Rheumatology Department Research Articles

Transmembrane Protein 187 (TMEM187) and Interleukin 1 Receptor Associated Kinase (IRAK1) gene polymorphism association with rheumatoid arthritis susceptibility in Egyptian patients

Rheumatoid arthritis (RA) is a chronic auto-inflammatory disease of connective tissue with progressive joint damage and systemic disorders. RA is considered a multifactorial disease triggered by a genetic predisposition and environmental factors. Polymorphisms have been identified in the Xq28 locus as risk loci for RA. The aim of study was to assess the association between two polymorphisms in the Xq28 region containing Transmembrane Protein 187 (TMEM187) gene (rs13397) and interleukin1 receptor associated kinase (IRAK1) gene (rs1059703) with the disease susceptibility and activity in Egyptian RA patients. This study was conducted on 100 RA patients and 100 age and sex matched normal controls, together were recruited from the Rheumatology Department, Beni-Suef University Hospital. We detected TMEM187 (rs13397) and IRAK1 (rs1059703) gene polymorphisms using the real time PCR TaqMan allelic discrimination assays. We found that the frequency of the major genotypes (GG) of TMEM 187 gene was higher in RA group (54%) compared to controls (50%); while the minor genotypes (AA) was higher in the control group (22%) compared to the diseased one (18%), but such differences did not reach statistical significance (p=0.599). Regarding the IRAK1 gene, we revealed that the frequency of the major genotypes (AA) of the rs1059703 was slightly higher in RA group (48%) compared to controls (46%); while the minor genotypes (GG) was the same in both groups (26%). However, there was higher incidence of minor genotype in the TMEM187 and IRAK1 genes in males; with a statistical significance (p=0.004 and 0.015, respectively). We concluded that the major allele G of TMEM187(rs 13397) could be considered as a risk genetic allele for RA in Egyptian populations.

RETROSPECTIVE ANALYSIS OF MEDICAL CHARTS OF PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME

Background. Antiphospholipid attracts the attention of clinicians in various fields of medicine. This pathology is characterized by a triad of clinical and laboratory signs: recurrent venous or arterial thrombosis with localization in any part of the bloodstream, obstetric pathology in the form of primary premature pregnancy and intrauterine death of the fetus with hematological disorders (thrombocytopenia, hemolytic anemia).
 Aim: to conduct a retrospective analysis of medical records of patients with antiphospholipid syndrome.
 Materials and methods. A retrospective analysis of outpatient/inpatient medical records of 54 patients with the main diagnosis: antiphospholipid syndrome between 2016 and 2021, who were examined and treated in the rheumatology department of the Multidisciplinary Medical Center of Odesa National Medical University, was conducted. The diagnosis of antiphospholipid syndrome was established according to the recommendations of EULAR (2019), Order of the Ministry of Health of Ukraine dated October 8, 2007 No. 626 "Clinical protocol for providing medical care to patients with Antiphospholipid Syndrome" and Order of the Ministry of Health of Ukraine No. 22 dated January 20, 2015. "Clinical protocol for providing medical care to patients with immune diseases."
 Results. In the general group of patients, the duration of this pathology at the time of the study ranged from 1 to 10 years. It was established that the number of appeals in connection with exacerbation of antiphospholipid syndrome depended on age and had probable differences. It was also noted that the total number of concomitant pathologies was correlated with the number of appeals regarding exacerbation of antiphospholipid syndrome. It was revealed that arthralgia and polyarthritis were most often detected in male patients aged 20-44 against the background of antiphospholipid syndrome, and skin manifestations in men aged 45-59. In women of the age group of 20-44 years, on the background of the diagnosed antiphospholipid syndrome, skin manifestations in the form of reticular liver, arthralgia and polyarthritis, as well as cardiovascular disorders, were most often noted. In women aged 45-59, arthralgia and polyarthritis and disorders of the cardiovascular system.
 Using the method of collecting anamnestic data and the results of clinical laboratory examination, in accordance with current medical care protocols and diagnostic criteria for antiphospholipid syndrome, a retrospective analysis confirms the fact that antiphospholipid syndrome occurs 5 times more often in women than in men.

The phenotype and genotype of Chinese adult patients with NLRP3-associated autoinflammatory disease.

NLRP3-associated autoinflammatory disease (NLRP3-AID) is a spectrum of autosomal dominant inherited diseases associated with NLRP3 gene mutations. Reports of Chinese NLRP3-AID cases are limited to date. In the present study, we aim to describe the phenotype and genotype of a cohort of Chinese adult NLRP3-AID patients METHODS: This single-center study included sixteen adult patients diagnosed with NLRP3-AID at Department of Rheumatology, Peking Union Medical College Hospital from April 2015 to September 2021. Whole-exome sequencing using next-generation sequencing was performed in each patient. Clinical data and mutational information were compared with a European cohort. The median age of disease onset was 16 (0-46) years old, and adult-onset was observed in 4 patients (25%). The median time of diagnosis delay was 20 (0-39) years. Five patients (31.3%) had family history of similar symptoms. The most common clinical manifestations were recurrent fever (93.8%), arthralgia/arthritis (81.3%), skin rash (75%), myalgia (62.5%), and central nervous system manifestations (50%). Heterozygous NLRP3 variants detected in these patients were p.T348M (n = 4, 25%), Q703K, V70M, K129R, M116I, P38S, V442I, D303G, G326E, A439V, K829T, L632F and V198M (n = 1, separately). All the variants were missense mutations. We reported the largest case series of Chinese adult NLRP3-AID patients. The distinct symptoms of NLRP3-AID patients suggest the heterogeneity of disease. P38S, M116I, K129R, V442I and K829T were identified as novel NLRP3 variants. These data expand the clinical phenotypic and genotypic profiles of NLRP3-AID. Key Points • We characterized the clinical and genetic features of sixteen Chinese adult NLRP3-AID patients. • Thirteen NLRP3 gene variants were confirmed in this cohort, and P38S, M116I, K129R, V442I and K829T were identified as novel variants. • Clinical data and mutation information were compared with a European cohort. • We hope these data would expand the phenotypic and genotypic profile of NLRP3-AID and raise the awareness of early diagnosis and accurate treatment among rheumatologists.

'Status Quo' on Different Aspects of Gender Distribution in Rheumatology in Germany-Results from a Nationwide Online Survey among Physicians.

Despite the increasing number of female medical students and fellows in Europe, women are still under-represented in higher academic careers and positions in medicine. The aim of this survey was to assess the 'status quo' on gender distribution among rheumatologists in Germany. A web-based anonymous survey (21 questions with multiple answers and free text) using QuestionPro® was distributed among rheumatologists in Germany via newsletters, social media and personal contact, including questions regarding hierarchical positions and work characteristics. Among the total of 170 respondents (72% women, 28% men, 1% diverse), 48% were rheumatologists in training, 35% were trained rheumatologists and 7% were heads of rheumatology departments. Regarding the gender ratio at different hierarchical levels, 74% of respondents reported more men than women in leadership positions. Part-time work was possible in the departments of 86% of respondents, with more women working part-time (56%) compared to men (29%). Most respondents stated their impression that employees working part-time did not have the same career chances as full-time workers in their departments. In total, 66% agreed that activities to improve gender equity are necessary. The highest need was seen in reconciling work and family through, e.g., part-time models, flexible childcare options at work and a higher acceptance of part-time work in leadership positions. According to our results, a gender imbalance is prevalent among rheumatologists in Germany, with lower numbers of women evident at higher hierarchical levels. Traditional role assignments are still represented by a higher proportion of part-time work in women. The establishment of structural changes to achieve better gender equity is needed.

Short-term Efficacy and Safety of Secukinumab in Pakistani Patients with Ankylosing Spondylitis and Psoriatic Arthritis

Objective: To determine the short-term efficacy and safety of Secukinumab in Pakistani patients with ankylosing spondylitis and psoriatic arthritis in regular clinical practice.
 Study Design: Case series.
 Place and Duration of Study: Department of Rheumatology, National Hospital and Medical Centre, Lahore Pakistan from Aug 2019 to Feb 2021.
 Methodology: Patients diagnosed with ankylosing spondylitis according to assessment of spondyloarthritis International Society criteria and psoriatic arthritis according to classification criteria for psoriatic arthritis (CASPAR), who were started on Secukinumab, were enrolled. Disease activity scores and adverse events were recorded in the 12th and 24th weeks. The primary outcomes were remission and low disease activity, and the secondary outcomes were the recording of Adverse events on each visit.
 Results: There were 42 patients registered. The mean duration of disease was 11.86±7.31 years for AS and 9.19±4.87 years for PsA patients. At 24 weeks of follow-up, 10% of patients discontinued treatment, 73.7% of AS patients, and 94.7% of PsA patients achieved complete remission or low disease activity. No significant AEs were noted at 24 weeks, except for upper respiratory tract infections. One patient developed a lower respiratory tract infection after 24 weeks of treatment, and one died during treatment (possible myocardial infarction).
 Conclusion: Secukinumab was effective and safe in patients with Ankylosing Spondylitis and Psoriatic Arthritis in this study,despite advanced disease with long disease duration.Keywords: Ankylosing spondylitis, Efficacy, Psoriatic arthritis, Safety, Secukinumab.

Short-Term Efficacy and Safety of Subcutaneous Tocilizumab in Rheumatoid Arthritis Patients in Pakistan: A Real-World Experience

Objective: To determine the real-world efficacy and safety of subcutaneous Tocilizumab in patients with rheumatoid arthritis.
 Study Design: Case series.
 Place and Duration of Study: Department of Rheumatology, National Hospital and Medical Centre, Lahore Pakistan, from Aug 2019 to Dec 2020.
 Methodology: In this study, 33 patients receiving subcutaneous Tocilizumab 162 mg every two weeks as monotherapy or with conventional synthetic disease modifying anti-rheumatic drugs were followed per standard study protocol. Increasing or decreasing the dosing interval was allowed according to disease activity at the discretion of the treating rheumatologist. The primary outcome was patients achieving Low Disease Activity as per Clinical Disease Activity Index (>2.8-10.0) and Disease Activity Score-28(>2.6-3.2). The secondary outcome was a clinically meaningful improvement in Disease Activity Score -28 (reduction of ≥1.2 units in Disease Activity Score-28 score). Adverse events were recorded at each follow-up visit.
 Results: Of the 33 patients, 25(75.8%) were biologics-naive, 9(27.2%) patients achieved the target of Low Disease Activity as per Clinical Disease Activity Index (2.9-10.0) and 8(24.2%) patients achieved Low Disease Activity as per Disease Activity Score-28(2.7-3.2). At six months, the proportion of patients achieving clinically meaningful improvement (decrease ≥1.2) in Disease Activity Score -28 was 13(54.2%). Overall, 14(42.4%) patients developed adverse events and 3(9.1%) patients discontinued Tocilizumab owing to adverse events.
 Conclusion: Tocilizumab has been seen to be an effective biologic in only one-third of patients, and it was tolerated in twothirds of the patients.Keywords: Conventional synthetic disease modifying anti-rheumatic drugs, Efficacy, Rheumatoid arthritis, Safety, Subcutaneous tocilizumab.

Eosinophilic Fasciitis: A Rare Mimicker of Systemic Sclerosis.

From the Department of Rheumatology, Brooke Army Medical Center, San Antonio, TX. The authors declare no conflict of interest. Disclaimer: The view(s) expressed herein are those of the author(s) and do not reflect the official policy or position of Brooke Army Medical Center, the US Army Medical Department, the US Army Office of the Surgeon General, the Department of the Army, the Department of the Air Force and Department of Defense, or the US Government. Correspondence: Seth J. VanDerVeer, DO, Department of Rheumatology, Brooke Army Medical Center, 3551 Roger Brooke Dr, Fort Sam Houston, TX 78234. E-mail: [email protected].

Frequency of Thyroid Disorders in Rheumatoid Arthritis Patients and its Association with Disease Activity: A Hospital Based Study

Objective: To establish the frequency of thyroid disorders among Rheumatoid Arthritis patients and its association with disease severity measured by DAS28-ESR.
 Study Design: Prospective longitudinal study.
 Place and Duration of Study: Department of Rheumatology, Federal Government Policlinic Hospital, Islamabad Pakistan, from Jan to Jun 2021.
 Methodology: The study included 87 consecutive patients either gender with Rheumatoid Arthritis visiting Rheumatology OPD. Thyroid profile and DAS28-ESR were performed in all the patients.
 Results: Out of 87 patients, 73(83.9%) were females, and 14(16.1%) were males. The mean age was 45.54±13.50 years. The mean DAS28-ESR was 4.45±0.68, with a minimum DAS28-ESR of 1.25 and a maximum of 8.40. The Disease severity calculated by DAS28-ESR was mild in 19(21.8%), moderate in 44(50.6%) and severe in 24(27.6 %) patients. The thyroid gland was abnormal in 23(26.1%) patients. 18(78.2%) of 23 patients with thyroid disorders belonged to the moderate to severe Rheumatoid arthritis disease activity group.
 Conclusion: Thyroid disorders are very common in Rheumatoid Arthritis patients. Thyroid workup should be part of investigations of Rheumatoid Arthritis for early detection of thyroid disorder and better disease control.

Joint Hypermobility and Musculoskeletal Pain in Paramedical Students and Staff at a Tertiary Care Hospital

Objective: to determine the frequency of joint hypermobility in paramedical students and staff at National Hospital Lahore.
 Study Design: Cross-sectional study.
 Place and Duration of Study: Department of Rheumatology, National Hospital and Medical Center, Lahore Pakistan, from Mar to Sep 2021. Methodology: Two hundred fifty paramedical students and staff of either gender, aged between 16 years to 35 years were enrolled. Localized Joint Hypermobility was defined as <3 Score and generalized Joint Hypermobility were defined as >4 scores on the Beighton Score Scale. Demographic information was obtained from each participant, and joint hypermobility was assessed by using the Beighton score.
 Results: Mean age of the study participnats was 26.6±4.6 years with 146(58.24%) females. Mean BMI was 23.9±4.0 kg/m2. Generalized Joint Hypermobility was seen in 51(20.4%), and 31(12.4%) had localized hypermobility. Out of 250 participants, 33 (13.2%) had musculoskeletal pain, while among 51 patients with generalized hypermobility, 12(23.5%) had musculoskeletal pain.
 Conclusion: One out of every five young healthy paramedics enrolled had generalized joint hypermobility. One-third of subjects had at least one clinically documented hypermobile joint. Almost every fourth person with generalized joint hypermobility has musculoskeletal pain compared to 1 in 10 persons without generalized hypermobility.
 Keywords: Beighton score scale, Generalized joint hypermobility, Localized joint hypermobility, Musculoskeletal pain.

Predicting physical activity and sarcopenia-related health outcomes in women with rheumatoid arthritis: A test of the self-determination theory.

This study aimed to examine a hypothetical model of physical activity (PA) and health outcomes related to sarcopenia in women with rheumatoid arthritis (RA) based on self-determination theory. Cross-sectional study. This study included 214 women diagnosed with RA from the outpatient rheumatology department of a university-affiliated hospital in South Korea. Data were collected from September 2019 to August 2020 through structured questionnaires and anthropometric measurements and analysed using path analysis to test the hypothesized model. The primary health outcomes were perceived health status and sarcopenia-related health (thigh circumference, handgrip strength and sarcopenia risk). The final model's fit indices were adequate. Physical activity was directly affected by motivation for PA, while depression, self-efficacy for PA, health care provider's autonomy support and basic psychological needs satisfaction indirectly affected PA. Physical activity directly affected perceived health status and thigh circumference, while perceived sarcopenia risk and handgrip strength were directly affected by disease activity and age. Patients were involved in a questionnaire-based survey.

Comparison of Upper Gastrointestinal Toxicity of Enteric-Coated Prednisolone Versus Non-EntericCoated Prednisolone Among Patients Managed at the Rheumatology Department

Objective: To compare the upper gastrointestinal toxicity of enteric-coated Prednisolone versus non-enteric-coated Prednisolone among patients managed at the rheumatology department.
 Study Design: Cross-sectional study.
 Place and Duration of Study: Rheumatology Department, Pak Emirates Military Hospital Rawalpindi, from May 2019 to Mar 2020.
 Methodology: Patients of any rheumatological or immune-based condition who were taking Prednisolone for more than three months were included in the study. Stool for occult was performed for all the patients from the laboratory of their hospital, and patients with the presence of melena were classed as having upper gastrointestinal toxicity.
 Results: Mean age of the study participants was 43.64±2.74 years, 43(17.2%) patients had the presence of upper gastrointestinal toxicity, while 207(82.8%) did not show the presence of upper gastrointestinal toxicity. Pearson chi-square test revealed that advancing age and use of non-enteric coated Prednisolone had a statistically significant association with upper gastrointestinal toxicity among the patients suffering from any rheumatological condition managed with Prednisolone (p-value <0.05).
 Conclusion: Upper gastrointestinal toxicity emerged as a common finding among the patients managed in the rheumatology department with Prednisolone for various immune-based disorders. The use of non-enteric coated tablets and advancing age emerged as strong predictors for upper gastrointestinal toxicity among the study participants.

Translation and Validation of the Urdu Version of the Methotrexate Intolerance Severity Score in Patients with Inflammatory Arthritis

Abstract Background: Methotrexate (MTX) is a cornerstone in the treatment of patients with inflammatory arthritis but is not tolerated well by patients. This intolerance can be identified using the Methotrexate Intolerance Severity Score (MISS). Objective: The objective of this study was to translate and validate the MISS in the Urdu language. Materials and Methods: It was a cross-sectional study done at the Department of Rheumatology, Combined Military Hospital, Lahore, from December 1, 2021, to March 31, 2022. A total of 181 patients with inflammatory arthritis taking MTX for >3 months participated in the study. The translated Urdu version was tested in these patients who attempted the questionnaire twice with a gap of 2 weeks. Cronbach’s alpha, item correlations, and kappa coefficients were used to evaluate internal consistency, acceptability, and reproducibility. A culturally admissible Urdu version of MISS was developed. Results: The results show a 90% response rate, <1% missing data with very good acceptability, and understanding of patients. There were satisfactory inter-item, dimension-item, and inter-dimension correlations. The kappa coefficient was more than 0.90 for most items except associative stomachache (0.79). Cronbach’s alpha coefficient was 0.78, much higher than 0.6, and in the validation cohort, it was 0.74. The internal and external consistency results for validation were satisfactory. There was a good understanding between the test and retest for every patient. The MISS showed a sensitivity of 72% and a specificity of 100% at a discriminative cutoff value of 6.5. Of the 181 patients receiving MTX, 48 (26.5%) experienced MTX intolerance. Conclusion: This validated Urdu version MISS is a sensitive and specific tool that can better detect this MTX intolerance. It discriminates well between MTX-tolerant and intolerant patients.

Noninfectious Uveitis in Rheumatology: Patterns, Treatment, and Outcomes.

The present study aimed to determine the pattern and cause of noninfectious uveitisin rheumatology practice. The secondary objective was to identify the pattern of treatment and outcomes. This retrospective cross-sectional study was conducted in the Department of Rheumatology, National Hospital and Medical Centre, Lahore, Pakistan. After receiving consent, electronic medical records (EMRs) of all patients with a diagnosis of noninfectious uveitis (NIU) from November 2019 to January 2023 were reviewed,and a total of 52 patients labeled as having noninfectious uveitis were identified. The collected data included age at diagnosis, anatomical location of uveitis, associated systemic disease, used medications, and outcomes.All cases had been diagnosed and assessed mutually by a rheumatologist and an ophthalmologist using the International Uveitis Study Group classification system to classify the pattern of uveitis by location, clinical course, and laterality and rule out the possibility of other ophthalmologic diseases. Disease activity was defined using the Standardization of Uveitis Nomenclature (SUN) guidelines. Data was analyzed on SPSS Statistics version 23 (IBM Corp, Armonk, NY, USA). The mean age of the patients in this study was 36.02 ± 43.31 years, with31 (59.6%) male patients. Anterior uveitis was the most common type observed among the patients at 55.8%,panuveitis was found in 25%, intermediate uveitis and posterior uveitis were seen in 9.6% each. Based on laterality, unilateral eye involvement was identified in 53.8% of patients. Spondyloarthritis (SpA)and idiopathic uveitis were observedin 34.6% and 28.8%, respectively. In this study, 28 (54.9%) patients were on conventional disease-modifying antirheumatic drugs (cDMARDDs), and 23 (45.1%) were on biological DMARDs. In the biologics group, 82% of patients were in remission in comparison to 60% in the cDMARDs group. To the best of our knowledge, this is the first report onnoninfectious uveitis in the Pakistani population. The study concluded that anterior uveitis is the most common type of uveitis and is more common in males. Spondyloarthropathy is one of the most common underlying systemic diseases. Human leukocyte antigen (HLA)-B27is associated more with uveitis. Biologics are more effective than cDMARDs in controlling the disease.Collaborative work between different specialties resulted in early diagnosis of underlying systemic disease, better management plans, and disease outcomes. To obtain further details on noninfectiousuveitis, a population-based study is needed in Pakistan.

Clinical features and prognosis of 118 children with histiocytic necrotizing lymphadenitis

Objective: To explore the clinical features and prognosis of children with histiocytic necrotizing lymphadenitis (HNL). Methods: The clinical data of 118 children with HNL diagnosed and treated in the Department of Rheumatology and Immunology of Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2021 were retrospectively analyzed. The clinical symptoms, laboratory examination, imaging examination, pathological findings, treatment and follow-up were analyzed. Results: Among the 118 patients, 69 were males and 49 were females. The age of onset was 10.0 (8.0, 12.0) years, ranging from 1.5 to 16.0 years. All the children had fever lymph node enlargement, blood system involvement in 74 cases (62.7%), skin injury in 39 cases (33.1%). The main manifestations of laboratory examination were increased erythrocyte sedimentation rate in 90 cases (76.3%), decreased hemoglobin in 58 cases (49.2%), decreased white blood cells in 54 cases (45.8%) and positive antinuclear antibody in 35 cases (29.7%). Ninety-seven cases (82.2%) underwent B-mode ultrasound of lymph nodes, showing nodular lesions with low echo in the neck; 22 cases (18.6%) underwent cervical X-ray and (or) CT; 7 cases (5.9%) underwent cervical magnetic resonance imaging. Lymph node biopsy was performed in all 118 cases, and the pathological results did not support malignant diseases such as lymphoma or Epstein-Barr virus infection, suggesting HNL. Fifty-seven cases (48.3%) recovered without treatment, 61 cases (51.7%) received oral steroid therapy, and 4 cases (3.4%) received indomethacin as anal stopper. The 118 cases were followed up for 4 (2, 6) years, ranging from 1 to 7 years, 87 cases (73.7%) had one onset and did not develop into other rheumatological diseases, and 24 cases (20.3%) had different degrees of recurrence, 7 cases (5.9%) had multiple system injuries, and all of the tested autoantibodies were positive for medium and high titers. All of them developed into other rheumatic immune diseases, among which 5 cases developed into systemic lupus erythematosus and 2 cases developed into Sjogren's syndrome; 7 cases were given oral steroid therapy, including 6 cases plus immunosuppressant and 2 cases receiving methylprednisolone 20 mg/kg shock therapy. Conclusions: The first-onset HNL portion is self-healing, hormone-sensitive and has a good prognosis. For HNL with repeated disease and multiple system injury, antinuclear antibody titer should be monitored during follow-up, and attention should be paid to the possibility of developing into other rheumatological diseases, with poor prognosis.

Analysis of the clinical characteristics and therapeutic effect of refractory juvenile dermatomyositis to tofacitinib

Objective: To elucidate the clinical features of patients with refractory juvenile dermatomyositis (JDM), and to explore the efficacy and safety of tofacitinib in the treatment of refractory JDM. Methods: A total of 75 JDM patients admitted to the Department of Rheumatology and Immunology in Shenzhen Children's Hospital from January 2012 to January 2021 were retrospectively analyzed, and to analyze the clinical manifestations, efficacy and safety of tofacitinib in the treatment of refractory JDM. Patients were divided into refractory group with using of glucocorticoids in combination with two or more anti-rheumatic drugs for treatment, and the presence of disease activity or steroid dependence after a one-year follow-up. The non-refractory group is defined as clinical symptoms disappeared, laboratory indicators were normal, and clinical remission was achieved after initial treatment, and the clinical manifestations and laboratory indexes of the two groups were compared. The Mann-Whitney U test, Fisher's precision probability test was used for intergroup comparison. Binary Logistic multivariate regression analysis was used to identify risk factors for refractory JDM. Results: Among the 75 children with JDM, 41 were males and 34 were females with a age of onset of 5.3 (2.3, 7.8) years. The refractory group consisted of 27 cases with a age of onset of 4.4 (1.5, 6.8) years, while the non-refractory group consisted of 48 cases with a age of onset of 5.9 (2.5, 8.0) years. Compared with 48 cases in the non-refractory group, the proportion of interstitial lesions and calcinosis in the refractory group was higher than that in the non-refractory group (6 cases (22%) vs. 2 cases (4%), 8 cases (30%) vs. 4 cases (8%), both P<0.05). Binary Logistic regression analysis showed that observation group were more likely to be associated with to interstitial lung disease (OR=6.57, 95%CI 1.22-35.31, P=0.028) and calcinosis (OR=4.63, 95%CI 1.24-17.25, P=0.022). Among the 27 patients in the refractory group, 22 cases were treated with tofacitinib, after treatment with tofacitinib, 15 of 19 cases (86%) children with rashes showed improvement, and 6 cases (27%) with myositis evaluation table score less than 48 score both were improved, 3 of 6 cases (27%) had calcinosis were relieved, and 2 cases (9%) had glucocorticoid-dependence children were successfully weaned off. During the tofacitinib treatment, there was no increase in recurrent infection, blood lipids, liver enzymes, and creatinine were all normal in the 22 cases. Conclusions: Children with JDM with calcinosis and interstitial lung disease are more likely to develop refractory JDM. Tofacitinib is safe and effective for refractory JDM.

Is multimodal occupational therapy in addition to usual care cost-effective in people with thumb carpometacarpal osteoarthritis? A cost-utility analysis of a randomised controlled trial

ObjectiveThe aim was to evaluate the cost-utility of a 3-month multimodal occupational therapy intervention in addition to usual care in patients with thumb carpometacarpal osteoarthritis (CMC1 OA).MethodsA cost-utility analysis was...

The joint ultrasound markers in the early diagnosis of psoriatic arthritis

Objectives. Early establishment of arthritis in PsA based on clinical data and ultrasound examination. Material and methods. The study was conducted between 2019-2023, in the rheumatology and arthrology departments of the Timofei Moşneaga Republican Clinical Hospital, or treated in outpatient. In order to meet the requirements of the study, 100 people were examined, including 70 patients with PsA. Results. In patients with PsA the most common changes were an increase in the amount of intra-articular fluid and the proliferation of the synovial membrane. In total, fluid was detected in 293 out of 3,232 joints (9.1%). Conclusions. Ultrasound is a highly informative method in detecting a wide range of morphological changes in the joints of patients with PsA. The highest index of sensitivity appeared when inflammatory fluid, cartilage changes, osteophytes and tenosynovitis were detected.

An ultrasonographic study of gouty arthritis: Synovitis and its relationship to clinical symptoms: A retrospective analysis.

Joint pain is the main symptom of acute attacks in patients with gout, which if not managed properly, can develop into chronic gout. The aim of this study was to investigate the correlation between ultrasound(US) features of gouty arthritis (GA) and its clinical manifestations to provide a basis for diagnosing and evaluating the disease. We retrospectively analyzed 182 sites in 139 patients with GA diagnosed by the Rheumatology and Immunology Department. Degree of pain was evaluated using the visual analog scale (VAS). Patients with GA were divided into active and inactive arthritis groups. Statistical differences between the two groups and the correlation between US features and clinical manifestations of the affected joints in patients with GA were analyzed. The groups had statistical significance in joint effusion, power Doppler ultrasonography (PDS),double contour sign, and bone erosion (p = 0.02, 0.001, 0.04, 0.04, respectively). Correlation analysis in this study showed that joint effusion and PDS were positively correlated with degree of pain (r s = 0.275, 0.269; p < 0.001, <0.001, respectively). Additionally, PDS was positively correlated with synovitis, joint effusion, bone erosion, and aggregates (r s = 0.271, 0.281, 0.222, 0.281; p < 0.001, <0.001, 0.003, <0.001, respectively). Pathological US features, such as joint effusion, synovitis, PDS and bone erosion were more likely to be detected in GA with clinical signs and symptoms. PDS was positively correlated with joint effusion and synovitis, pain was closely related to PDS and joint effusion, which suggested that the clinical symptoms of GA were related to inflammation, reflecting the patient's condition to some extent. Therefore, musculoskeletal US is a useful clinical tool for managing patients with GA and can provide a reliable reference for diagnosing and treating GA.

Rheumatoid Arthritis or Imitator?

H syndrome is an autosomal recessive multisystemic disease with a very low prevalence rate, characterized by indurated cutaneous hyperpigmentation, hypertrichosis, and various systemic manifestations. A 37-year-old woman was referred to the rheumatology department with a prediagnosis of rheumatoid arthritis due to deformity in the hands and feet and mild sensorineural hearing loss since the age of 17.

Real-life drug retention rate and safety of rituximab when treating rheumatic diseases: a single-centre Swiss retrospective cohort study

BackgroundIn Switzerland, rituximab (RTX) is licenced for the treatment of rheumatoid arthritis (RA) and ANCA-associated vasculitis (AAV) but is frequently used off-label to treat other auto-immune diseases (AID), especially connective tissue diseases (CTD). We aimed to characterise the use of RTX in AID in a real-life Swiss setting and compare RTX retention rates and safety outcomes between patients treated for RA, CTD and AAV.MethodsA retrospective cohort study of patients who started RTX in the Rheumatology Department for RA or AID. The RTX retention rate was analysed using Kaplan–Meier survival curves. Occurrences of serious adverse events (SAE), low IgG levels and anti-drug antibodies (ADA) were reported.ResultsTwo hundred three patients were treated with RTX: 51.7% had RA, 29.6% CTD, 9.9% vasculitis and 8.9% other AIDs. The total observation time was 665 patient-years. RTX retention probability at 2 years (95%CI) was similar for RA and CTD 0.65 (0.55 to 0.73), 0.60 (0.47 to 0.72) and lower for vasculitis 0.25 (0.09 to 0.45). Survival curves for RTX retention matched closely (p = 0.97) between RA and CTD patients but were lower for patients with vasculitis due to a higher percentage of induced remission. Patients with vasculitis (95%) and CTD (75%) had a higher rate of concomitant glucocorticoid use than RA (60%). Moderate to severe hypogammaglobulinaemia was observed more frequently in patients with vasculitis (35%) than with RA (13%) or CTD (9%) and was associated with an increased risk of presenting a first infectious SAE (HR 2.01, 95% CI 1.04 to 3.91). The incidence rate of SAE was 23.3 SAE/100 patient-years (36% were infectious). When searched, ADAs were observed in 18% of the patients and were detected in 63% of infusions-related SAE. 10 patients died during RTX treatment and up to 12 months after the last RTX infusion, 50% from infection.ConclusionRTX retention rates are similar for patients with RA and CTD but lower for those with vasculitis due to more frequent remission. Patients treated with RTX for vasculitis present more SAE and infectious SAE than patients with RA and CTD, potentially due to a higher use of concomitant glucocorticoids and the occurrence of hypogammaglobulinaemia.