Incidence and disease burden of autoimmune inflammatory rheumatic diseases after non-pharmaceutical interventions in the COVID-19 era: A nationwide observational study in Korea.

BACKGROUND: Infections are considered risk factors for autoimmune inflammatory rheumatic diseases (AIRDs), the incidence of which is considered to have been impacted by the COVID-19 pandemic. The impact of non-pharmaceutical interventions (NPIs) on the incidence of AIRDs and their associated health care services and medical expenses in Korea was investigated. METHODS: We included all AIRD cases reported between January 2016 and February 2021 based on the National Health Insurance Service data. We evaluated changes in incidence trends for each AIRD before and after NPI implementation (Feb 2020 to Feb 2021) using segmented regression analysis. Changes in health care utilization and medical costs for each AIRD before and after NPI implementation were also investigated. RESULTS: After NPI implementation, monthly incidence rates declined significantly by 0.205 per 1 000 000 (95% confidence interval [CI], -0.308 to -0.101, p < .001) in patients with systemic lupus erythematosus (SLE). No significant changes in the incidence of all AIRDs other than SLE were observed before and after implementation. Further, annual outpatient department visits per patient were lower during implementation for all diseases, except juvenile idiopathic arthritis (JIA). The prescription days per outpatient visit increased significantly during implementation for all diseases, except JIA and ankylosing spondylitis. During implementation, the total annual medical costs per patient tended to decrease for all diseases, except JIA and mixed connective tissue disease. CONCLUSION: Implementation of NPIs to contain the pandemic led to a reduction in the incidence of SLE and changed patterns of medical care utilization and treatment cost for most AIRDs.

Classification of rheumatoid arthritis: is it time to revise the criteria?

Classification criteria have been developed for rheumatoid arthritis (RA) and other rheumatic diseases in order to gather a homogeneous patient population for clinical studies and facilitate the timely implementation of therapeutic measures. Although classification criteria are not intended to be used for diagnosis, they are frequently used to support the diagnostic process in clinical practice, including clinical decision-making. The 2010 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria for RA are capable of identifying the majority of symptomatic patients with RA already in the earliest stages of the disease who are not yet showing radiographic changes. These patients will also profit from the early implementation of therapy with disease-modifying antirheumatic drugs (DMARDs). However, the risk of misclassification is higher as compared with the former 1987 ACR criteria, which were considerably less sensitive to the recognition of patients with early RA. Of note, the presence of rheumatoid factors (RFs) and anticitrullinated protein antibodies (ACPAs) has been attributed equal weight in the 2010 ACR/EULAR criteria and may contribute up to 50% of the score needed for being classified as RA. However, while ACPAs have been proven to be the most specific serological markers of RA, the specificity of RF is moderate, especially at lower titres. This may lead to the misclassification of RF-positive patients and, consequently, the unjustified implementation of DMARD therapy. Therefore, issues arise on how comprehensive the criteria should be and whether they should be updated and adapted to findings from the past two decades that might increase both their specificity and sensitivity.

Patient-perspective and feasibility of home finger-prick testing to complement and facilitate large-scale research in rheumatology.

BACKGROUND: During the COVID-19 pandemic, we developed a digital research platform to longitudinally investigate COVID-19-related outcomes in patients with rheumatic diseases and healthy controls. We used home finger-prick testing in order to collect serum samples remotely and increase the overall efficiency of the platform. The aim of the present study was to evaluate the success rate of the finger prick and patients' perspective towards the finger prick. METHODS: Serum samples were collected up to five times during follow-up, either via a venepuncture at the research institute or a finger prick from participants' home. Participants were asked to complete a digital evaluation questionnaire of the finger prick after their attempts. RESULTS: A total of 2135 patients and 899 controls performed at least one finger prick and were included in this study. The first finger prick was successfully done by 92% (95% CI: 90% to 93%) of patients, 94% (95% CI: 92% to 95%) of controls, 93% (95% CI: 92% to 94%) of all participants aged ≤70 years and 89% (95% CI: 86% to 92%) of all participants aged >70 years. Sex did not impact these success rates. Repeated failure occurred in 11/439 (0.8%) patients and 4/712 (0.6%) controls. Both patients and controls were less willing to perform a finger prick for individual healthcare compared with scientific research. CONCLUSION: The vast majority of participants, among which elderly and patients with rheumatic diseases, were able to successfully draw the required amount of blood for serological analyses. This shows that finger-prick testing is suitable for a high-throughput implementation to monitor patients remotely.

Risk perception, well-being, depression and anxiety in children and adolescents with rheumatic diseases during the COVID-19 pandemic - results from the prospective multicenter KICK-COVID study in Germany.

OBJECTIVE: To investigate the psychosocial burden in children and adolescents with juvenile rheumatic diseases during the COVID-19 pandemic. METHODS: As part of the multicentre observational KICK-COVID study linked to the National Pediatric Rheumatology Database, adolescents < 21 years and parents of children < 12 years with rheumatic diseases answered questions on perceptions of health risk (PHR) due to SARS-CoV2, stress, well-being (WHO-5) and symptoms of depression (PHQ-9) and anxiety (GAD-7). Data were collected at routine visits from June to December 2021 and assessed for association with demographic and clinical parameters, treatment and patient-reported outcomes by multivariable regression analyses. RESULTS: Data from 1356 individuals (69% female, 50% adolescents) were included. Median PHR on a numeric rating scale (NRS, 0-10) was 4 (IQR 2-6), median perceived stress was 3 (IQR 1-6). Adolescents reported a worse well-being with a significantly lower median WHO-5-score (60, IQR 40-76) than parents reported for their children < 12 years (80, IQR 68-84). Moderate to severe symptoms of depression and anxiety were reported by 14.3% and 12.3% of the adolescents, respectively. PHR was significantly higher in patients with systemic lupus erythematosus, methotrexate or biologic disease-modifying anti-rheumatic drug therapy than in patients without these characteristics, whereas lower WHO-5 or higher PHQ-9 or GAD-7 scores were only associated with poorer patient-reported health status and physical functioning. CONCLUSION: The perception of health risk due to SARS-CoV2 infection was not paralleled by an impairment of mental health, which were, however, significantly correlated with self-rated health status and functional capacity, highlighting the importance of patient-reported outcome assessment. TRIAL REGISTRATION: German Clinical Trials Register (DRKS), no. DRKS00027974. Registered on 27th of January 2022.

Exploring paediatric rheumatology care: a ten-year retrospective analysis of the patient population in Ghana.

BACKGROUND: Rheumatic diseases can seriously impact children's general health, development, and growth. However, due to a lack of resources, paediatric rheumatology is a largely underdeveloped speciality in many African nations. Children with rheumatic disorders face obstacles in accessing specialized medical care, including lack of specialists, care centres, medication access, and limited research and education to increase understanding of paediatric rheumatic disease among healthcare practitioners. This study described the disease characteristics, prevalence, and challenges faced by paediatric rheumatic disease patients receiving care at a teaching hospital in Accra, Ghana. METHODS: A retrospective record-based study was conducted among all paediatric cases presenting to the rheumatology clinic of the Korle Bu Teaching Hospital (KBTH) from January 2011 to December 2021. Data collected include clinical features, laboratory findings at disease presentation, andtherapeutic regimens prescribed per standard guidelines and experiences. RESULTS: A total of 121 cases were identified as of 2021, indicating a point prevalence of 0.0011%. The majority (73%) were females with a mean age of 13.4 ± 3.2 years. The mean duration of symptoms in months experienced by patients before being successfully referred to a rheumatologist was 18 months. There were significant differences between referred and confirmed diagnoses, especially in cases involving mixed connective tissue diseases (MCTD), systemic lupus erythematosus (SLE), and juvenile dermatomyositis (JDM), suggesting that these conditions may be under-recognised. Arthralgia and arthritis were the most common presenting symptoms. More than three-quarters (86.8%) of the cases studied were treated with steroids (oral or intravenous). In cases requiring immunosuppressive therapy, methotrexate was the most commonly prescribed in 33.9% of instances. Mortality was recorded at 8.3%, with the majority involving SLE cases. Most (95.7%) of the primary caregivers expressed positive experiences regarding care received at the adult rheumatology clinic. CONCLUSION: There were significant delays in diagnosis and diagnostic accuracy for patients with paediatric rheumatic disease (PRD). This highlights the pressing need for strengthening paediatric rheumatology services in Africa, including increasing awareness about these conditions among the public and healthcare providers to improve early diagnosis and quality of life for children with these conditions.

The characteristics and predictors of mortality in periprosthetic fractures around the knee.

Aims: Periprosthetic fractures (PPFs) around the knee are challenging injuries. This study aims to describe the characteristics of knee PPFs and the impact of patient demographics, fracture types, and management modalities on in-hospital mortality. Methods: Using a multicentre study design, independent of registry data, we included adult patients sustaining a PPF around a knee arthroplasty between 1 January 2010 and 31 December 2019. Univariate, then multivariable, logistic regression analyses were performed to study the impact of patient, fracture, and treatment on mortality. Results: Out of a total of 1,667 patients in the PPF study database, 420 patients were included. The in-hospital mortality rate was 6.4%. Multivariable analyses suggested that American Society of Anesthesiologists (ASA) grade, history of peripheral vascular disease (PVD), history of rheumatic disease, fracture around a loose implant, and cerebrovascular accident (CVA) during hospital stay were each independently associated with mortality. Each point increase in ASA grade independently correlated with a four-fold greater mortality risk (odds ratio (OR) 4.1 (95% confidence interval (CI) 1.19 to 14.06); p = 0.026). Patients with PVD have a nine-fold increase in mortality risk (OR 9.1 (95% CI 1.25 to 66.47); p = 0.030) and patients with rheumatic disease have a 6.8-fold increase in mortality risk (OR 6.8 (95% CI 1.32 to 34.68); p = 0.022). Patients with a fracture around a loose implant (Unified Classification System (UCS) B2) have a 20-fold increase in mortality, compared to UCS A1 (OR 20.9 (95% CI 1.61 to 271.38); p = 0.020). Mode of management was not a significant predictor of mortality. Patients managed with revision arthroplasty had a significantly longer length of stay (median 16 days; p = 0.029) and higher rates of return to theatre, compared to patients treated nonoperatively or with fixation. Conclusion: The mortality rate in PPFs around the knee is similar to that for native distal femur and neck of femur fragility fractures. Patients with certain modifiable risk factors should be optimized. A national PPF database and standardized management guidelines are currently required to understand these complex injuries and to improve patient outcomes.

Rheumatic diseases in migrant patients resident in Tuscany: epidemiological data analysis and single-center experience.

OBJECTIVE: In the last decades, the number of foreigners in Tuscany has considerably increased with a multiethnic distribution. We reviewed the main rheumatic diseases in the foreign population resident in Tuscany and also reported the experience at the Rheumatology Division of the University Hospital of Careggi, Florence, in order to identify the areas of origin of these patients and the main rheumatic diseases observed in them. METHODS: The collaboration with the Tuscan Region provided data about foreign patients residing in Tuscany on January 1, 2021 (country of origin, chronic diseases). Moreover, we conducted a retrospective review of the clinical charts of our Rheumatologic Division from January 1, 2019, to December 31, 2020. RESULTS: In Tuscany, on January 1, 2021, there were 61,373 patients with chronic inflammatory rheumatic diseases, and 3994 of them (6.51%) were foreigners. Most patients were born in Europe (39.03%), followed by the Balkans (15%), South America (11.27%), and North Africa (10.31%). Inflammatory joint diseases, Sjögren syndrome, and systemic lupus erythematosus were the most frequent diseases. In the period 2019-2020, 511 foreign patients visited our Rheumatology Division and mainly originated from the Balkans (34.64%), South America (18%), and European countries (16.44%). In these patients, chronic inflammatory joint diseases and connective tissue diseases (systemic sclerosis, Sjögren syndrome, and systemic lupus erythematosus) were the most prevalent diseases. CONCLUSIONS: This study provides a picture of the rheumatic diseases affecting foreign patients residing in Tuscany that are in agreement with the epidemiological data previously provided.

Exploring complex links: inflammatory rheumatic diseases and men's health.

This article examines the complex interactions between inflammatory rheumatic diseases (IRDs) and men's health. It delves into the effects of IRDs on reproductive health, erectile dysfunction, prostate involvement, male osteoporosis, body composition, physical activity, and coping mechanisms. The findings show that the prevalence of sexual dysfunction varies among different diseases, underscoring the necessity for comprehensive counseling. The link between IRDs and prostate health, with a substantial rise in benign prostatic hyperplasia among IRD patients, demonstrates the condition's importance. In contrast to popular belief, osteoporosis mostly affects women; the current study highlights the growing identification of male osteoporosis, particularly in the setting of IRDs. Male RA patients had a significant loss in bone mineral density, highlighting the importance of increasing awareness and tailored therapy to address osteoporosis in men. IRDs affect body composition, with male RA patients showing imbalances characterized by decreased lean body mass and increased fat mass. Given the dynamic nature of these conditions, coping with IRDs necessitates thorough and individualized diversified approaches. The complex link between IRDs and men's health demands continuing research, including longitudinal studies and tailored therapies. The essay promotes a patient-centered approach, recognizing the unique obstacles that males with IRDs confront.

Nursing and allied health workforce in Australian public rheumatology departments is inadequate: a cross-sectional observational study.

Rheumatological conditions are complex and impact many facets of daily life. Management of people with rheumatological conditions can be optimised through multidisciplinary care. However, the current access to nursing and allied health professionals in Australia is unknown. A cross-sectional study of nursing and allied health professionals in Australian public rheumatology departments for adult and paediatric services was conducted. The heads of Australian public rheumatology departments were invited to report the health professionals working within their departments, referral pathways, and barriers to greater multidisciplinary care. A total of 27/39 (69.2%) of the hospitals responded. The most common health professionals within departments were nurses (n = 23; 85.2%) and physiotherapists (n = 10; 37.0%), followed by pharmacists (n = 5; 18.5%), psychologists (n = 4; 14.8%), and occupational therapists (n = 4; 14.8%). No podiatrists were employed within departments. Referral pathways were most common for physiotherapy (n = 20; 74.1%), followed by occupational therapy (n = 15; 55.5%), podiatry (n = 13; 48.1%), and psychology (n = 6; 22%). The mean full-time equivalent of nursing and allied health professionals per 100,000 population in Australia was 0.29. Funding was identified as the most common barrier. In Australia, publicly funded multidisciplinary care from nurses and allied health professionals in rheumatology departments is approximately 1.5 days per week on average. This level of multidisciplinary care is unlikely to meet the needs of rheumatology patients. Research is needed to determine the minimum staffing requirements of nursing and allied health professionals to provide optimal care.

Comparison of systemic lupus international collaborating clinics 2012 classification criteria and European league against rheumatism/American college of rheumatology 2019 classification criteria for early detection of childhood onset systemic lupus erythematosus (multi-center study).

OBJECTIVE: To assess the performance of the new EULAR/ACR criteria, particularly for early detection of cSLE, in comparison to the SLICC criteria among the pediatric population in multiple centers in Saudi Arabia. METHODS: We conducted a retrospective study that enrolled pediatric patients up to the age of 14 years who've been diagnosed with SLE and followed in pediatric rheumatology clinics at 9 multi-tertiary hospitals in Saudi Arabia from 2010 to 2021 as a case group and were compared to a similar group of pediatric patients who've had defined rheumatological diseases other than SLE with a positive ANA titer (≥1:80) as controls. In total, 245 patients were included and distributed as 129 cases (diagnosed by expert pediatric rheumatologists) versus 116 patients in the control group. All relevant clinical information, including history, physical examination findings, and laboratory tests, was documented at the initial presentations. Then, the two sets of SLE classification criteria were applied to both groups to define who's going to meet both or either one of them. The exclusion criteria included those who had insufficient data or had overlapping or undifferentiated diseases. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), receiver operating curve (ROC), and accuracy were calculated for SLICC 2012 and EULAR/ACR 2019 criteria (total scores≥ 10 and ≥ 13). We performed a Chi-squared test to compare sensitivity and specificity of SLICC 2012 and EULAR/ACR 2019. RESULTS: For SLICC (cut-off ≥4 criteria), the sensitivity was found to be 96.9% (95% CI 92.6%-99.4%) and the specificity was 94.8% (95% CI 89.6%-98.32%), with PPV and NPV of 95.4% and 96.5%, respectively. The ROC for it was 0.96 (95% CI 0.93-0.99), and this criterion had an accuracy of 95%. Regarding EULAR/ACR (total score ≥ 10), the performance measure showed a sensitivity of 99.2% and a specificity of 86.2%. Similarly, PPV was 88.9%; while NPV was a little higher (99.0%) than SLICC. The ROC for EULAR/ACR (total score ≥ 10) was 0.93 (95% CI 0.89-0.96), and this criterion had an accuracy of 93%. However, there was no statistically significant difference between the sensitivity and specificity of either using SLICC or EULAR/ACR (total score ≥ 10), as reflected by a p-value of 0.86 using the Chi-squared test. Although applying the EULAR/ACR with a total score of ≥ 13 revealed lower sensitivity (93.8%) than both the SLICC and the EULAR/ACR (total score ≥ 10), the specificity for it was found to increase up to 91.4% (85.7-96.2%) compared to the (86.2%) specificity of the EULAR/ACR (total score ≥ 10). CONCLUSION: In this cohort among the Saudi population with childhood-onset SLE, the new EULAR/ACR 2019 criteria efficiently enable early detection of SLE, although a more frequent rate of false positives was observed with them. Escalating the total score from ≥ 10 to ≥ 13 in the cSLE population improved the specificity close to that of SLICC 2012. Further prospective studies in pediatrics need to be done for the validation of a cut- off score of ≥ 13 in cSLE rather than the traditional score of ≥ 10 in aSLE.

Paxlovid use is associated with lower risk of cardiovascular diseases in COVID-19 patients with autoimmune rheumatic diseases: a retrospective cohort study.

BACKGROUND: Paxlovid has been shown to be effective in reducing mortality and hospitalization rates in patients with coronavirus disease 2019 (COVID-19). It is not known whether Paxlovid can reduce the risk of cardiovascular diseases (CVD) in COVID-19-surviving patients with autoimmune rheumatic diseases (AIRDs). METHODS: TriNetX data from the US Collaborative Network were used in this study. A total of 5,671,395 patients with AIRDs were enrolled between January 1, 2010, and December 31, 2021. People diagnosed with COVID-19 were included in the cohort (n = 238,142) from January 1, 2022, to December 31, 2022. The Study population was divided into two groups based on Paxlovid use. Propensity score matching was used to generate groups with matched baseline characteristics. The hazard ratios (HRs) and 95% confidence intervals of cardiovascular outcomes, admission rate, mortality rate, and intensive care unit (ICU) admission rate were calculated between Paxlovid and non-Paxlovid groups. Subgroup analyses on sex, age, race, autoimmune diseases group, and sensitivity analyses for Paxlovid use within the first day or within 2-5 days of COVID-19 diagnosis were performed. RESULTS: Paxlovid use was associated with lower risks of cerebrovascular complications (HR = 0.65 [0.47-0.88]), arrhythmia outcomes (HR = 0.81 [0.68-0.94]), ischemic heart disease, other cardiac disorders (HR = 0.51 [0.35-0.74]) naming heart failure (HR = 0.41 [0.26-0.63]) and deep vein thrombosis (HR = 0.46 [0.24-0.87]) belonging to thrombotic disorders in AIRD patients with COVID-19. Compared with the Non-Paxlovid group, risks of major adverse cardiac events (HR = 0.56 [0.44-0.70]) and any cardiovascular outcome mentioned above (HR = 0.76 [0.66-0.86]) were lower in the Paxlovid group. Moreover, the mortality (HR = 0.21 [0.11-0.40]), admission (HR = 0.68 [0.60-0.76]), and ICU admission rates (HR = 0.52 [0.33-0.80]) were significantly lower in the Paxlovid group than in the non-Paxlovid group. Paxlovid appears to be more effective in male, older, and Black patients with AIRD. The risks of cardiovascular outcomes and severe conditions were reduced significantly with Paxlovid prescribed within the first day of COVID-19 diagnosis. CONCLUSIONS: Paxlovid use is associated with a lower risk of CVDs and severe conditions in COVID-19-surviving patients with AIRD.

[Patient education in rheumatology]. / Éducation thérapeutique du patient en rhumatologie.

In Switzerland, patient education in rheumatology faces challenges, despite national advances following the development of the concept of self-management support by the Federal Office of Health. Yet it is a fundamental pillar of rheumatic disease management, meeting the needs of both patients and healthcare professionals. It helps to improve the overall quality of care and is recommended by the European Alliance of Associations for Rheumatology (EULAR). Training in PE needs to be strengthened, as does the evaluation of programmes, which must take account of the specific nature of rheumatic diseases. An integrated, patient-centered approach is essential to overcome these obstacles and promote PE in rheumatology.

En Suisse, l'éducation thérapeutique du patient (ETP) en rhumatologie rencontre des défis malgré des avancées nationales à la suite de l'élaboration du concept de soutien à l'autogestion par l'Office fédéral de la santé publique. C'est pourtant un pilier fondamental de la prise en charge des maladies rhumatismales répondant aux besoins à la fois des patients et des professionnels de la santé. Elle permet une amélioration globale de la qualité des soins et est recommandée par l'Alliance européenne des associations de rhumatologie (EULAR). La formation en ETP nécessite d'être renforcée, tout comme l'évaluation des programmes devant intégrer la spécificité des pathologies rhumatismales. Une approche intégrée et axée sur le patient est essentielle pour surmonter ces obstacles et promouvoir l'ETP en rhumatologie.

Is There a Link between the Molecular Basis of Juvenile Idiopathic Arthritis and Autoimmune Diseases? Systematic Review.

Juvenile Idiopathic Arthritis (JIA) is currently the most common chronic rheumatic disease in children. It is known to have no single identity, but a variety of diagnoses. Under-diagnosis is a barrier to early treatment and reduced complications of the disease. Other immune-mediated diseases may coexist in the same patient, making research in this area relevant. The main objective was to analyse whether links could be established between the molecular basis of JIA and other immune-mediated diseases. Early diagnosis may benefit patients with JIA, which in most cases goes undetected, leading to under-diagnosis, which can have a negative impact on children affected by the disease as they grow up. METHODS: We performed a PRISMA systematic review focusing on immune molecules present in different autoimmune diseases. RESULTS: A total of 13 papers from different countries dealing with the molecular basis of JIA and other immune diseases were evaluated and reviewed. CONCLUSIONS: Most of the autoimmune diseases analysed responded to the same group of drugs. Unfortunately, the reason for the under-diagnosis of these diseases remains unknown, as no evidence has been found to correlate the immunomolecular basis with the under-diagnosis of these immune-mediated diseases. The lack of information in this area means that further research is needed in order to provide a sound basis for preventing the development of immune-mediated diseases, especially in children, and to improve their quality of life through early diagnosis and treatment.

Comparison of the SLE Risk Probability Index (SLERPI) scale against the European League Against Rheumatism/American College of Rheumatology (ACR/EULAR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria.

INTRODUCTION: Timely diagnosis and proper recognition of Systemic Lupus Erythematosus (SLE) is essential to establish early management in inpatients and outpatients. There are different classification scales to identify SLE, which include various clinical and serological aspects. In 2021, the SLE Risk Probability Index (SLERPI) was published, which focuses predominantly on the clinical characteristics of patients with suspected SLE and uses a simple algorithm for early recognition of the disease. The aim of this study is to compare the European League Against Rheumatism/American College of Rheumatology (ACR/EULAR) classification criteria, the Systemic Lupus International Collaborating Clinics (SLICC) criteria, and the SLERPI criteria in a cohort of Colombian patients with SLE and to analyze the correlations observed between their absolute scores. METHODS: A registry of SLE patients from two referral hospitals in Bogotá, Colombia, was used. 2021 SLERPI, 2019 ACR/EULAR, and 2012 SLICC scores were calculated for each patient and the correlations found between the scales were analyzed. The sensitivities of each were compared, and frequency analyses were conducted among different clinical and laboratory variables. RESULTS: Between 2016 and 2019, 146 patients diagnosed with SLE were registered, including inpatients and outpatients. The median age was 36 years (interquartile range 26-51), and 82.2% were women. According to the SLERPI criteria, a high prevalence of antinuclear antibodies (92%), immunological disorders (71%), and arthritis (64%) were observed. The most used treatments were corticosteroids (87.6%) and chloroquine (67.8%). A Spearman evaluation analysis was performed, with a moderately strong correlation of 0.76 (p = .000) between the SLERPI and ACR/EULAR scales and very strong correlation of 0.80 (p = .000) between the SLERPI and SLICC. Patients classified with SLE according to the SLERPI scale exhibited a higher incidence of hematological compromise, along with elevated levels of serological markers such as anti-DNA antibodies. Additionally, this group more commonly received treatments involving corticosteroids and azathioprine, and displayed a higher prevalence of hypertension. CONCLUSION: The SLERPI scale could be useful in the diagnosis of SLE, especially in early stages, given its good correlation with other classification scales and its good sensitivity.

Crystal induced arthropathies-a comparative study of 40 patients with apatite rheumatism, chondrocalcinosis and primary synovial chondromatosis.

Introduction: Apatite rheumatism (AR), chondrocalcinosis (Ch-C), and primary synovial chondromatosis (prSynCh) are regarded as distinct clinical entities. The introduction of the non-staining technique by Bély and Apáthy (2013) opened a new era in the microscopic diagnosis of crystal induced diseases, allowing the analysis of MSU (monosodium urate monohydrate) HA (calcium hydroxyapatite), CPPD (calcium pyrophosphate dihydrate) crystals, cholesterol, crystalline liquid lipid droplets, and other crystals in unstained sections of conventionally proceeded (aqueous formaldehyde fixed, paraffin-embedded) tissue samples. The aim of this study was to describe the characteristic histology of crystal deposits in AR, Ch-C, and prSynCh with traditional stains and histochemical reactions comparing with unstained tissue sections according to Bély and Apáthy (2013). Patients and methods: Tissue samples of 4 with apatite rheumatism (Milwaukee syndrome), 16 with chondrocalcinosis, and 20 with clinically diagnosed primary synovial chondromatosis were analyzed. Results and conclusion: Apatite rheumatism, chondrocalcinosis, and primary synovial chondromatosis are related metabolic disorders with HA and CPPD depositions. The authors assume that AR and Ch-C are different stages of the same metabolic disorder, which differ from prSynCh in amorphous mineral production, furthermore in the production of chondroid, osteoid and/or bone. prSynCh is a defective variant of HA and CPPD induced metabolic disorders with reduced mineralization capabilities, where the deficient mineralization is replaced by chondroid and/or bone formation. The non-staining technique of Bély and Apáthy proved to be a much more effective method for the demonstration of crystals in metabolic diseases than conventional stains and histochemical reactions.

Safety of biologic agents for the management of rheumatic diseases during pregnancy.

PURPOSE OF REVIEW: To discuss the current understanding regarding the use of biologic therapeutics in pregnancy. RECENT FINDINGS: Our understanding of the mechanisms underlying the potential fetal and infant exposure to biologics as well as a growing body of empirical evidence from real world use of biologics in pregnancy have demonstrated that biologics are generally compatible preconception and during pregnancy. Long-term effects of exposure to biologic agents in utero are not known, but will be uncovered in time. Biosimilars, which are becoming more popular, may not always share the same safety profiles as their originators. SUMMARY: Biologics have revolutionized the management of rheumatologic disease and ushered in a new era of clinical remission among patients. These agents, developed and introduced into clinical use at the beginning of the new millennium, are very potent, yet their efficacy in treating disease often in reproductive aged women, raises questions regarding their safety during pregnancy. These therapeutics can cause immunosuppression and can inhibit immunologic circuits that are not only involved in disease pathophysiology but hypothetically could impact the development of the fetal immune system. Reassuringly, biologics, typically antibodies or antibody-based proteins, are introduced to the fetus via the typical route of transplacental antibody transfer, and thus only begin to be transferred in appreciable amounts in the second trimester (after organogenesis). From theoretic and empirical standpoints, biologic use during pregnancy appears well tolerated for fetal development and to not substantially affect infant immune development.

Look after the COVID-19 pandemic: Mortality rates among patients with rheumatic diseases.

AIM: Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection may have a more severe course in patients with underlying disease or who have had immunosuppression. In this study, it was aimed to determine the frequency of coronavirus disease 2019 (COVID-19) and the mortality rates related to COVID-19 among patients with rheumatic disease. METHODS: The patients who were followed up with rheumatic disease in the rheumatology outpatient clinic in a tertiary hospital were retrospectively assessed if they had COVID-19 infection or not between March 2020 and January 2022. RESULTS: A total of 10 682 patients were evaluated. There were 2928 (27.4%) COVID-19-positive and 7754 (72.6%) COVID-19-negative patients. The mean age of COVID-19-positive patients was 46.2 ± 14.6 years, and 65.8% were female. Forty-two (1.4%) patients died due to COVID-19. Among COVID-19-negative patients, 192 patients died. The most common rheumatic disease among patients with COVID-19 was spondyloarthritis (SpA) (30.4%). Corticosteroids were the most common treatment agent in COVID-19-positive patients regardless of mortality. Thirty-one (73.8%) patients were receiving corticosteroids, and 35 (83.3%) patients were receiving immunosuppressive agents among patients with mortality. According to the logistic regression analysis, older age, male gender, and receiving corticosteroid, hydroxychloroquine, mycophenolate mofetil, tofacitinib, rituximab, and cyclophosphamide were found to be related to increased mortality. CONCLUSION: COVID-19 is a serious infection and the current study emphasized that patients with rheumatic diseases had increased mortality rates, particularly in patients who were old, male, and on immunosuppressive treatments.

[Update on Sjögren's syndrome : Diagnostics, treatment, and challenges]. / Update Sjögren-Syndrom : Diagnostik, Therapie und Fallstricke.

Sjögren's syndrome (SjS) is the most common connective tissue disease with a prevalence of 1:200. Predominantly affecting women, SjS is associated with destruction of the exocrine glands, leading to xerophthalmia and xerostomia. In over 50% of patients, there are also extraglandular manifestations, leading to multiple organ manifestations including polyneuropathies and interstitial lung disease as well as symptoms such as fatigue and arthralgia. Diagnostic procedures include biomarkers, in particular anti-SS-A/Ro antibodies, histology of salivary glands, and salivary gland sonography. There are currently no licensed immunosuppressive drugs for SjS, so current treatment is often based on off-label use of drugs. The European League Against Rheumatism (EULAR) has recently published treatment recommendations based on the prevailing organ manifestations. Several promising controlled trials with novel compounds and concepts are currently in progress.