensitivity for synovitis may have predictive value as MR imaging. Main objectives: The introduction and standardization MSUS of joints in children with JIA. Determining the range of MSUS physiological findings. Determining the clinical significance of MSUS changes and standardization of pathological findings. MSUS criteria for the remission in joint. Methods: MSUS will be performed by two trained operators: in patients without joint disease in six age-related categories and in patients with any subtype of active JIA. Comparison MSUS findings in clinically active joints in JIA and joints of healthy controls of the same age. Comparison of the clinical findings in joints with MSUS and MR and correlation with standardized parameters JIA activity, functional limits, quality of life.

• MeSH
• Child MeSH
• Arthritis, Juvenile diagnostic imaging   MeSH
• Cartilage, Articular MeSH
• Magnetic Resonance Imaging MeSH
• Synovitis diagnostic imaging   MeSH
• Ultrasonography, Doppler methods   MeSH
• Inflammation MeSH
• Check Tag
• Child MeSH

• Conspectus
• Pediatrie
• NML Fields
• pediatrie
• ortopedie
• revmatologie
• NML Publication type
• závěrečné zprávy o řešení grantu AZV MZ ČR

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and the resulting coronavirus 2019 disease (COVID-19) have spread all around the world since 2019 and have affected millions of people. The development of COVID-19 vaccines helped to decelerate the spread of the virus. However, as in the case of vaccines against other infectious diseases, adverse events can also present with COVID-19 vaccines. CASE PRESENTATION: We report here a rare case of a 53-year-old man with knee-joint synovitis, after the first dose of messenger RNA vaccine, with no fever and a negative COVID-19 reverse transcription polymerase chain reaction test. During a clinical examination the suspicion of pyogenic arthritis was excluded by blood tests and by a complex joint effusion examination, including a microbiological and cytological-energy analysis of the synovial fluid. The treatment received by our patient consisted of 3 doses of dexamethasone administered intravenously over a period of 3 days. All the symptoms improved after this therapy, and in the 3-week follow-up period we recorded full recovery with no consequences. CONCLUSION: Case reports on patients undergoing COVID-19 vaccination should be examined in order to detect rare and long-term side-effects. This is the first report to present the outcomes of an ultrastructural analysis of post-vaccination synovitis.

• MeSH
• COVID-19 * MeSH
• Knee Joint MeSH
• Middle Aged MeSH
• Humans MeSH
• SARS-CoV-2 MeSH
• Synovial Fluid MeSH
• Synovitis * MeSH
• Vaccination MeSH
• COVID-19 Vaccines MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH

BACKGROUND: There is no international consensus on an optimal ultrasound score for monitoring of rheumatoid arthritis (RA) on patient-level yet. Our aim was to reassess the US7 score for the identification of the most frequently pathologic and responsive joint/tendon regions, to optimize it and contribute to an international consensus. Furthermore, we aimed to evaluate the impact of disease duration on the performance of the score. METHODS: RA patients were assessed at baseline and after 3 and 6 months of starting/changing DMARD therapy by the US7 score in greyscale (GS) and power Doppler (PD). The frequency of pathologic joint/tendon regions and their responsiveness to therapy were analyzed by Friedman test and Cochrane-Q test respectively, including the comparison of palmar vs. dorsal regions (chi-square test). The responsiveness of different reduced scores and the amount of information retained from the original US7 score were assessed by standardized response means (SRM)/linear regression. Analyses were also performed separately for early and established RA. RESULTS: A total of 435 patients (N = 138 early RA) were included (56.5 (SD 13.1) years old, 8.2 (9.1) years disease duration, 80% female). The dorsal wrist, palmar MCP2, extensor digitorum communis (EDC) and carpi ulnaris (ECU) tendons were most frequently affected by GS/PD synovitis/tenosynovitis (wrist: 45%/43%; MCP2: 35%/28%; EDC: 30%/11% and ECU: 25%/11%) and significantly changed within 6 months of therapy (all p ≤0.003 by GS/PD). The dorsal vs. palmar side of the wrist by GS/PD (p < 0.001) and the palmar side of the finger joints by PD (p < 0.001) were more frequently pathologic. The reduced US7 score (GS/PD: palmar MCP2, dorsal wrist, EDC and ECU, only PD: dorsal MCP2) showed therapy response (SRM 0.433) after 6 months and retained 76% of the full US7 score's information. No major differences between the groups of early and established RA could be detected. CONCLUSIONS: The wrist, MCP2, EDC, and ECU tendons were most frequently pathologic and responsive to therapy in both early and established RA and should therefore be included in a comprehensive score for monitoring RA patients on patient-level.

• MeSH
• Humans MeSH
• Adolescent MeSH
• Arthritis, Rheumatoid * diagnostic imaging   drug therapy   pathology   MeSH
• Tendons diagnostic imaging   MeSH
• Severity of Illness Index MeSH
• Synovitis * pathology   MeSH
• Ultrasonography MeSH
• Wrist MeSH
• Wrist Joint diagnostic imaging   pathology   MeSH
• Check Tag
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Observational Study MeSH