American College Of Rheumatology/European League Against Rheumatism Research Articles

Effective Assessment of Rheumatoid Arthritis Disease Activity and Outcomes Using Monocyte Chemotactic Protein-1 (MCP-1) and Disease Activity Score 28-MCP-1.

The effectiveness of monocyte chemotactic protein-1 (MCP-1) and Disease Activity Score 28 (DAS28)-MCP-1 (DAS28-MCP-1) in assessing rheumatoid arthritis (RA) disease activity is unclear, although some studies have demonstrated their potential usefulness. The present study investigated relationships between MCP-1 and different DAS28 measures, the occurrence of residual swollen joints in different DAS28 remission statuses, changes in medication dosage in relation to the 2005 modified American Rheumatism Association and 2011 American College of Rheumatology/European League against Rheumatism (ACR/EULAR) remission definitions, and the correlations between different DAS28-related scores and Health Assessment Questionnaire Disability Index (HAQ-DI) scores in two RA patient cohorts. The results revealed that the MCP-1 level was correlated with five disease activity measures (DAS28-erythrocyte sedimentation rate [DAS28-ESR], DAS28-C-reactive protein [CRP], Simplified Disease Activity Index (SDAI), Clinical Disease Activity Index (CDAI), and DAS28-MCP-1) in multivariable regression analysis (all p < 0.05; ESR, CRP, and MCP-1 as independent variables). However, ESR was not significantly associated with SDAI and CDAI scores (p = 0.343 and 0.323, respectively). Residual swollen joints were more frequently observed in patients who met the DAS28-ESR remission criteria (<2.6) compared with those meeting the other four remission criteria, with a difference ranging from 71% to 94%. Among patients meeting the DAS28-ESR remission criteria (<2.6), medication changes (dose increase by ≥30% or new medications prescribed) were less frequent in those who also met the 2011 ACR/EULAR remission criteria than in those who did not meet them (p = 0.006). Moreover, the correlation coefficients for the relationship between DAS28-ESR and HAQ-DI scores were the lowest among the five disease activity measures. In conclusion, MCP-1 and DAS28-MCP-1 are effective in assessing RA disease activity, with less residual joint swelling and less frequent medication increases observed in the DAS28-MCP-1 remission < 2.2 subgroup.

Systemic lupus international collaborating clinics-2012 and European league against rheumatism/American college of rheumatology-2019 classification criteria for systemic lupus erythematosus associated with childhood-onset auto-immune cytopenia.

Systemic Lupus Erythematosus (SLE) can be diagnosed using the 2012 criteria of the Systemic Lupus International Collaborating Clinics (SLICC) and, more recently, the 2019 criteria of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR). Hematological involvement is scored differently by these classifications. Our objective was to compare both criteria in a cohort of children with autoimmune cytopenia (AIC)-associated SLE. We included 79 patients with childhood-onset AIC as the first manifestations of SLE. The median age at SLE diagnosis was 14.5years (1.1-21.4years). The SLICC criteria were fulfilled by 76/79 (96%) patients and the EULAR/ACR criteria by 72/79 (91%) patients during follow-up. The SLICC and EULAR/ACR criteria were discordant (not concomitantly fulfilled) in 25/79 (32%) patients. Non-hematological clinical manifestations were more frequently observed in SLE diagnosis when the criteria were concordant (30/54, 56%) than when they were not (5/25, 20%) (p = 0.004). In 16/25 (64%) discordant patients, the SLICC criteria allowed earlier diagnosis of SLE. Finally, the attribution of a maximum weight of 6 to the hematological involvement of the EULAR/ACR criteria increased the sensitivity thereof from 63/79 (80%) to 76/79 (96%) in our population. The SLICC 2012 and EULAR/ACR 2019 criteria do not effectively diagnose SLE in children when AIC is the predominant feature. The SLICC criteria appear to be more effective in this population of SLE patients. An increase in the maximum weight of hematological involvement to 6 increases the sensitivity of the EULAR/ACR criteria for SLE diagnosis in children.

Increased predictive value of optical spectral transmission in early rheumatoid arthritis through use of patient-adjusted cut-off scores

ObjectivesThe aims of this study were to suggest patient-adjusted optical spectral transmission (OST) cut-off values for the first time and to develop clinical models that predict the probability of an early rheumatoid arthritis (RA) diagnosis based on OST findings and the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria as a reference standard.MethodsOST examinations were performed in newly diagnosed RA patients and healthy controls by the HandScan device. Moreover, RA patients underwent a full clinical [tender/swollen joint counts (TJC/SJC), disease activity score-28 (DAS28)] and laboratory evaluation. OST confounding factors were examined via logistic multivariate regression analyses and patient-adjusted OST-cut-offs were subsequently determined. Furthermore, statistical models to calculate the probability of an RA diagnosis, based on the measured OST values and the presence of OST influencing factors, were developed. Finally, correlations of OST with RA activity parameters were assessed.Results1.584 joints of 72 early RA patients were examined via OST and compared to 2.200 joints of 100 healthy controls and 1.166 joints of 53 patients with non-inflammatory arthralgia (NIA), respectively. Overall OST diagnostic performance was excellent in the whole cohort between RA- and healthy control-group [Area-Under-the-Curve (AUC): 0.810 (95%CI: 0.746–0.873); p < 0.0001], and further improved in RA-patients with ≥ 1 swollen wrist/finger joint(s) [AUC: 0.841 (95%CI: 0.773–0.908); p < 0.0001]. Comparison between RA patients and patients with non-inflammatory arthralgia showed similar results by an AUC of 0.788 (95%-CI: 0.709–0.867; p < 0.0001), and further improved in RA patients with ≥ 1 swollen wrist/finger joint(s) [AUC: 0.822 (95%CI: 0.74–0.90); p < 0.0001]. For the assessment of an adjusted RA diagnosis probability, two gender-specific statistical models were developed, based on OST values and patient age. OST cut-off values of 11.2 and 18.21 were calculated for female and male patients with active disease (sensitivity 93% and 67%; specificity 71.2% and 90%), respectively. Among RA patients, OST was associated moderately/significantly with DAS28 (r = 0.42,p < 0.001) and swollen joint count (rho = 0.355,p = 0.002).ConclusionThe development of patient-adjusted OST cut-off values and the suggested statistical models significantly enhance OST’s diagnostic performance, supporting its utility in differentiating between RA and non-inflammatory conditions. Future research should include a broader spectrum of arthritis types to validate OST’s comprehensive diagnostic utility also across various inflammatory arthritides.Trial registrationDRKS00016752 (German Registry of Clinical Trials)

The spectrum of idiopathic inflammatory myopathies: a Tunisian cohort

Background Idiopathic inflammatory myopathies (IIM) encompass a heterogenous group of auto-immune diseases. The identification of myositis specific antibodies (MSA) and their associations with distinct phenotypes has improved the categorization of these conditions. Objective The aim of this study was to describe and report the clinical and immunological characteristics of IIM among Tunisian patients. Method A retrospective study conducted in the internal medicine department at the Rabta University Hospital Center over 22 years, including adult patients with IIM according to the American college of rheumatology/European league against rheumatism (ACR/EULAR) classification criteria and Connors’ criteria for anti-synthetase syndrome (ASS). Inclusion body myositis and myositis associated with other conditions were excluded. Demographic, clinical, and immunological characteristics were analyzed and compared. Results Ninety-seven patients were included (Male/female ratio= 0.36, mean age = 48.4 +- 13.8 years). Muscular involvement was present in 88% of patients, affecting locomotor muscles (88%), gastrointestinal (43%), laryngeal (10%), cardiac (8%), and respiratory (1%) systems. Muscle weakness was primarily noted in the pelvic girdle (81%), scapular region (74%), axial muscles (20%), and distal muscles (5%). Myolysis was observed in 77% of patients, and histological evidence of myositis in 73%. Diffuse interstitial pneumonia (DIP) was present in 45% of patients, cutaneous involvement in 85%, and articular involvement in 48%. MSAs were detected in 52% of patients. Analysis revealed significantly higher frequencies of amyopathic forms, DIP, palmar hyperkeratosis, and articular involvement in the ASS group. The DM group exhibited higher frequencies of gastrointestinal signs, Gottron’s papules, heliotrope rash, photosensitive rashes, ulcerations, and skin necrosis. The NAM group had higher frequencies of gastrointestinal signs, myolysis, and lower frequencies of DIP and cutaneous involvement. Conclusion Our findings corroborate previously established clinico-immunological associations reported in the literature underscoring the need for a combined clinico-serological approach in classifying IIM.

Undetected intraabdominal sepsis due to prolonged corticosteroid therapy in systemic lupus erythematosus: a case report and literature review

Gastrointestinal manifestations are not included in the criteria for diagnosing systemic lupus erythematosus (SLE) by the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) 2019, however, gastrointestinal disorders are one of the potentially fatal complications of SLE. Mesenteric vasculitis and thrombosis are the two main mechanisms that cause gastrointestinal disorders in SLE patients. Long-term immunosuppressants used by SLE patients might alter the immune system’s response to infection, leading to immunoparalysis. The delay in recognizing might cause sepsis, thus increasing the mortality risk. A 47-year-old woman with a history of SLE complained of hematochezia. The patient had been consistently consuming 12 mg of methylprednisolone every day for the past five years without doctor’s supervision. Past medical history of congestive heart failure (CHF) caused by coronary arterial disease (CAD). After 24 days hospitalized for observation of hematochezia, an emergency laparotomy was performed due to deterioration caused by peritonitis. Intraoperative, 60 cm of jejunal necrosis was identified and intestinal resection was done. The patient’s condition improved in two days after emergency laparotomy and intensive treatment. The undetected intraabdominal sepsis caused by jejunal infarction and gut necrosis due to long-term use of corticosteroid that masked the manifestations of severe infection in the gastrointestinal tract infarction and necrosis. Abbreviations: NLR - neutrophil to lymphocyte ratio; SGOT - serum glutamic oxaloacetic transminase; SGPT - serum glutamic pyruvic transminase; eGFR - estimated glomerular filtration rate; PT - prothrombin time; APTT - activated partial thromboplastin time; INR - international normalized ratio Keywords: Systemic lupus erythematosus, Intraabdominal sepsis, Immunoparalysis, Corticosteroid Citation: Leman YE, Irawany V, Manggala SK. Undetected intraabdominal sepsis due to prolonged corticosteroid therapy in systemic lupus erythematosus: a case report and literature review. Anaesth. pain intensive care 2024;28(4):765−771; DOI: 10.35975/apic.v28i4.2525 Received: March 13, 2024; Reviewed: May 17, 2024; Accepted: May 21, 2024

Immunoregulatory Cells and Cytokines Discriminate Disease Activity Score 28-Remission Statuses and Ultrasound Grades in Rheumatoid Arthritis Patients with Non-High Disease Activity.

It is not clear whether immunoregulatory cytokines and cells are associated with Disease Activity Score 28 (DAS28) scores and ultrasound grades/scores. Here, we investigated the relationships between immunoregulatory cytokines or cells and different DAS28 scores or ultrasound grades/scores in patients with rheumatoid arthritis (RA). This study enrolled 50 RA patients (with 147 visits) who had remission/low/moderate DAS28-ESR scores (92% in remission and low disease activity) at baseline. Blood was collected and an ultrasound was performed three times in a year. Percentages of regulatory B cells and T regulatory type 1 cells and M2 macrophage numbers in the blood were examined. Plasma levels of 10 immunoregulatory cytokines IL-4, IL-5, IL-9, IL-10, IL-13, IL-27, IL-35, TGF-β1, sTNF-R1, and sTNF-R2 and monocyte chemotactic protein-1 (MCP-1) were assessed using ELISA assay. The correlations of cytokines and cells with different DAS28 scores and ultrasound grades were investigated, and cytokines and cells were compared between different categories of DAS28 scores and ultrasound grades. Plasma TGF-β1 levels were higher in the DAS28-ESR < 2.6 (remission) subgroup than in the DAS28-ESR ≥ 2.6 (nonremission) subgroup (p = 0.037). However, plasma TGF-β1 levels were higher in the high ultrasound grade subgroup than those in the low ultrasound grade subgroup (p = 0.007). The number of M2 macrophages was lower in the DAS28-MCP-1 < 2.2 subgroup than in the DAS28-MCP-1 ≥ 2.2 subgroup (p = 0.036). The levels of TGF-β1, sTNF-R2, IL-10, and IL-27 were higher in patients with high ultrasound grades than in those with low ultrasound grades. IL-27 was also higher in the nonremission DAS28-ESR subgroup than the remission one (p = 0.025). Moreover, sTNF-R1 levels in the 2011 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) remission subgroup were significantly lower than in the 2011 ACR/EULAR nonremission subgroup (p = 0.007). This trend was reflected in that lower sTNF-R1 levels correlated with low DAS28-MCP-1 scores (rho = 0.222, p = 0.007). We conclude that high plasma TGF-β1 levels indicate the DAS28-ESR remission (<2.6) subgroup and the high ultrasound grade subgroup. IL-27 probably connects the nonremission DAS28-ESR to high ultrasound grades. Low sTNF-R1 levels probably link low DAS28-MCP-1 scores with the 2011 ACR/EULAR remission subgroup. It suggests that incongruent immuno-inflammatory abnormalities exist between DAS28 scores and ultrasound grades, and are also dissimilar among various DAS28-formula categories. Therefore, this study may provide a basis for further research into individual cytokines and immunoregulatory cells behind each DAS28 formula and ultrasound grades/scores.

A case of Buerger's disease with vasculopathy and skin fibrosis requiring differential diagnosis from systemic sclerosis.

Buerger's disease is characterized by peripheral ischemia due to occlusion of small- and medium-sized arteries in the extremities. This report describes a case of Buerger's disease in a 51-year-old male who presented with findings resembling systemic sclerosis. The patient exhibited Raynaud's phenomenon in year X-3, which developed to skin hardening, nail avulsion, and ulceration of the right fingers in year X. Diagnostic testing showed positive microvasculopathy on nailfold videocapillaroscopy (NVC) and positive fibrosis on skin biopsy. Although the patient fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for systemic sclerosis, several findings in this case were atypical for systemic sclerosis, including left-right asymmetry in finger involvement, nail loss, and negative autoantibody tests. Contrast-enhanced computed tomography showed poor perfusion of the right ulnar artery, and a heavy smoking history was established in the patient case. Therefore, based on Shionoya's criteria, he was diagnosed with a case of Buerger's disease confined to the upper extremity. Smoking cessation and vasodilator therapy resulted in the prompt resolution of ischemic symptoms, skin hardening, and ulcerations. Furthermore, NVC abnormalities improved, and ulnar artery occlusion showed reperfusion on repeat testing. The present case suggests that hypoxemia-driven microvasculopathy may contribute to vascular occlusion and skin fibrosis observed in this atypical presentation.

Questionnaire survey of the application of four objective indicators necessary for standardized diagnosis of Sjögren's syndrome

Objective: To investigate the current status and challenges of carrying out the four objective indicators which are necessary for the Sjögren's syndrome (SS) diagnosis in hospitals all over China. Methods: A questionnaire survey was conducted online by Questionstar from May to July 2023 among rheumatologists nationwide, to investigate whether unstimulated salivary flow (UWSF), Van Bijsterveld score (VBS), Schirmer test and labial gland focus score (FS) are carried out in their hospitals and the challenges that hinder their development. A cohort of patients with established SS was enrolled to verify the importance of the four objective indicators in diagnosing SS. Statistical analyses were performed using the chi-square test. Results: The questionnaire was completed by rheumatologists from 660 hospitals in 225 cities of 32 provinces, autonomous regions and municipalities all over China (one doctor from each hospital completed the questionnaire), of which 548 (83.0%) were tertiary care hospitals. The rate of carrying out the objective indicators in 660 hospitals was low: UWSF (290/660, 43.9%), FS (497/660, 75.3%) and VBS (393/660, 59.5%). The percentage of hospitals who consider it difficult to carry out UWSF, VBS, minor labial gland biopsy and Schirmer test was 92.6%(611/660), 69.4%(458/660), 59.8%(395/660) and 58.6%(387/660), respectively. All four objective indicators mentioned above could be carried out in only 139 (21.1%) hospitals. In 521 hospitals in which less than four objective indicators could be carried out, 23.2% (121/521) of rheumatologists selected clinical experience to diagnose SS. A total of 180 patients with SS diagnosed by perfecting all objective indices and meeting the 2016 the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria were included, 173 females (96%), aged (46.6±13.6) years, with the missed diagnosis rate was 17.8% (32/180) assuming their labial FS was unavailable. In 166 patients with established SS who met the classic 2002 AECG criteria, 160 females (96%), aged (47.0±13.6) years, the missed diagnosis rate was 52.4% (87/166) assuming their labial FS was unavailable; or 10.8% (18/166) assuming their UWSF was unavailable. SS diagnosis couldn't be estimated according to 2002 AECG criteria, assuming both labial FS and UWSF were unavailable in 156 (94.0%) patients with positive anti-SSA/Ro; or assuming either labial FS or UWSF was unavailable in 10 (6.0%) patients with negative anti-SSA/Ro. Conclusion: The application rates of four objective indicators necessary for SS diagnosis are low, the rate of carrying out labial gland biopsy should be increased, and the labial FS reports and UWSF test should be standardized.

Role of Ultrasound in Evaluation of Median and Ulnar Nerves in Rheumatoid Arthritis Patients in Correlation with Nerve Conduction Studies

Abstract Background Rheumatoid arthritis (RA) is a systemic inflammatory disease of unknown etiology characterized by the involvement of the joints. RA is also associated with vasculopathy, peripheral, autonomic, and entrapment neuropathy. The presence of peripheral neuropathy contributes significantly to the functional limitation in rheumatoid arthritis patients. Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy and consists of the compression of the median nerve as it courses through the carpal tunnel followed by the cubital tunnel syndrome which is the most common site for entrapment for the ulnar nerve. Aim of the Work to evaluate the role of ultrasound in evaluation of median and ulnar nerves in rheumatoid arthritis patients in correlation with nerve conduction studies. Patients and Methods to elucidate this aim, 25patients diagnosed with RA, diagnosed according to the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosis of RA. And 25 healthy control subjects (matched for age and body mass index) were enrolled. Results this study was carried on 25 patients, 9 (34.7%) of whom were females, and 17 (68%) were males. The mean age of the studied group was (38.96±6.24 years old). The mean disease duration was 7 ±1.13 years; the mean disease activity index was 4.28 ±0.15. All the studied cases suffer from needle sensation, 24 (96) had numbness and 17(68) had burning sensation. All the studied cases in patient group had distal weakness and negative sensory testing (light touch, pinprick, temperature and vibration). As regarding to extra-articular manifestations, the most common was dry mouth and dry eye in all the studied cases followed by itchy skin in 13(52) patient, then joints aches and subcutaneous nodules in 2(8) patient and purpura was seen in only a case. Conclusion US has the advantage of early detection of median and ulnar nerves neuropathy and can assess the morphology of median and ulnar nerves and its surroundings. US measurements of median and ulnar nerves have a comparable diagnostic value as a non- invasive and an alternative modality for the evaluation of carpal tunnel syndrome and cubital tunnel syndrome.

Salivary Gland Scintigraphy in Sjögren's Syndrome: A Retrospective Study of Diagnostic Accuracy and Correlation With Histological and Immunological Biomarkers.

Introduction Sicca syndrome, characterized by xerophthalmia and xerostomia, is associated with various autoimmune and non-autoimmune conditions, posing diagnostic challenges. Sjögren's syndrome (SS) is the most prevalent systemic autoimmune disease linked to sicca symptoms. This study evaluates the diagnostic accuracy of salivary gland scintigraphy (SGS) in distinguishing SS from non-Sjögren's sicca conditions, alongside other diagnostic tests. Methods A retrospective analysis was conducted at Hospital Universitario La Paz from December 2019 to March 2023, including 142 patients diagnosed with sicca syndrome. Correlations between qualitative and quantitative SGS data (GE Healthcare, Chicago, Illinois) and multiparametric sicca evaluations were assessed. Results Among the 142 patients, 84 (59.15%) were classified as having SS, with 55 (65.48%) seropositive for anti-Ro antibodies. Abnormal SGS results were found in 135 (95.07%) patients. Qualitative SGS categorized seven (4.93%) as mild, 53 (37.32%) as moderate, 50 (35.21%) as severe, and 21 (14.79%) as functionally annulled. Moderate or worse impairment had a sensitivity of 0.88 and a specificity of 0.17. Functional annulment had a sensitivity of 0.17 and a specificity of 0.97. Quantitative SGS using ejection fraction thresholds of ≤30% and ≤20% had sensitivities of 0.35 and 0.18 and specificities of 0.84 and 0.94, respectively. Quantitative SGS metrics correlated with unstimulated whole salivary flow (WUSF; 0.243; p=0.003) and inversely with lymphocytic infiltration (-0.281; p=0.001). The 2016 American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) classification criteria for Sjögren's syndrome demonstrated an area under the curve (AUC) of 0.932, which improved to 0.951 with the inclusion of SGS parameters. Conclusions SGS is a significant diagnostic tool in the multiparametric evaluation of sicca syndrome, showing strong correlations with histological and immunological markers. Its integration into diagnostic criteria enhances the differentiation between SS and non-Sjögren's sicca conditions, suggesting its potential inclusion in future classification frameworks.

Unraveling the Clinical Features and Outcomes of IgG4-Related Ophthalmic Disease.

Background/Objectives: IgG4-related ophthalmic disease (IgG4-ROD), characterized by lymphoplasmacytic infiltration, fibrosis, and elevated IgG4 levels, presents diagnostic challenges while offering insights into immune-mediated inflammatory disorders. The aim of this study was to comprehensively examine the clinical features and outcomes of IgG4-ROD. Materials and Methods: A retrospective study was conducted on 33 patients diagnosed with IgG4-ROD, fulfilling the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria. The demographic characteristics of the IgG4-ROD patients were compared with those of 37 patients diagnosed with IgG4-related disease (IgG4-RD) in departments other than ophthalmology (IgG4-nonROD) at the same hospital during the same period. The patients diagnosed with IgG4-ROD were initially treated with glucocorticosteroid (GCS) monotherapy, GCS combined with steroid-sparing agents (SSAs; mycophenolate mofetil, azathioprine, hydroxychloroquine), biologic agent (rituximab) monotherapy, or watchful waiting. The primary outcome was the assessed treatment response at 6 months, and the secondary outcome was the evaluation of recurrence at 1 year after initial treatment. A response was evaluated as the absence of ocular signs and symptoms, either clinically or radiologically. Results: Eyelid swelling (17 patients, 51.5%) was the most common symptom, and lacrimal gland (17 patients, 51.5%) was the most frequent site of involvement. The response rate for GCS monotherapy was 33.3% (3 out of 9 patients), while the response rate for GCS combined with SSA was 60.0% (9 out of 15 patients). The lacrimal gland group demonstrated a significantly higher treatment response compared to the non-lacrimal gland group (66.7% vs. 20.0%, p = 0.013), and the combination of GCS and SSA resulted in a significantly higher treatment response than the GCS monotherapy (77.8% vs. 33.3%, p = 0.045). The group including hydroxychloroquine (HCQ), which comprised 5 out of 33 patients (15.2%), showed no recurrence at 1 year. Conclusions: The combination therapy of GCS and SSA for IgG4-ROD can be considered an effective treatment approach and HCQ could be considered as a potential adjunctive therapy for IgG4-ROD.

Application of Immunoblot assay (ANA 23 Profile) for detection of autoantibodies in systemic sclerosis

Objective: Examine the clinical and subclinical characteristics and the rate of autoantibodies in systemic sclerosis (SSc) using Immunoblot technique (ANA 23 Profile). Research subjects and methods: Cross-sectional descriptive study of 57 patients diagnosed with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria and were tested for autoantibodies using the Immunoblot technique (ANA 23 Profile) at the National Hospital of Dermatology and Venereology. Results: Among 57 SSc patients, the female/male ratio was 5/1. The average age of disease onset was 49.3±13.3, with 57.9% of patients over the age of 50. Diffuse SSc was the most common (70%). Raynaud was the most common symptom (98.2%), followed by gastroesophageal reflux (86%), and pulmonary fibrosis (70.2%). Heart and kidney damage were less frequently observed, accounting for 15.8 and 5.3%, respectively. Positive rates of the three major antibodies in SSc included anti-Scl-70 antibody (63.2%), anti-centromere antibody (17.5%), and anti-RNA polymer III antibody (12.3%), respectively. Scl-70 autoantibodies were mainly found in diffuse skin types (91.7%) while CENP autoantibodies were mainly found in localised skin types (90%) with p&lt;0.05. RNAP III antibodies were mainly found in diffuse skin types (85.7%), however, this difference was not statistically significant (p&gt;0.05). Conclusion: The presence of Scl-70 was related to the diffuse skin types while the presence of CENP was related to localised skin types.

A Clinical Picture of Unselected Patients with Systemic Lupus Erythematosus in a Tertiary Hungarian Center-A Spectrum Ranging from Pure Lupus to Overlap Syndromes.

Introduction: Systemic lupus erythematosus (SLE) is a multidimensional disease; however, the association of another systemic autoimmune disease further complicates its clinical presentation. Aim: We decided to investigate whether the association of overlap syndromes is linked with a different clinical picture compared to pure lupus and whether this association changes the sensitivity of the following commonly used criteria: the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR), the ACR-1997 and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria. Method: We performed a retrospective observational study among 382 patients afflicted with lupus: we measured as much of the full clinical and laboratory picture as possible in an unselected cohort. The diagnosis of SLE and other systemic autoimmune diseases was established by the rheumatologist in routine care and then the authors compared the characteristics of patients with pure lupus and those with overlapping pathologies. The diagnosis rates were compared to those that were determined based on the three classification criteria in order to identify various sensitivities and whether the existence of an overlap affects their rates. The fulfillment of each set of criteria was calculated using an Excel-based automatic calculation. Results: Among the patients, the ACR 1997's sensitivity was 81.2% (310 patients), and the SLICC 2012 criteria achieved 94.5% sensitivity (361 patients). The 2019 EULAR/ACR classification criteria resulted in a slightly lower sensitivity (90.3%-345 patients) when compared to the original publication (96%) due to the lower sensitivity of our anti-nuclear antibody (ANA) test (measured via enzyme-linked immunosorbent assay (ELISA)). Nearly all ANA-negative (21/22-95%) patients showed a positive lupus-associated antibody test. The proportion of ANA-negative cases showed no significant difference among pure and overlap patients. No significant difference was found between patients with overlap (138 patients-36%) and pure SLE (244 patients-64%) through the use of these criteria, with the exception of the SLICC criteria (ACR: 80.4% vs. 81.6%; SLICC: 97.4% vs. 92.6%, p = 0.035; EULAR/ACR 2019: 91.4% vs. 89.6%). Patients with an overlap syndrome were significantly older (55 vs. 50 years, p = 0.001), more likely to suffer from interstitial lung disease (ILD: 20% vs. 11%, p = 0.0343) and less frequently showed class III/IV lupus nephritis (7% vs. 14%, p = 0.029) when compared with their pure lupus counterparts. Conclusion: All investigated criteria regarding sensitivity were similar to the original publication's findings. The sensitivity of the EULAR/ACR 2019 classification criterion in cases with overlap syndrome proved excellent, with results very similar to patients afflicted with pure SLE. In the presence of an overlap syndrome, we found significantly fewer patients with lupus nephritis III/IV but no differences in other typical lupus organ manifestation beyond the kidney, whereas we found a higher proportion of ILD in patients with an overlap, indicating that the presence of an overlap syndrome significantly influences the observed clinical picture in real-world conditions.

Significance of High-Frequency Sonography for the Subclinical Progression of Rheumatoid Arthritis.

This study aims to investigate the use of high-frequency sonography as a tool for detecting inflammatory and destructive changes in the hand and foot joints of patients with early and long-term RA. This study employs a prospective cohort design involving 162 patients diagnosed with Rheumatoid arthritis (RA) who meet the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria. Patients were divided into two groups based on disease duration: Group 1 (n = 74) included patients with a disease duration of up to 2 years, or early РА (ERA;), Group 2 (n = 88) consisted of patients with a disease duration exceeding 2 years, or long-term persistent РА (LtRA). All patients underwent a clinical assessment of their joints, as well as radiography and arthrosonography, at the beginning of the study and again at 6 and 12 months later. In the general group of patients, ultrasound examination revealed signs of synovitis in the joints of the hands more frequently (66%) compared with clinical examination (56% by a number of swollen joints [NSJ] and 55% by a number of painful joints [NPJ], P < .01). After 6 months of treatment, 12% of the patients achieved full US remission and 24% achieved partial US remission. Within the scope of comprehensive RA diagnostics, arthrosonography of the joints of the hands and feet, utilizing a combination of greyscale and power Doppler, may surpass radiography in detecting early RA. This method allows for a more accurate assessment of disease activity and progression rates.

Sex-Specific Differences in Cytokine Production Capacity in Patients with Gout Compared to Controls

Gout, an inflammatory disease orchestrated by interleukin-1β activation and release, is more prevalent in men. The clinical profiles of patients with gout report differences by sex. This study aims to investigate sex-specific cytokine profiles in circulation and in stimulated peripheral blood mononuclear cells (PBMCs) of patients with gout and controls. Participants included in the gout group met the criteria of the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR). The control group included individuals with varying levels of serum urate and absence of gout. PBMCs were stimulated in vitro for 24 h with various TLR ligands. Cytokines were determined in culture supernatants and plasma. Plasma IL-1Ra and high-sensitivity C-reactive protein (hsCRP) were higher in men with gout compared to men without gout whereas no significant differences in circulating cytokines were observed in women. PBMCs of patients with gout showed higher cytokine production of IL-1β, IL-1Ra, and TNF-α following 24 h stimulation, predominantly observed in women. We identified sex-specific cytokine production in gout in response to in vitro stimulation. While men with gout had higher levels of circulating cytokines, stimulated PBMCs of women with gout show an enhanced capacity for cytokine production. These data may suggest potentially different regulatory mechanisms of inflammation in men and women with gout.

A Deep Learning Approach to Detect Rheumatoid Arthritis Using Medical Images and Blood Samples

Rheumatoid arthritis (RA) is a long-term autoimmune disease that mainly affects the joints. It happens when the immune system, which should aid in defending the body against illness and infection, unintentionally targets healthy tissues. This may result in joint pain, edema, stiffness, and loss of function. RA postures are a noteworthy clinical challenge due to their inveterate nature and potential for joint deformation and systemic complications. Conventional demonstrative strategies frequently drop briefly in giving exact and opportune discovery, requiring imaginative approaches. In this way of thinking, the developed novel system coordinates profound learning strategies with multi-modal information, counting blood biomarkers and restorative pictures of influenced joints of the hand, to revolutionize RA discovery and seriousness assessment. By leveraging a comprehensive dataset and considering indication length, the proposed approach outperforms the confinements of existing strategies, advertising prevalent symptomatic precision and early discovery capabilities. Through fastidious assessment, the system illustrates the diminishment of wrong positives and untrue negatives, hence progressing persistent results and diminishing long-term joint damage. Furthermore, this novel system contributes to progressing the understanding of RA pathophysiology and treatment viability by cultivating logical advances through the development of a comprehensive dataset. As part of the multi-modal framework integration process, the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) 2010 criteria are taken into account, and the system is improved by drawing on existing systems or discovered techniques. Specifically, the investigation was done on automating RA seriousness assessment using Profound Learning computations associated with hand X-ray pictures. Notwithstanding these advances, difficulties persist in accurately identifying early indications of RA from common variations or other joint disorders, as well as in identifying subtle joint deviations from the norm. To improve demonstration accuracy and advance in the comprehension of care outcomes in Rheumatoid Joint pain determination and severity evaluation, machine learning methodologies and multi-modal information integration were used.

The value of parotid sialography in the diagnosis and staging of Sjogren's syndrome

Background/purpose2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) primary Sjögren's syndrome (SS) diagnostic criteria did not incorporate radiographic examination while staging SS according to salivary gland imaging and serological autoantibody tests was not discussed. The aim is to study the value of parotid sialography for diagnosing SS, and to initially explore the method of staging SS based on the results of imaging and serological autoantibody tests. Materials and methods287 patients’ clinical records were included. The sensitivity and specificity of parotid sialography in the diagnosis of SS were investigated. SS patients were categorized into early stage (autoantibody positive, imaging does not support SS), active stage (autoantibody positive, imaging supports SS), and quiescent stage (autoantibody negative, imaging supports SS), clinical characteristics of different stages were compared. ResultsThe sensitivity of parotid sialography for the diagnosis of SS was 82.6%, the specificity was 71.5%. 10-minute USFR of the patients in the active stage (0.18 ± 0.38 ml/10min) was significantly lower than that of early stage (0.34 ± 0.47 ml/10min) and quiescent stage (0.54 ± 0.52 ml/10min), P = 0.010, and the rate of confirmed SS was significantly higher in the active stage (82.9%) than that in the early stage (44.4%) and the quiescent stages (14.8%), P < 0.001. ConclusionParotid sialography remains valuable in the diagnosis of SS. Performing imaging and serological autoantibody tests before lip gland biopsy may reduce invasive examinations for patients without significantly increasing the rate of missed diagnosis. According to imaging and serological autoantibody tests, SS can be categorized into early, active, and quiescent stages.

Factors associated with severe infection in rheumatoid arthritis patients: lessons learned from the COVID-19 pandemic.

This aimed to identify the factors associated with severe/critical coronavirus disease 2019 (COVID-19) infection in rheumatoid arthritis (RA) patients. Two-hundred RA patients diagnosed according to the American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) classification criteria with proven COVID-19 infection were recruited and categorized according to the world health organization (WHO) COVID-19 severity grading into 2 groups: patients with mild/moderate COVID-19 (n = 164) and patients with severe/critical COVID-19 (n = 36). Comparison between both groups was done to identify the risk factors associated with severe/critical infection. Incidence of RA disease activity flare defined as increase in clinical disease activity index (CDAI) more than 10 points following infection was calculated. Multivariate analysis identified history of previous serious infection, age > 60years, and diabetes as factors positively associated, whereas COVID-19 vaccination was negatively associated with severe/critical infection. Following COVID-19 infection, the number of patients with severe/critical COVID-19 who had high RA disease activity and the incidence of flares was significantly higher in comparison to patients with mild/moderate COVID-19 (P < 0.001 and 0.003; respectively). Age > 60years, diabetes, and history of previous serious infections are risk factors for severe/critical COVID-19, while vaccination has a protective role in RA patients. Infection particularly when severe is associated with risk of disease flare.

Application of the 2022 ACR/EULAR criteria to Chinese patients with previously diagnosed eosinophilic granulomatosis with polyangiitis: The concordance rate between the 2022 ACR/EULAR criteria and the 1990 criteria for EGPA in China.

This study aimed to investigate the concordance rate between the 2022 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria and the 1990 criteria for eosinophilic granulomatosis with polyangiitis (EGPA) in Chinese patients. 68 patients were diagnosed with EGPA according to the 1990 ACR criteria in the Department of Respiratory and Critical Care Medicine, Beijing Chao-yang Hospital Department, from May 2005 to December 2022, and those patients were reclassified using the 2022 ACR/EULAR criteria for EGPA. Among the 68 patients, 88.2% (60 cases) met the 2022 ACR/EULAR criteria for EGPA, and 11.8% (8 cases) patients failed to meet the new criteria. Only 66.2% of the patients were detected blood eosinophil count ≥ 1 × 109/L, while more patients (77.9%) met the item blood eosinophil count > 10% in the 1990 criteria. The report rate of nasal polyps was 13.2% in these patients. The concordance rate between the 2022 ACR/EULAR criteria and the 1990 criteria for EGPA was 88.2%. Compared with the 1990 criteria, the 2022 ACR/EULAR put more weighting on the blood eosinophil count and nasal polyps, while the diagnostic rate of them is low in Chinese patients. Thus improving the positive rate is important and routinely screening for nasal polyps should be performed in the future.

Frequency of EMG abnormalities in idiopathic inflammatory myopathies under the EULAR/ACR classification criteria.

The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for idiopathic inflammatory myopathies (IIM) have been widely used in recent times. However, no studies have focused on electromyography (EMG) findings of IIM, considering the criteria. This study aimed to elucidate the frequency of EMG abnormalities, particularly fibrillation potentials and positive sharp waves (Fib/PSW), the most objective EMG findings of IIM. Clinical and EMG records of adult patients who were clinically diagnosed with polymyositis (PM), dermatomyositis (DM), amyopathic DM (ADM), or inclusion body myositis (IBM) were retrospectively reviewed and classified according to the EULAR/ACR classification criteria. The frequency of Fib/PSW in EMG was investigated in the recruited cases. Seventy-nine patients with clinically diagnosed IIM (44 with PM, 17 with DM, 7 with ADM, and 11 with IBM) were recruited. After classification using EULAR/ACR, 75 satisfied definite or probable IIM (61 and 14, respectively), and the frequency of Fib/PSW in this group was 95%. Furthermore, the remaining 4 patients with insufficient IIM probability also showed Fib/PSW. Fib/PSW may also be seen in cases with insufficient IIM probability not satisfying the criteria. EMG may help detect muscle involvement in these cases through Fib/PSW.