ACR Criteria Research Articles

Prevalence of FMS Diagnosis According to ACR 2016 Revised Criteria in a Pain Therapy Centre in Italy: Observational Study.

Background and Objectives: Fibromyalgia syndrome (FMS) is a multifaceted disease with a strong preference for the female sex. It is characterised by chronic widespread pain, sleep-wake disorders, fatigue, cognitive disturbances, and several other somatic symptoms. Materials and Methods: In this prospective observational study, we analysed data regarding 302 patients who were referred to our pain centre for a first clinical assessment evaluation and were then inspected for the physician-based 2016 revision of the ACR diagnostic criteria for FMS, regardless of the final diagnosis previously made by the pain therapist. Results: Among the 280 patients who adhered to the 2016 ACR questionnaire, 20.3% displayed positive criteria for FMS diagnosis. The level of agreement between the FMS discharge diagnosis made by the pain clinician and the ACR 2016 criteria-positivity was moderate (kappa = 0.599, with moderate agreement set at a kappa value of 0.6). Only four patients (1.7%) diagnosed as suffering from FMS at discharge did not satisfy the minimal 2016 ACR diagnostic criteria. Conclusions: This prospective observational study confirmed the diagnostic challenge with FMS, as demonstrated by the moderate grade of agreement between the FMS diagnosis at discharge and the positivity for 2016 ACR criteria. In our opinion, the use of widely accepted diagnostic guidelines should be implemented in clinical scenarios and should become a common language among clinicians who evaluate and treat patients reporting widespread pain and FMS-suggestive symptoms. Further methodologically stronger studies will be necessary to validate our observation.

Mitochondrial structural alterations in fibromyalgia: a pilot electron microscopy study.

The pathogenesis of fibromyalgia (FM), characterised by chronic widespread pain and fatigue, remains notoriously elusive, hampering attempts to develop disease modifying treatments. Mitochondria are the headquarters of cellular energy metabolism, and their malfunction has been proposed to contribute to both FM and chronic fatigue. Thus, the aim of the current pilot study, was to detect structural changes in mitochondria of peripheral blood mononuclear cells (PBMCs) of FM patients, using transmission electron microscopy (TEM). To detect structural mitochondrial alterations in FM, we analysed PBMCs from seven patients and seven healthy controls, using TEM. Patients were recruited from a specialised Fibromyalgia Clinic at a tertiary medical centre. After providing informed consent, participants completed questionnaires including the widespread pain index (WPI), symptoms severity score (SSS), fibromyalgia impact questionnaire (FIQ), beck depression inventory (BDI), and visual analogue scale (VAS), to verify a diagnosis of FM according to ACR criteria. Subsequently, blood samples were drawn and PBMCs were collected for EM analysis. TEM analysis of PBMCs showed several distinct mitochondrial cristae patterns, including total loss of cristae in FM patients. The number of mitochondria with intact cristae morphology was reduced in FM patients and the percentage of mitochondria that completely lacked cristae was increased. These results correlated with the WPI severity. Moreover, in the FM patient samples we observed a high percentage of cells containing electron dense aggregates, which are possibly ribosome aggregates. Cristae loss and possible ribosome aggregation were intercorrelated, and thus may represent reactions to a shared cellular stress condition. The changes in mitochondrial morphology suggest that mitochondrial dysfunction, resulting in inefficient oxidative phosphorylation and ATP production, metabolic and redox disorders, and increased reactive oxygen species (ROS) levels, may play a pathogenetic role in FM. We describe novel morphological changes in mitochondria of FM patients, including loss of mitochondrial cristae. While these observations cannot determine whether the changes are pathogenetic or represent an epiphenomenon, they highlight the possibility that mitochondrial malfunction may play a causative role in the cascade of events leading to chronic pain and fatigue in FM. Moreover, the results offer the possibility of utilising changes in mitochondrial morphology as an objective biomarker in FM. Further understanding the connection between FM and dysfunction of mitochondria physiology, may assist in developing both novel diagnostic tools as well as specific treatments for FM, such as approaches to improve/strengthen mitochondria function.

Bone mineral density in lower thoracic vertebra for osteoporosis diagnosis in older adults during CT lung cancer screening

BackgroundQuantitative computed tomography (QCT)-based lumbar bone mineral density (LBMD) has been used to diagnose osteoporosis. This study explored the value of lower thoracic BMD (TBMD) in diagnosing osteoporosis in older adults during CT lung cancer screening.MethodsThis study included 751 subjects who underwent QCT scans with both LBMD and TBMD. 141 of them was selected for a validation. Osteoporosis was diagnosed based on LBMD using the ACR criteria (gold standard). TBMD thresholds were obtained using receiver operating characteristic curve. TBMD was also translated into LBMD (TTBMD) and osteoporosis was defined based on TTBMD using ACR criteria. The performance of TBMD and TTBMD in identifying osteoporosis was determined by Kappa test. The associations between TBMD- and TTBMD-based osteoporosis and fracture were tested in 227 subjects with followed up status of spine fracture.ResultsThe performance of TBMD in identifying osteoporosis was low (kappa = 0.66) if using the ACR criteria. Two thresholds of TBMD for identifying osteopenia (128 mg/cm3) and osteoporosis (91 mg/cm3) were obtained with areas under the curve of 0.97 and 0.99, respectively. The performance of the identification of osteoporosis/osteopenia using the two thresholds or TTBMD both had good agreement with the gold standard (kappa = 0.78, 0.86). Similar results were observed in validation population. Osteoporosis identified using the thresholds (adjusted hazard ratio (HR) = 18.72, 95% confidence interval (CI): 5.13–68.36) or TTBMD (adjusted HR = 10.28, 95% CI: 4.22–25.08) were also associated with fractures.ConclusionCalculating the threshold of TBMD or normalizing TBMD to LBMD are both useful in identifying osteoporosis in older adults during CT lung cancer screening.

Mezagitamab in systemic lupus erythematosus: clinical and mechanistic findings of CD38 inhibition in an autoimmune disease

ObjectiveTo evaluate safety and mechanism of action of mezagitamab (TAK-079), an anti-CD38 monoclonal antibody, in patients with moderate to severe systemic lupus erythematosus (SLE).MethodsA phase 1b double-blind, placebo-controlled, multicentre study...

Sleep disorders and other medical and socio-demographic factors in systemic scleroderma.

We aimed to investigate sleep disorders in patients with systemic scleroderma (SSc) and its relationship with socio-demographic and medical factors and to provide a suitable solution to better control the disease and improve the quality of life in these patients. This cross-sectional study evaluated SSc patients seen at a rheumatology clinic from September 1, 2022, through April 1, 2023.The patients were examined by the main investigator of the project and entered the study after taking the medical history and meeting the criteria of ACR 2013 Classification Criteria. Pittsburgh Sleep Quality Index (PSQI), Insomnia Severity Index (ISI), Epworth Sleepiness Scale (ESS) and STOP-Bang Questionnaire were employed to investigate sleep disorders. A total of 103 patients were included in the study. The average age of the patients was 48.42 ± 12.4 years. PSQI showed lower quality of sleep scores among SSc (68% of patients), which was significantly related to the degree of skin stiffness in patients, telangiectasia, interstitial lung disease (ILD) in computed tomography (CT) scan, patient age, duration of the disease, and pulmonary artery pressure (PAP). STOP-Bang Questionnaire revealed that obstructive sleep apnea (OSA) was significantly associated with telangiectasia, ILD, patient age, disease onset age, disease duration, body mass index and PAP. Insomnia had a statistically significant relationship with telangiectasia, ILD and patient age. Drowsiness during daily activities was not significantly related to any of the individual variables and disease-related variables. Sleep disorders are common in patients with systemic scleroderma. Telangiectasia, ILD and patient age were related to all sleep quality disorders and respiratory apnea and insomnia. Furthermore, the amount of skin involvement significantly causes disturbances in the quality of sleep of patients, where in the group with diffuse skin stiffness, 80% of patients exhibited disturbances in the quality of sleep. Therefore, paying attention to sleep health can be an effective factor in improving the quality of life of patients with SSc.

Fibromyalgia: recent concepts and treatments

Fibromyalgia is a chronic pain syndrome characterized by widespread pain often accompanied by symptoms that compromise quality of life, such as fatigue, sleep disorders, cognitive dysfunction, mood disorders. The pathology affects millions of people around the world and is still often overlooked and misunderstood. Although its pathophysiology is not fully understood, it is believed that Central Sensitization plays a preponderant role in the genesis of the disease. In addition, several studies demonstrate genetic polymorphisms in genes related to pain syndromes, pain thresholds, psychological disorders, and others pathologies frequently associated with Fibromyalgia. Different diagnostics criteria have been proposed to define the disease, however, the 2010 ACR criteria revised in 2016 is considered the most suitable. Functional imaging exams were of great importance in determining the pathophysiological mechanisms and so, the therapeutic options. A variety of randomized controlled studies and systematic reviews demonstrated the therapeutic efficacy of pharmacological and non-pharmacological approach for Fibromyalgia. This article aims to provide a current view of Fibromyalgia concepts, from possible pathophysiological mechanisms to pharmacological and non-pharmacological therapeutics.

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.

Cross-sectional association between social and demographic factors and disease activity in rheumatoid arthritis

BackgroundThis study aimed to assess the association between social factors, demographic parameters, and disease activity among rheumatoid arthritis (RA) patients.MethodsThe University of Pittsburgh Rheumatoid Arthritis Comparative Effectiveness Research (RACER) registry was used for this study and included patients meeting 1987 ACR criteria for RA enrolled between 2010–2015. The registry collected clinical and laboratory data at each visit, permitting the calculation of disease activity measures that included Disease Activity 28-C Reactive Protein (DAS28-CRP). The current study was conducted as a cross-sectional study in which baseline data were used to construct multiple logistic regression models assessing the relationship between disease activity measures (DAS28-CRP), functional capacity (health assessment questionnaire (HAQ)), selected demographic and social factors (occupation, education, income, marital status, race, gender, age, and BMI), and clinical/laboratory variables.ResultsThe analyses included 729 patients with baseline DAS28-CRP and social/demographic data. The mean age at enrollment was 59.5 (Standard Deviation (SD) = 12.7) years, 78% were female, and the median RA disease duration was 9.8 (Interquartile Range (IQR): 3.7, 19.1) years. We dichotomized the DAS28-CRP score and defined scores above or below 3.1 as high versus low RA disease activity. Most patients with high RA disease activity (N = 326, 45%) had less than a college degree (70%), were not working/retired/disabled (71%), and had an annual income under $50 K (55%). We found that higher body mass index (BMI) (Odds Ratio (OR) = 1.04, 95% CI: 1.01—1.08), longer disease duration (> 2 and < 10 years versus ≤ 2 years of disease) (OR = 0.45, 95% CI: 0.25—0.78), and being retired (OR = 1.74, 95% CI: 1.02—2.98) were associated with RA disease activity.ConclusionIncreased RA activity may be associated with various social factors, potentially leading to more severe and debilitating disease outcomes. These findings provide evidence to support efforts to monitor disparities and achieve health equity in RA.

Clinical profiles in pediatric systemic lupus erythematosus: a retrospective study

Aims: This study aimed to analyse clinical and laboratory findings, prognosis, and survival of systemic lupus erythematosus (SLE) patients, differentiating according to gender, pubertal status, and renal involvement. Methods: Ninety-six pediatric SLE patients, diagnosed using ACR criteria, were retrospectively analyzed. Inclusion criteria comprised age under 18, meeting at least four ACR criteria, and six months of monitoring. Data encompassed demographics, symptoms, diagnosis, organ involvement, autoantibodies, treatment, prognosis, and survival. Categorization was based on gender and pubertal status. Renal biopsies followed WHO-ISN classification, with asymptomatic findings termed “silent lupus nephritis.” Biopsied patients were divided into proliferative and non-proliferative lupus nephritis categories, excluding irreversible damage cases. Outcomes studied included remission, relapse, end-stage renal failure, and mortality. Results: Among 96 participants, females constituted 82.3%, males 17.7%, resulting in a female-to-male ratio of 4.6:1. Mean age at diagnosis was 11.9 years, with 37 prepubertal (38.5%) and 59 pubertal (61.5%) cases. Oral-nasal ulcers (p=0.01) were more prevalent in males related to system involvement. Nephrotic syndrome prevalence increased from 21.6% in prepubertal to 44.1% in pubertal cases (p=0.025). Positive Anticardiolipin IgM antibodies decreased from 56.2% in prepubertal to 25.9% in pubertal cases (p=0.047). Type IV lupus nephritis was predominant, followed by Type II, in prepubertal and pubertal groups and both genders. Proliferative lupus nephritis showed higher rates of renal involvement (95.7% vs. 65.6%), nephrotic syndrome (46.8% vs. 21.9%), proteinuria (89.4% vs. 62.5%), hematuria (57.4% vs. 28.1%), elevated creatinine (43.5% vs. 9.7%), and low albumin (67.4% vs. 23.3%). Cases with proliferative lupus nephritis had higher neuropsychiatric involvement (36.2% vs. 12.5%), seizures (25.5% vs. 3.1%, p=0.008), and increased hemolytic anemia rates (78.7% vs. 56.2%, p=0.033). Thirteen had silent lupus nephritis, revealing various types through biopsy. All reported deaths occurred within the first five years, resulting in stable 91% survival rates at 5, 10, and 15 years. Conclusion: This study provides insights into the clinical, prognostic, and survival characteristics of pediatric systemic lupus erythematosus (SLE), revealing notable patterns related to gender, pubertal development, and renal involvement. There is an association between proliferative lupus nephritis and renal involvement, nephrotic syndrome, and neuropsychiatric symptoms. Significantly, silent lupus nephritis highlights the complex renal implications, necessitating diligent surveillance for prompt intervention.

A retrospective analysis of the prevalence and impact of associated comorbidities on fibromyalgia outcomes in a tertiary care center.

This retrospective study was designed to analyze the prevalence and impact of associated comorbidities on fibromyalgia (FM) outcomes (functionality, pain, depression levels) for patients who participated in an intensive multicomponent clinical program in a tertiary care center. Participants included a sample of 411 patients diagnosed with FM at a large tertiary medical center using the 2016 ACR criteria. Patients completed an intensive 2-day cognitive behavioral treatment (CBT) program, filled out the Fibromyalgia Impact Questionnaire Revised (FIQR), the Center for Epidemiologic Studies Depression Scale (CES-D), the Pain Catastrophizing Scale (PCS), and were followed for 6 months after treatment completion. T-tests were performed to analyze differences between the presence or absence of select comorbidities for the three outcomes at follow-up. Statistically significant comorbidities (p < 0.05) were used as predictors in multivariable logistic regression models. The FM associated comorbidities in this cohort that had significant impact on the measured outcome domains after treatment program completed were Obesity (FIQR p = 0.024), Hypothyroidism (CES-D p = 0.023, PCS p = 0.035), Gastroesophageal reflux disease GERD (PCS p < 0.001), Osteoarthritis (CES-D p = 0.047). Interestingly, Headache, the most frequent FM associated comorbidity in this cohort (33.6%), did not have a significant impact on the outcome domains at follow-up. Obesity (18.2%) was the only FM associated comorbidity significantly impacting all three outcome domains at follow-up. The present study suggests that addressing obesity may significantly impact outcomes in FM patients.

Attitudes toward and management of fibromyalgia: A national survey of Saudi Arab's medical practitioners.

Widespread pain is characteristic of fibromyalgia. Patients consult different specialities, and hence, diagnosis and treatment is often delayed. To evaluate and assess the attitudes and management of fibromyalgia among medical practitioners in Saudi Arabia a cross-sectional study was conducted through an online questionnaire in November 2021. Screening demographics included age, sex, place of work, speciality, year of graduation, and practical experience. Questions about fibromyalgia (general knowledge and management, ACR criteria 2010, essential symptoms, diagnosis, and treatment) were also part of the questionnaire. There were 103 respondents, out of which 88(85.4%) have heard about fibromyalgia and 21(20.4%) had managed cases of fibromyalgia, while 32(31.1%) knew about the 2010 ACR diagnostic criteria. Commonest reported characteristics of fibromyalgia were excessive fatigue 74(71.8%), widespread pain 71(68.9%), weakness 54(52.4%), and sleep disturbance 38(36.9%). About 76(73.8% reported it is a clinical diagnosis and 75(72.6%) reported having the knowledge about the treatment of fibromyalgia. It was concluded that 88(85.4%) practitioners know fibromyalgia. However, knowledge about the diagnosis and treatment of fibromyalgia was not satisfactory.

Screening of Cognitive Impairment in patients with Rheumatoid arthritis

AbstractBackgroundRheumatoid arthritis (RA) is a chronic inflammatory disease that affects 0.5‐1% of the population, mainly women and older adults. There is increasing evidence of the presence of cognitive impairment in patients with RA, with a prevalence of around 30%. Some underlying mechanisms involved could be inflammation within the brain, high cardiovascular comorbidity, and the use of immunosuppressive agents that can be neurotoxic with prolonged use, among others. The Montreal Cognitive Assessment (MoCA) is a validated tool for detecting cognitive impairment in our country. The objective of this study is to determine the frequency of cognitive impairment in patients with RA compared to a group of patients with osteoarthritis.MethodWe assessed adult patients diagnosed with RA (2010 ACR/EULAR criteria) and osteoarthritis (ACR criteria) according to selection criteria. Demographic and clinical data were collected. The MoCA test was administered to all patients considering the cut‐off value &lt;26 points to define the presence of cognitive impairment. The study was approved by the institutional ethics and research committee.ResultA total of 60 patients were included: 40 with RA (G1) and 20 with osteoarthritis (G2), the majority being women in both groups, mean age was 66.9 (SD 11.7) years and 71.4 (SD 10.6) years, respectively. There were no statistically significant differences in education, socioeconomic status, or vascular risk factors among groups. The frequency of cognitive impairment was significantly higher in G1 compared to G2: 42.5% (95% CI 28‐58.5) versus 15% (95% CI 4‐40)(p 0.026). Regarding MoCA domains, attention and memory were more affected in G1 compared to G2. In the multivariate analysis, the diagnosis of RA and older age were factors significantly associated with cognitive impairment.ConclusionIn our RA population, the frequency of cognitive impairment was 42.5%. RA diagnosis and older age were factors related to cognitive impairment. A substantial number of patients and the administration of a standard neuropsychological test will help us to validate these results.

Late Onset Systemic Lupus Erythematosus - Clinical and Autoantibody Profile and its Comparison with Young Onset Systemic Lupus Erythematosus.

The aim of the study was to determine the clinical features & autoantibody profile of patients having late onset Systemic Lupus Erythematosus (SLE) and to compare with young onset SLE due to its scarce data from India. All patients who fulfilled the 1997 ACR criteria for SLE were included. Late onset patients were >50 years of age and young onset were <50 years >18 years at the time of first SLE-related symptom. Clinical, laboratory, and autoantibody (ENA 25 & APLA) profiles were compared between the two groups using descriptive statistics and chi square test. Of the 305 patients, 69 had late onset (75.4% females). Mean age was 59.42±6.7 years (Late onset lupus) and 33.13±8.44 years (young onset lupus). The most common symptom was arthritis (60%) followed by oral ulceration (50%), fever (43%), and serositis (37.68%). Most common antibody was SSA/Ro60 (50%) and anti-SSA/Ro52 (46%). Interstitial lung disease (ILD) (14.5%), pancytopenia (13%) and diffuse alveolar haemorrhage (4.3%) were more frequent in late onset group. Statistically significant differences were found between two groups in terms of photosensitivity (p=0.009), malar rash (p=0.005), excessive hair loss (p=0.0006), Raynaud's phenomenon (p=0.001), lymphadenopathy (p=0.01), nephritis (p=0.0007), ILD (p=0.01), anti-dsDNA (p=0.005), anti-nucleosome (p=0.01), anti-Sm (p=0.007), Ribosomes P0 (p=0.0004). This study suggests that late onset SLE has distinct clinical and serological manifestations when compared with young onset SLE patients.

Evaluation of MicroRNA-451a Expression in Lupus Nephritis

The pathogenesis of Lupus Nephritis (LN) is still unclear, causing difficulties in diagnosis and therapeutic management. Recent studies have suggested that microRNAs (miRNAs) may play an important role in the pathogenesis of LN. However, the role of miRNA-451a in LN has not been widely studied and known, and there is controversy in the results of studies that have been done. This study aimed to analyze the expression of miRNA-451a in LN and lupus without nephritis and to evaluate its correlation with hemoglobin levels, leukocyte count, leukocyturia, and erythrocyturia. Samples from 45 lupus participants were collected, consisting of 22 with LN and 23 without nephritis. The expression of microRNA 451a was determined by reverse transcriptase real-time PCR. Renal disorder criteria based on ACR criteria. Statistical analysis using Mann-Whitney, Spearman correlation, ROC curve, and table 2X2. MiRNA-451a expression in LN (median 0.31; range 0.06-7.21) was lower than in lupus without nephritis (median 1.31; range 0.07-13.26) with a significant difference (p=0.041). The cut-off value for comparing the two groups was 0.7531 with an accuracy of 75%. The correlation between miRNA 451a expression and hemoglobin levels, blood leukocyte count, erythrocyturia, and leukocyturia showed no significant correlation (p &gt; 0.05). MicroRNA-451a expression in lupus nephritis was lower than in lupus without nephritis.

Frequency of fibromyalgia in a cohort of Turkish patients with lung cancer and its effect on pain, sleep quality, fatigue and quality of life.

This study aimed to examine the frequency of fibromyalgia (FM) in patients with lung cancer and evaluate its effect on patients' pain, sleep quality, fatigue, and quality of life parameters. The study was designed as a prospective cross-sectional and a total of 116 lung cancer patients were included. FM classification was made according to the 2016 ACR criteria. All patients were evaluated using a visual analog scale, fibromyalgia impact questionnaire, multidimensional assessment of fatigue, EuroQol 5D scale, Jenkins sleep scale, and Beck depression inventory. Patients were divided into FM-positive and negative groups according to the presence of FM. Demographic and clinical parameters were investigated between the groups. FM was detected in 14 (12.2%) patients. The mean age of the patients was 62.2 ± 8.4 years. There was a male predominance in 93 (80.2%) patients. No statistical difference was found between the groups in terms of body mass index, age, symptom duration, chemotherapy, and radiotherapy history. A statistically significant difference was found between FM positive and negative groups in FIQ, multidimensional assessment of fatigue, JSS, EQ-5D, visual analog scale, and Beck depression inventory scores. FM is seen more frequently in patients with lung cancer and has a negative effect on sleep, quality of life, fatigue, and mental functions. We think that physicians should not ignore the presence of FM when treating patients diagnosed with lung cancer.

SAT476 A Case of Thyroid Hemiagenesis

Abstract Disclosure: P. Kachhadia: None. S. Khan: None. S. Aldasouqi: None. A. Abu Limon: None. Introduction: Thyroid hemiagenesis is congenitally rare, in which one side of the thyroid does not develop. Hemiagenesis is more common with an absent left thyroid lobe, at a 4:1 ratio. Though prevalence is sited at 0.05%, actual incidence of hemiagenesis amongst the population is unknown as most patients are euthyroid and asymptomatic. We present a case of a patient who was referred to our clinic for unilateral thyroid enlargement on physical exam. Thyroid Ultrasound (US) was done which showed an absent left thyroid lobe along with enlarged right thyroid lobe and hypervascularity. There was also an incidental thyroid nodule noted on US. Case Presentation: Patient is a 38 year old female who presented to the clinic 4 years prior for enlarged thyroid nodule noted on ultrasound. With symptoms of dysphagia and a visibly enlarged gland, an US with labs was prompted by her primary physician. US showed absent left thyroid lobe and right lobe enlargement with hypervascularity and incidental subcentimeter nodule. Clinically and biochemically, the patient was euthyroid. Singular right lobe was verified on clinic real time ultrasound and on NM uptake scan. The 24 hour uptake was at 30.5%, with the upper limit of normal being 30%. With increased uptake and hypervascularity, thyroid stimulating immunoglobulin and thyrotropin receptor antibodies were checked and were not detected. It was thought that the right thyroid gland enlargement and hypervascularity were compensatory due to the left hemiagenesis. The thyroid nodule was monitored, and within 3 years of original imaging, it enlarged to 0.98 x 0.59 x 0.39 cm, with TI-RADS 5 assessment per ACR criteria. Patient chose to undergo thyroid biopsy which showed Bethesda III, Follicular Lesion of Undetermined Significance (FLUS). Microscopy showed follicular cells arranged in microfollicles and crowded groups with round, enlarged, irregular nuclei and overlapping nucleoli. Intranuclear chromatin clearing and intranuclear grooves are present. Background is hemorrhagic with minimal colloid. Molecular Thyroseq testing was at 5-10% chance of malignancy or noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP). Genetic markers were only positive for copy number alterations. Discussion: Thyroid Hemiagenesis, though rare, has been associated with other thyroid pathologies such as hypothyroidism, hyperthyroidism and thyroid malignancies. Genetic studies have not found a common background within cases reported. As these patients are more likely to incur additional thyroid pathologies, questions still remain as to the clinical management and outcome of such patients. Due to concerning cytological features for PTC, the patient was offered right thyroidectomy and will be looking to make a decision in the near future. Presentation Date: Saturday, June 17, 2023

Challenges and insights in managing difficult-to-treat rheumatoid arthritis: real-world clinical perspectives.

The treatment options for rheumatoid arthritis (RA) have expanded with the availability of biological and targeted synthetic disease-modifying anti-rheumatic drugs. Despite all these developments and treatments, an important group of patients remain symptomatic and have not achieved clinical remission. The terminology "difficult-to-treat" (D2T) has been developed to describe this group. This study aimed to determine the frequency of D2T RA among our patients according to the EULAR 2021 definition of D2T RA and to identify the differences in demographic and disease characteristics, contributing factors, and disease burden. The study included 302 consecutive patients diagnosed with RA according to the 2010 ACR criteria. These patients were categorised into the D2T and non-D2T RA groups. Risk factors independently associated with D2T RA were identified using logistic regression analysis. Of the 302 patients (mean age, 56.5 years, 80.1% female, 75% seropositive), 27 (8.9%) had D2T RA. Those with D2T RA had a lower age at diagnosis and longer disease duration and showed significantly higher rates of peripheral erosion, Sjögren's syndrome, extra-articular manifestations, and PtGA-PhGA discordance, together with high disease activity scores. Furthermore, the median number of comorbidities and concomitant fibromyalgia was significantly higher in the D2T RA group. In the multiple regression analysis, D2T RA was independently associated with higher HAQ-DI, RF levels, and concomitant fibromyalgia. D2T RA requires more intensive management, and patients with D2T RA have higher disease activity, poorer functional status, and quality of life than those without D2T RA.

Acute Hepatopancreatitis Revealing Systemic Lupus Erythematosus: About A Case

Digestive manifestations during systemic lupus erythematosus (SLE) are rarely reported in the literature, in particular, hepato-pancreatic manifestations. We report the case of a patient in whom SLE was revealed by acute hepato-pancreatitis with mixed acute hepatitis, acute pancreatitis fulfilling 2 diagnostic criteria (high lipasemia and Balthazar stage C pancreatitis on the abdominal CT scan). The diagnosis was retained on the ACR criteria. The evolution under corticosteroid therapy and resting of the digestive tract was favorable.

Comparative assessment of sensitivity and specificity of three variants of classification criteria for systemic lupus erythematosus in a cohort of Russian patients

The clinical and serologic heterogeneity of systemic lupus erythematosus (SLE) presents challenges for diagnosis, particularly in the earliest stages of the disease when there are insufficient signs to make a reliable diagnosis. To make a comparative assessment of sensitivity and specificity of various classification criteria of SLE on a cohort of patients of Nasonova Research Institute of Rheumatology. A total of 252 patients were included in the study; 152 (60%) of 252 patients had reliable SLE (mean age 36 [29.5-46] years, duration of disease 9 [3.4-19] years). Of 252 patients, 26 (11%) had PAPS (mean age 36.5 [31-42] years, duration of disease 4.6 [1-10.4] years). Systemic sclerosis was diagnosed in 74/252 (29%) patients, (mean age 51.5 [42-59] years, duration of disease 9 [5-16] years). The quality of the classification function of the criteria was assessed by ROC analysis. SLE was diagnosed in 131 (86%) of 152 patients using the American College of Rheumatology - ACR)-1997 criteria, in 145 (95%) using the The Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria, and in 144 (94.7%) using the European League Against Rheumatism (EULAR)/ACR 2019 criteria. ANF positivity was the least statistically significant of all signs in relation to the diagnosis of SLE. The area under the curve (AUC) for ANF≥1/160 titers was AUC 0.654 for the ACR-97 criteria, AUC 0.616 for the SLICC-12 SLE criteria, and AUC 0.609 for the 2019 EULAR/ACR criteria. ROC analysis of the relationship between the number of criteria/points and a reliable diagnosis of SLE revealed a high diagnostic accuracy - the AUC for all SLE criteria was greater than 0.940. In the ROC analysis of patients with SLE and PAFS, indicating the number of diagnostic criteria, sensitivity was 86% for ACR-1997, 95% for SLICC-2012, 95% for EULAR/ACR 2019, and specificity was 100, 62 and 62%, respectively. The classification criteria SLICC-2012 and EULAR/ACR 2019 are more sensitive for the diagnosis of SLE in the Russian population, and the criteria ACR-1997 are more specific. All three variants of the SLE classification criteria have sufficient sensitivity and specificity for their use in real clinical practice.

Validation of the Toronto Psoriatic Arthritis Screening 2 (TOPAS-2) questionnaire in an Argentinian population

IntroductionToPAS-2 is a questionnaire designed to detect cases of psoriatic arthritis (PsA) in patients with psoriasis (PsO). ObjectiveTo validate the ToPAS-2 questionnaire in patients with psoriasis in Argentina. Materials and methodsPatients ≥18 years old with a diagnosis of PsO. As a control group, patients diagnosed with PsA (CASPAR criteria) and osteoarthritis (OA) (ACR criteria) were included. All patients completed the ToPAS-2 questionnaire and were then evaluated by a rheumatologist. Blood tests were performed to determine Rheumatoid Factor (RF) and erythrocyte sedimentation rate (ERS). Radiographs of the hands, feet, panoramic view of the pelvis, Ferguson, and lumbar and cervical spine were performed. Statistical analysisDescriptive statistics. ROC curves for determination of sensitivity, specificity, and cut-off points of the questionnaire. A p<.05 was considered significant. Results126 patients were evaluated, 54.8% female with a mean age of 54.5 (±14.9) years. The mean schooling was 10.4 (±4.1) years. A total of 45.2% had a diagnosis of PsA, 38.9% of PsO, and 15.9% of OA. The ToPAS-2 result was X 8.4 (±3.6). The mean age according to the groups was 55.5 (±12.5), 48.1 (±15.3), and 67.1 (±11.3) years, with 40.4% female, 57.1%, and 90% respectively. The mean of the ToPAS-2 according to the different groups was 11 (±2.3), 6.8 (±3.0), and 4.6 (±1.9) (p<.01 between all groups) for APs, PsO, and OA, respectively. Of the 49 patients with PsO, 16 patients were diagnosed as PsA, after evaluation by the rheumatologist. The mean ToPAS-2 in these patients was significantly higher than those not diagnosed as PsA [10.1 (±1.8) vs 5.2 (±2)] (p<.001). In the ROC curve analysis, the area under the curve was .97 (95% CI .94–.97). A TOPAS cut-off value of 8.5 had a sensitivity of 88.9% and a specificity of 96% for the diagnosis of PsA. ConclusionThe ToPAS-2 questionnaire proved to be a valid, sensitive, and specific tool for the detection of PsA in patients with psoriasis.