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"nBackground: Systemic Lupus Erythematosus (SLE) is a prototypic autoimmune disease with diverse clinical manifestations in association with autoantibodies to components of the cell nucleus. SLE as a chronic autoimmune disease has a worldwide distribution. There is a wide variation in the natural history of SLE among different ethnic and geographic groups. Our SLE registry is one of the largest series in Asia-Pacific region. The aim of this study was to show the manifestations of SLE in Iranian patients. "n"nMethods: This study is on clinical and Para clinical manifestations of SLE according to the database of the Rheumatology Research Center (RRC), Tehran University of Medical Sciences as a major referral center for rheumatic disease in Iran during the period of 1976 to 2009. "n"nResults: A total of 2143 SLE patients were studied. The female to the male ratio was 8.8:1 and the mean age at the presentation was 24.2± 10 Years. Prevalence of clinical manifestations included: musculoskeletal, cutaneous, renal, neuropsychiatric, pulmonary, cardiac and hematologic were 85.2%, 83.1%, 66.6%, 24%, 22.3%, 17.5% and 67.1% respectively. There were seen positive FANA in 78.3% and anti-DNA in 70% of patients. Overlap syndrome and positive family history with other autoimmune diseases were detected in 14.9% and 3.4% of patients respectively. "n"nConclusion: The prevalence of some manifestations (such as cutaneous and renal involvement) in our patients were similar to those of nearby countries (with similar climate), while other manifestations (such as hematologic and joint involvement) were similar to the European countries (with similar ethnicity). Genetic and/or climatic factors may lead to different presentations of lupus.

"nBackground: To evaluate the association between age, sex, BMI, waist/hip ratio, smoking, religion, ethnicity, education and knee osteoarthritis. "nMethods: Eligible subjects were randomly included from participants of Tehran COPCORD study, of whom 480 subjects with knee osteoarthritis were compared to 490 subjects without (case-control study). Using a questionnaire developed by COPCORD group (Asia & Oceania), we enquired about the risk factors of knee osteoarthritis i.e. age, sex, BMI, Waist/Hip ratio, religion, ethnicity, education and smoking. Knee osteoarthritis was defined using ACR criteria. Each knee was unit of analysis using GEE technique to evaluate these associations. "nResults: Age (OR; 1.096; CI95%: 1.091-1.1; P: 0.00) and sex (OR; 2.85; CI95%: 2.49-3.28; P: 0.00) showed significant association with knee osteoarthritis. Overweight (OR; 1.81; CI95%: 1.28-2.55; P: 0.00) and obesity (OR; 3.3; CI95%: 2.34-4.66; P: 0.00) both showed higher risk for knee osteoarthritis. The association between waist/hip ratio and knee osteoarthritis showed an OR of 5.28, CI95%: 0.89-31.44; P: 0.07. However, this association was only borderline significant. People with different religion or ethnicity and smokers had no extra risks for knee osteoarthritis. Higher education is a protective factor for knee osteoarthritis as people who had university education compared to people with no/primary education showed a lower risk for knee osteoarthritis (OR; 0.54; CI95%: 0.38-0.78; P: 0.00). "nConclusions: Our study confirmed that elderly, females, overweight and obese people are at higher risk to develop knee osteoarthritis as found in western societies. Higher education is a protective factor against knee osteoarthritis. Ethnicity, religion and smoking showed no extra risk of knee osteoarthritis.

Background: The complicated autoimmune disease known as systemic lupus erythematosus (SLE) is characterized by abnormal immunological B cell modulation that exhibits enhanced survival. The genes Tp53, MDM2, and CDKN2A (ARF) play crucial roles in cellular regulation. In this study, we aim to elucidate the status of these genes. This might be a factor in the B cells' higher survival rate in SLE., Methods: B cells were obtained from the peripheral blood of participants and cultured, with a subset of the cells activated using anti-IgM. The expression levels of Tp53, MDM2, and CDKN2A genes were assessed both at baseline and after activation., Results: Tp53 and CDKN2A exhibited decreased expression in both baseline and activated B cells, with further decreases observed active and inactive SLE. Conversely, MDM2 demonstrated increased expression in baseline B cells, with a more pronounced increase observed in activated B cells of active SLE. Correlation analysis indicated several significant positive and negative associations, particularly between disease activity indicators and MDM2 expression, as well as among the studied genes., Conclusion: The dysregulation of the CDKN2A-MDM2-p53 axis in B cells of SLE may lead to increased B cell survival due to reduced Tp53 and CDKN2A expression and elevated MDM2 levels. In addition, MDM2 gene expression levels show a significant positive correlation with disease activity indices like anti-dsDNA titer and SLEDAI score in SLE patients., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2025. Published by Elsevier B.V.)

Non-traumatic avascular necrosis of the femoral head (AVNFH) is a progressive condition that often affects young individuals, leading to bone death and eventual collapse. This degeneration is causing pain and limited function in daily activities over time. This study will plan to enrol 53 male and female participants, aged 18-50 years, who have been diagnosed with AVNFH. Participants will be randomly assigned to one of two groups: (1) the Exe+Functional group , which will receive 12 weeks of mixed therapeutic exercises combined with functional resistance exercises and self-management education and (2) the control group , which will receive the same interventions but without functional exercises. The Exe+Functional group will engage in two times per week sessions of low-intensity resistance, and mobility exercises, along with a one time per week functional exercise session. The control group will have 3 weekly sessions of low-intensity exercises (resistance and mobility) without functional exercises. The primary outcomes of the study will be pain, measured using a Visual Analogue Scale (VAS) and functional pain, assessed using a Numeric Rating Scale (NRS). Secondary outcomes will include the Harris Hip Score, the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) for hip osteoarthritis, the Timed Up and Go (TUG) test, the 30 s Chair Stand Test, active range of motion, muscle strength and the 10-metre walk test. Data will be collected at baseline (preintervention), postintervention at 12 weeks and at a 4 month follow-up. Trial registration number: IRCT20220510054814N2., Competing Interests: None declared., (Copyright © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by dysregulated immune responses and multi-organ involvement. Dysregulation of apoptosis, a key process for maintaining immune homeostasis, plays a critical role in the pathogenesis of SLE. MicroRNAs (miRNAs), small non-coding RNAs that regulate gene expression, have emerged as important modulators of apoptosis in immune cells, influencing the balance between immune tolerance and autoimmunity., Objectives: This review aims to comprehensively summarize recent advancements in understanding the roles of miRNAs in apoptosis regulation within immune cells in SLE, highlighting their therapeutic potential for restoring immune balance and mitigating disease progression., Results: Aberrant expression of specific miRNAs contributes to the dysregulation of apoptosis in SLE immune cells. Pro-apoptotic miRNAs, such as miR-125b and miR-150, are often downregulated, leading to enhanced survival of autoreactive immune cells. Conversely, anti-apoptotic miRNAs, including miR-21, are upregulated, further disrupting the delicate balance of immune cell apoptosis. Dual-function miRNAs, such as miR-155, exhibit context-dependent roles based on cellular environments and target gene interactions. This dysregulation promotes the persistence of autoreactive immune cells and the development of autoimmunity., Conclusions: miRNAs play critical roles in modulating apoptosis pathways, making them promising therapeutic targets for SLE. Restoring the balance of pro-apoptotic and anti-apoptotic miRNAs could help reinstate immune tolerance and reduce tissue damage. Future research should focus on elucidating miRNA targetomes, improving delivery systems, and addressing off-target effects to fully harness their therapeutic potential., (© 2025 The Author(s). Immunity, Inflammation and Disease published by John Wiley & Sons Ltd.)

Background: In patients with Systemic lupus erythematosus (SLE), osteonecrosis of various joints is a debilitating complication associated with the disease and its treatment, in which a considerable proportion of osteonecrosis may be asymptomatic. Recognizing the crucial role of early and timely detection, as well as appropriate management of asymptomatic osteonecrosis, in preventing joint destruction, we conducted a study to evaluate the prevalence of asymptomatic osteonecrosis in SLE patients who have already been diagnosed with symptomatic osteonecrosis. Additionally, we aimed to examine the relationship between proposed risk factors of osteonecrosis and the development of asymptomatic osteonecrosis., Methods: In this cross-sectional study, Patients with recently diagnosed symptomatic osteonecrosis of at least one joint were selected by reviewing data from the digital medical record system of the Rheumatology Research Center. The patients underwent three-phase Single Photon Emission Computed Tomography (SPECT) bone scintigraphy to screen for other asymptomatic osteonecrotic joints. MRI was subsequently performed on the asymptomatic osteonecrotic sites for further diagnostic confirmation. The study evaluated the prevalence of asymptomatic osteonecrosis, the extent of joint involvement, the specific locations of osteonecrosis, the most commonly affected joints, and the risk factors for asymptomatic osteonecrosis., Results: Eight out of the 17 patients (47%) who participated in our research were found to have asymptomatic osteonecrosis. The most commonly affected joint without symptoms was the left knee (25%), while the most frequently affected joint with symptoms was the left hip (23.07%). The only statistically significant difference observed between patients with and without asymptomatic osteonecrosis in this study was the age at which the disease first appeared (p = 0.046) and this age was higher among patients with asymptomatic osteonecrosis., Conclusions: Our research provides further evidence of the high incidence of asymptomatic osteonecrosis in individuals with SLE due to the nature of the disease and the frequent use of high-dose corticosteroids. It underscores the importance of early detection through whole-body SPECT bone scintigraphy and MRI, as well as prompt intervention in order to avert the incapacitating effects of osteonecrosis., Competing Interests: Declarations. Ethics approval and consent to participate: The study was conducted in accordance with the ethical principles outlined in the Declaration of Helsinki and received approval from the ethics committee of Tehran University of Medical Sciences (IR.TUMS.MEDICINE.REC.1400.1003). Informed consent was obtained from all patients who participated in the study, allowing their inclusion. Consent for publication: The informed consent included the provision for the possibility of publishing the research results. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Background: Systemic lupus erythematosus is a multisystemic rheumatic disease with different clinical features. Disturbance in apoptosis regulation seems to be a major factor in SLE development., Objective: Survivin plays a key role in mitosis and inhibiting apoptosis. A study was conducted to examine the expression level of survivin and miRNAs that affect survivin transcript levels in patients with SLE., Methods: We isolated peripheral blood mononuclear cells from 50 inactive SLE patients and 50 healthy controls. RNA is extracted and converted to cDNA. The quantitative real-time polymerase chain reaction is conducted to assess the expression levels of survivin total and its variants with effective miRNAs in PBMCs., Results: Expression levels of miR-34a-5p (fold change = 1.5, p + + = 0.027), and 218-5p (fold change = 1.5, p + + = 0.020) were significantly increased. While miR-150-5p (fold change = 0.56, p + + = 0.003) was significantly decreased. The mRNA expression of survivin-WT (fold change = 0.63, p + + = 0.002) was significantly downregulated in SLE patients compared to the healthy controls. Survivin total and its two major variants (survivin-2B, and survivin-ΔEx3) did not differ significantly between SLE patients and controls., Conclusion: Although survivin-TS and its two variants (survivin-2B, and survivin-ΔEx3) were not differently expressed in SLE patients, survivin-WT had altered expression. Despite aberrant miRNA expression in PBMCs from SLE patients, survivin and miRNA expression were not associated with leukopenia. The pathogenesis of SLE disorder might be linked to survivin's other roles in the immune system aside from anti-apoptotic functions., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Key Clinical Message: Malignancy may be a possible cause of systemic lupus erythematosus (SLE) flare-ups, and it is necessary to consider it in the context of treatment resistance. In this case, we present a challenging instance of concomitant nodal marginal zone B-cell lymphoma (NMZL) and SLE flare-up in a 41-year-old male patient., Abstract: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can cause various symptoms and affect multiple organs in the body. It is also associated with the development of malignancies, especially lymphomas. This case report discusses a patient who experienced a flare-up of SLE along with hypercalcemia, which led to the diagnosis of nodal marginal zone B-cell lymphoma (NMZL). This is the first case of its kind to be reported. A 41-year-old man with a 10-year history of SLE and antiphospholipid syndrome (APS) was referred to our center due to several symptoms, including fatigue, oral lesions, dyspnea, bilateral wrist pain and inflammation, mild pericardial effusion, organ enlargement, pancytopenia, high erythrocyte sedimentation (ESR) level, high anti-double stranded DNA (anti-dsDNA) level, low complement level, resistant hypercalcemia, and high brain natriuretic peptide (pro-BNP) level. After further testing, it was discovered that the patient had NMZL, which was the ultimate diagnosis. He underwent six cycles of the R-CHOP chemotherapy regimen, and his clinical and laboratory conditions improved during follow-ups. The initial case of SLE flare-up, with concomitant NMZL is being reported as the final diagnosis. In simpler terms, it is possible for lymphoma to manifest as a potential cause of SLE flare-ups, and clinicians should be mindful that they need to consider malignant conditions when faced with treatment resistance., Competing Interests: The authors declare that they have no competing interests., (© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.)

Objectives: The aim of this study was to culturally adapt and assess the validity and reliability of the Exercise Adherence Rating Scale (EARS) in Persian language for patients with knee osteoarthritis., Methods: The original English version of the EARS was forward-translated to Persian (by an expert and a non-expert in the field of exercise and health science) and then backward-translated to English by two people, and then by a committee of five, pre-final Persian version of EARS was created. Patients were provided with a three-month exercise program, three times a week, through telerehabilitation. After completion of the exercise program, patients filled out the Persian version of EARS and the Scanlan questionnaire. Three weeks later, patients completed the EARS and Scanlan questionnaire again. During the study, patients recorded the number of exercise sessions weekly in a standardized diary form. Face validity was assessed by ten patients, using the item impact method. Content validity was assessed by five experts and quantified using the content validity ratio and content validity index. Agreement between EARS and Scanlan questionnaire was assessed using Spearman test and Bland-Altman plot. The reliability of the Persian version of EARS was assessed using the intraclass correlation coefficient and Cronbach's α., Results: A total of 30 patients (3 men, 27 women), with a mean age of 59 years (SD ​= ​10), participated in this study. All items of the Persian version of EARS had item impact method scores above 1.5, indicating acceptable face validity. The scale-content validity index/average for relevancy and simplicity components were calculated as 0.87 and 0.85, respectively, indicating good content validity. Bland-Altman plot showed good agreement between EARS and Scanlan questionnaire at baseline and three weeks later. Cronbach's alpha was 0.96, indicating excellent internal consistency. The intraclass correlation coefficient (95% CI) was 0.996 (0.991, 0.998), indicating excellent reliability., Conclusions: The Persian version of EARS demonstrated acceptable cultural adaptation, reliability, and validity in patients with knee osteoarthritis. The use of the Persian version of EARS can be a reliable and valid tool to assess exercise adherence in patients with knee osteoarthritis., Level of the Evidence: II., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Osteonecrosis is a major cause of morbidity for patients with systemic lupus erythematosus (SLE). Although core decompression is an approved and trusted technique to prevent further joint deterioration, this surgical method seems to be less beneficial for SLE patients. We aimed to evaluate the outcomes of core decompression in SLE patients with primary stages of femoral head osteonecrosis., Methods: In this study, 23 patients (39 affected hip joints) with osteonecrosis of the femoral head with stage II of the disease, based on the Ficat-Arlet classification system, underwent core decompression. Also, patients demographic characteristics, clinical data, medication history, comorbidities, immunological findings, hip plain radiographs, history of total hip arthroplasty after core decompression, and patients satisfaction with joint function according to the Oxford hip score questionnaire were obtained., Results: In the study, 53.8% of affected joints showed signs of radiographic deterioration in follow-up imaging. Sixty-one and a half percent (61.5%) of patients had unsatisfactory joint performance. A third (33.3%) of affected hip joints underwent total hip arthroplasty up to 5 years from core decompression. SLE patients with a history of receiving bisphosphonate were 83.2% less dissatisfied with their joint function than patients without a history of bisphosphonate use (P < 0.02). Of the 23 studied cases, the mean cumulative dose of prednisolone before and after core decompression surgery was 46.41 mg and 14.74 mg respectively. Besides, one case (2.6%) that had a high anti-phospholipid antibodies level during follow-up did not have any radiographic deterioration, and 9 cases (23.1%) had some degrees of radiographic deterioration., Conclusions: The patients group that used bis-phosphonate, had a higher level of satisfaction with joint function after core decompression. Patients with high-level anti-phospholipid antibodies are related to a poor prognosis after core decompression., (© 2023. The Author(s).)

Background: Dermatomyositis (DM) is a systemic autoimmune disease characterized by distinct skin lesions and a clinically heterogeneous constellation of systemic manifestations. This disease poses a challenge to clinicians because of its rarity, diverse clinical presentations, and variable organ involvement, resulting from an autoimmune attack on affected organs, which could be triggered by environmental factors in genetically susceptible individuals. Renal involvement is rare, with immunoglobulin M (IgM) nephropathy yet to be reported in patients with DM., Case Presentation: A 38-year-old man was admitted to Shariati Hospital, affiliated with Tehran University of Medical Sciences, with proximal weakness of the upper and lower extremities that had developed in the preceding month after receiving the Sinopharm COVID-19 vaccine. The patient was diagnosed with DM based on the heliotrope rash, Gottron's papules, progressive proximal muscle weakness, and paraclinical findings. IgM nephropathy developed subsequently, diagnosed by light and immunofluorescence microscopy., Conclusion: We describe the first case of IgM nephropathy in a DM patient following COVID-19 vaccination. This phenomenon requires further investigation into the possible crosslinks between the pathogenesis of IgM nephropathy with DM and the COVID-19 vaccine. Diagnosing renal complications in DM patients promptly and accurately can help to achieve the best outcomes., (© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objectives: Systemic lupus erythematosus (SLE) with a high morbidity rate (7% in general and 325/100.000 in reproductive period) was known as "the mother of autoimmune diseases." But healthcare providers hadn't reliable scales to measure the effectiveness of interventions to improve reproductive health. Women with systemic lupus erythematosus (SLE) express concern about a significant gap in their reproductive and sexual health during medical care due to the lack of a specific scale to measure this concept. This study developed psychometrically specific scale to evaluate this concept in Iranian women., Methods: The study utilized a sequential-exploratory mixed-methods design, wherein the concept of reproductive health was clarified during the qualitative phase using conventional content analysis (inductive-deductive) to create a pool of RHASLE (Reproductive Health Assessment in SLE) items. During the psychometric process, the face, content, and construct validities of the RHASLE were checked with 650 individuals (320 using exploratory factor analysis [EFA] and 330 using confirmatory factor analysis [CFA]) through interviews and completion of the questionnaire. Convergent and divergent validities were then checked, and the reliability was assessed through stability and internal consistency. Finally, the measurement error, responsiveness, and interpretability of the scale were evaluated and confirmed., Results: The study's findings revealed that the RHASLE consisted of 5 factors (33 items): physical-psychological disorder (13 items), relaxing feeling (6 items), spirituality (3 items), high-risk pregnancies (5 items), and sexual satisfaction (6 items). These factors explained a total of 99.97% of the variance in the concept of reproductive health. Confirmatory factor analysis confirmed the good model fit, and its validity and reliability were deemed acceptable., Conclusion: The RHASLE consisted of 27 items with high internal consistency, stability, responsiveness, and interpretability. That was able to get approval of Consensus-Based Standards for the Selection of Health Measurement Instruments (COSMIN) checklist. Based on the results, RHASLE is a valid and reliable multidimensional scale that encompasses various aspects of reproductive health (physical, sexual, psychological, social, and spiritual)., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Behcet disease (BD) as a variable vessel vasculitis is mainly characterized by ocular involvement, genital and oral aphthosis, and erythema nodosum. However, major organ involvements including gastrointestinal involvement, nervous system, and vascular involvement are among the severe complications. Osteonecrosis is a rare complication of patients with BD. We aim to report the largest series of BD patients suffering from osteonecrosis., Methods: We have retrospectively reviewed all patients in Iran Behcet's Disease Registry and reported those with osteonecrosis. Patients' medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed., Results: Seven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated., Conclusions: ON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated., (© 2023. The Author(s).)

Aim: Avascular necrosis (AVN) or osteonecrosis is characterized by death of bone tissue due to endothelial damage and vascular abnormality. Coronavirus can induce endothelial damage and abnormal blood clotting, so that COVID-19 is known as a vascular disease. We aim to evaluate the relationship between AVN and COVID-19., Case: Here we present a 39-year old man with severe COVID-19 and corticosteroid consumption who developed late onset AVN of both hips 20 month after COVID-19., Conclusion: An awareness of the possible osteonecrosis for all physicians dealing with patients with musculoskeletal problems following COVID-19 is necessary., (© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objectives: Eye involvement is a main presentation of Behcet's disease. This study was performed to evaluate possible determinants affecting the occurrence of eye involvement, especially the role of early systemic treatment with immunomodulatory drugs on the incidence of ocular involvement., Methods: This is a retrospective cohort study performed on 1166 Behcet's patients in the Behcet's Clinic of Rheumatology Research Center. All patients were followed up for at least 10 years and a maximum of 15 years. Data analysis was performed using survival analysis models including Kaplan-Meier Survival analysis, Logrank test, and Cox's proportional hazards regression., Results: 1166 Behcet's patients were evaluated. 80 patients who had eye involvement as the first manifestation of the disease were excluded and 1086 participants entered the analysis. Among them, 647 patients (59.6%) developed ocular involvement 7.8 ± 6.7 years after the first symptom. Immunomodulatory treatment before ocular involvement reduced the risk by 3 times (P-value <0.001)., Conclusion: This study demonstrated that the initiation of immunomodulatory treatment prior to eye involvement can reduce the risk of eye involvement in Behcet's patients. Therefore, reducing the onset time of disease symptoms and providing appropriate treatment can reduce Behcet's disease ocular complications., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 The Authors.)

Introduction: This study aims to investigate whether a pain neuroscience education (PNE) combined with a blended exercises programme including aerobic, resistance, neuromuscular, breathing, stretching and balance exercises and diet education provides greater pain relief and improvement in functional and psychological factors than PNE and blended exercises alone and whether 'exercise booster sessions (EBS)' approach may improve outcomes and increase adherence in patients with knee osteoarthritis (KOA) (by telerehabilitation (TR))., Methods and Analysis: In this single-blind randomised controlled trial, 129 patients (both genders; age >40) diagnosed with KOA will be randomly assigned to one of the 2 2 treatment combinations as: (1) blended exercises alone (36 sessions over 12 weeks), (2) PNE alone (three sessions over 2 weeks), (3) combination of PNE with blended exercises (exercise three times a week for 12 weeks combined with three sessions of PNE) and (4) a control group. Outcome assessors will be blinded towards group allocation. The primary outcome variables are the visual analogue scale (VAS) and Western Ontario and McMaster Universities Osteoarthritis index in knee osteoarthritis (WOMAC) score. Secondary outcomes include Pain Self-Efficacy Questionnaire (PSEQ), Depression, Anxiety and Stress Scale (DASS), Tampa Scale for Kinesiophobia (TSK), Short Falls Efficacy Scale International (FES-I), Pain Catastrophising Scale (PCS), Short Form Health Survey (SF-12) and Exercise Adherence Rating Scale (EARS), 30 s sit-to-stand test (30CST), Timed Up and Go (TUG), lower limbs' muscle strength and lower limb joints' active range of motion (AROM) will be performed at baseline, 3 and 6 months' postinterventions. The primary and secondary outcomes will assess at baseline, 3 months and 6 months postinterventions.The findings will be useful in establishing an effective treatment strategy covering multiple aspects behind KOA. The study protocol is conducted in clinical settings, thereby enhancing the possibility of future implementation of the treatments in the healthcare systems and self-care management. Results in comparison between groups will help to clarify the most effective of mixed-method TR (blended exercise, PNE, EBS with diet education) on more improvement in pain, functional and psychological factors in patients with KOA. This study will combine some of the most critical interventions, to be able to introduce a 'gold standard therapy' in the treatment of KOA., Ethics and Dissemination: The trial has been approved by the ethics committee for research involving human subjects of the Sport Sciences Research Institute of Iran (IR.SSRC.REC.1401.021). The study findings will be published in international peer-reviewed journals., Trial Registration Number: IRCTID: IRCT20220510054814N1., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Systemic Lupus erythematosus (SLE) is a multisystem autoimmune disease. This disease triggers sexual dysfunction due to physiological, cognitive and mental effects. Since sexual function is often ignored in these patients, this study aimed to investigate the effect of sexual counseling based on EX‑PLISSIT model on improving the sexual function of married women with SLE. This clinical trial was conducted on 101 married women suffering from SLE (18-49 years) residing in Tehran. Randomization was performed by random block allocation with six blocks in a rheumatology clinic. A demographic questionnaire, the Depression, Anxiety, Stress Scales-21 Items (DASS-21), and the female sexual function index were used to collect the data. The intervention group received counselling sessions every week while the control group (n = 55) received routine care for the disease. Eight and twelve weeks after completion of counselling sessions, outcomes compared between the intervention and control groups, using independent ttest, paired t-test, repeated measures and Chi-squared test. The scores of sexual function in both groups did not differ significantly before the intervention ( p  > 0.05). At the first follow-up session, the score of all sexual function domains except sexual pain increased significantly in the intervention group while a significant reduction was observed in all domains of sexual function in the control group ( p  < 0.05). In the second follow-up, sexual function significantly increased in the intervention group whereas it significantly decreased in the control group ( p  < 0.05). According to the findings of this study, counseling based on EX-PLISSIT model positively affected the sexual function of women with SLE. Therefore, this model can be used as a cost-effective and simple counseling method to improve the sexual functions., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest., (© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023, Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Introduction: Systemic lupus erythematosus (SLE) is a multisystem disorder that can affect multiple organs; psychiatric manifestations including depression and anxiety are commonly seen in SLE. The aim of this study is to explore the prevalence of depression, anxiety, and stress, and assess the quality of life (QOL) in patients with SLE and also evaluate associated risk factors., Material and Methods: In this cross-sectional study, adult patients with SLE were identified through our institution's SLE data registry. Participants were evaluated with three questionnaires: Depression, Anxiety, and Stress Scale (DASS-42), General Health Ouestionnaire-28 (GHQ-28), and World Health Organization quality of life instrument short form (WHO-QOL BREF)., Results: A total of 222 patients were included in the study, 203 (91%) of whom were female and 19 were male (9%). Participants had a mean age of 35.6 ±9.5 years. According to DASS-42 questionnaire, 22.1%, 28.7% and 20.3% of patients had varying degrees of depression, anxiety, and stress, respectively. Based on GHQ-28 questionnaire, 137 (62%) of patients reported some degree of distress. Quality of life score was 12.8, 13, 14.3, and 13.9 in physical health, psychological health, social relationships, and environmental health, respectively., Conclusions: We found that depression, anxiety, and stress are common in patients with SLE, and quality of life is significantly affected. A high percentage of patients with SLE deal with some degree of distress. Routine evaluation of the quality of life and psychological disturbances is recommended in patients with SLE. Non-pharmacological interventions as well as specialist referral should be considered in patients with anxiety, depression, or stress., Competing Interests: The authors declare no conflict of interests., (Copyright: © 2023 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie.)

Background: The present, open-labeled study aimed to compare the distal approach (DA) for local corticosteroid injection (LCI) with the conventional proximal approach (PA) in alleviating the symptom and improving the electrodiagnostic parameters of the patients with carpal tunnel syndrome (CTS)., Methods: A total of 60 participants with nonsevere CTS were included in the present randomized controlled trial (RCT), of which 29 and 31 were assigned to the DA and PA groups, respectively. Each group received a single, landmark-guided injection of local methylprednisolone. The participants were assessed preintervention and 3 months later using the measures of visual analog scale (VAS), Boston Carpal Tunnel Questionnaire (BCTQ), hand grip strength, and nerve conduction study (NCS)., Results: Following a 3-month follow-up, both groups had significant improvements in VAS, both functional and severity subscales of BCTQ, hand grip strength, and some electrodiagnostic parameters (all P -values < .05). Moreover, the DA group had a significantly lower procedure duration than the PA group (9.80 ± 1.12 vs. 27.61 ± 1.77; P < .001)., Conclusions: LCI using the DA should be considered a feasible, safe, and effective therapeutic method in patients with mild to moderate CTS. It had a shorter procedure duration than conventional PA, while their clinical and electrophysiological results were similar.

Takayasu arteritis (TA) is an extremely uncommon vasculitis that primarily affects the aorta and its branches. Due to the genetic and ethnicity effect, a diverse array of TA clinical manifestations has been reported worldwide. The purpose of the present study was to compare the clinicodemographic characteristics and pattern of vascular involvement of Iranian and Turkish TA patients. This study was a retrospective, cross-sectional investigation of 126 TA patients in Iran and Turkey. All of the variables analyzed were extracted from historical medical records. In 126 TA patients, the ratio of females to males was 8.6:1, and the average age at onset of disease was 30.5±11.1 years. Fatigue (49.2%) and a weak or absent pulse (79.4%) were the most prevalent symptoms and signs, respectively. The most prevalent angiographic classifications were types V and I in Iranian patients (41.09%) and type I in the Turkish population (47.7%) The left subclavian artery was the vessel most frequently affected by TA (66.6%). Our findings indicated that there were no significant differences between the two countries in terms of clinicodemographic characteristics or vascular involvement. Some clinical manifestations, such as claudication, were more prevalent in the Turkish population due to a higher incidence of occlusive lesions in the right subclavian artery.

Background: Severe acute respiratory syndrome coronavirus 2 may be associated with late-onset necrotizing myositis, mimicking autoimmune inflammatory myositis; however, the exact underlying pathogenesis of severe acute respiratory syndrome coronavirus 2-induced myositis is still unclear., Case Presentation: Herein, we report a rare case of necrotizing autoimmune myositis in a 67-year-old middle eastern male following coronavirus disease 2019 infection, who presented with muscle weakness. The patient had positive anti-NXP2. The diagnosis of necrotizing autoimmune myositis was made according to muscle weakness, increased liver enzymes, electromyography and nerve conduction velocity results, and muscle biopsy. The patient underwent a full malignancy evaluation, which was unremarkable, and was discharged in relatively well condition with a daily dose of 1 mg/kg prednisolone and azathioprine 150 mg (2 mg/kg)., Conclusion: Our report highlights the already known possible protracted sequence of coronavirus disease 2019 infection and the potential for delayed-onset necrotizing myositis., (© 2022. The Author(s).)

Objective: Knee osteoarthritis (KOA) is one of the growing health problems with a considerable burden. With recent research on the possible effectiveness of antioxidants in the remission of KOA symptoms, a systematic review and meta-analysis was required to confirm this hypothesis., Design: Literature studies were searched on the most comprehensive databases such as PubMed, International Scientific Indexing, and Scopus, with no language and time restrictions. On 17 July 2021, a search strategy was developed based on the roots of "osteoarthritis (OA)" and "antioxidants," with no time or language limitations. As the primary outcome, pain was evaluated based on all indicators for evaluating pain [e.g., Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) pain scores, the visual analog scale (VAS), and the numerical rating scale (NRS)]. The symptoms and functions of KOA and quality of life (QOL) were also considered as secondary outcomes, each of which was measured and reported by the corresponding instrument in the studies. To measure the changes in pain, symptoms, and functions of participants, we included randomized controlled trials with a placebo control or other medical therapeutic interventions. Publication bias was assessed using Begg's funnel plot and Egger's regression test, which was deemed to be statistically significant at 0.1, and the results were checked by the trim-and-fill test., Results: After refinement, data were extracted from 31 documents from 7,698 primary searched papers. Using the VAS as a reliable psychometric measuring instrument, the present study revealed that a significant difference in the characteristics of disease-related symptoms of patients with KOA was reached after antioxidant therapy (standardized mean difference (SMD): 0.467, 95% confidence interval (CI): 0.303-0.632, p < 0.0001). The results reported by WOMAC confirmed no significant difference in the combined score, difficulty score, pain score, and stiffness score., Conclusion: As the first comprehensive systematic review of the association between antioxidant supplementation and KOA, this study showed that antioxidants can decrease disease-related symptoms in patients with KOA. The results can be useful for health policy decisions and future related studies., Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display&#95;record.php?ID=CRD42022351060, identifier: CRD42022351060., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Nejadhosseinian, Djalalinia, Haerian, Alikhani, Mansour, Mousavian, Mardani-Fard, Kasaeian and Faezi.)

A 55-year-old lady with a nine-year history of controlled sarcoidosis developed vasculitis after Sinopharm COVID-19 vaccine (BBIBP- CorV). She was ultimately diagnosed with mononeuritis multiplex based on EMG-NCV findings and administered methylprednisolone and cyclophosphamide pulse therapy for 5 days, and then continue with prednisolone and a monthly pulse of cyclophosphamide., (© 2022 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Background: Trigger finger is the most common flexor tendinopathy affecting the general population. We evaluated the effects of adding a static metacarpophalangeal joint splint to corticosteroid injection for the management of trigger finger in the short term., Methods: We carried out a randomized controlled trial with two parallel arms in Department of Physical Medicine and Rehabilitation at a university hospital. We randomly allocated 60 participants (34 women) with trigger fingers other than the thumb to two groups (both n = 30). The mean (SD) age was 41.5 (7.6) years. All participants received a single injection of 40 mg methylprednisolone plus 0.5 ml of lidocaine at the A1 pulley. Patients in the splint group wore a full time static splint for blocking the metacarpophalangeal joint for 3 months. The primary outcome was the Numerical Pain Rating Scale and the secondary outcomes were Boston questionnaire scores for symptom severity and functional status, grip strength, and the stages of stenosing tenosynovitis. We measured the outcomes at baseline, and in 1 and 3 months post-intervention., Results: Both interventions were effective; however, the splint group showed more reductions in pain (p = 0.013) and symptom severity (p = 0.047) and a larger decrease in the stages of tenosynovitis (p = 0.004) after 3 months. There was no significant difference in decreasing functional scores between the groups (p = 0.162). The splint group had a better (but not statistically significant) restoring grip strength (p = 0.056)., Conclusion: Wearing of a static metacarpophalangeal joint splint for 3 months following a single injection of corticosteroid increases and stabilises the benefits of the treatment for trigger finger., (© 2022 John Wiley & Sons Ltd.)

Complement dysfunction results in impaired ability in clearing apoptotic cell debris that may stimulate autoantibody production in systemic lupus erythematosus (SLE). Herein, we provided a comprehensive search to find and meta-analyze any complement gene polymorphisms associated with SLE. The ITGAM, C1q, and MBL gene polymorphisms were included in this meta-analysis to reveal the exact association with SLE risk. Electronic databases, including Scopus, PubMed, and Google Scholar, were searched to find studies investigating the ITGAM, C1q, and MBL gene polymorphisms and SLE risk in different populations. The pooled odds ratio (OR) and its corresponding 95% confidence interval (CI) were used to analyze the association between ITGAM, C1q, and MBL gene polymorphisms and susceptibility to SLE. According to inclusion criteria, a total of 24 studies, comprising 4 studies for C1QA rs292001, 5 studies for C1QA rs172378, 9 studies for ITGAM rs1143679, 8 studies for MBL rs1800450, 3 studies for MBL2 rs1800451, and 3 studies for MBL2 rs5030737, were included in the final meta-analysis. A significant positive association was found between rs1143679 and SLE risk, while rs1800451 significantly associated with decreased SLE susceptibility. In summary, ITGAM gene rs1143679 SNP and MBL gene rs1800451 SNP were positively and negatively associated with SLE risk, respectively., (© 2021. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Objective: Systemic lupus erythematosus (SLE), the most common form of lupus, is a multisystemic rheumatic disease with different clinical features that generally affect women of childbearing age. The common symptoms of SLE are very similar to other autoimmune and non-autoimmune disorders, thereby it is known as a thousand faces disease. In this article, we are going to discuss some of the most updated information about immune system-related factors, cells, and cytokines involved in SLE pathogenesis., Methods: Different electronic databases, especially PubMed/MEDLINE, Scopus, and Google Scholar, were searched to review and analyze relevant literature on the role of innate and adaptive immune cells and cytokines in the pathogenesis of SLE. A search for relevant literature was accomplished using various keywords including systemic lupus erythematosus, apoptosis, autoantibodies, immunopathogenesis of SLE, adaptive and innate immune cells, inflammatory cytokines, hormones, etc. RESULTS AND CONCLUSION: The most important characteristic of SLE is the production of antibodies against different nuclear autoantigens like double-strand DNA and RNA. The depositions of the immune complexes (ICs) that are generated between autoantibodies and autoantigens, along with aberrant clearance of them, can lead to permanent inflammation and contribute to tissue or organ damage. Related mechanisms underlying the initiation and development of SLE have not been clarified yet. Although, defects in immune tolerance, enhanced antigenic load, hyperactivity of T cells, and inappropriate regulation of B cells contribute to the pathogenic autoantibodies generation. Besides, sex hormones that influence the immune system seem to act as triggers or protectors of SLE development., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Introduction: Dermatomyositis (DM) and polymyositis (PM) are known as two major types of idiopathic inflammatory myopathies (IMMs). During the past years, growing data strongly suggest the clinical significance of myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs). The present study aimed to determine the profile of MSAs and MAAs, subsequently to address the clinical significance of these autoantibodies in Iranian myositis patients., Methodology: In this cross-sectional study, 28 DM and 24 PM patients were entered. Demographic and clinical characteristics were collected by direct examination and patients' medical record. The existence of MSAs and MAAs was assessed by indirect immunofluorescence then using immunoblotting (FA 1510-1005-1, DL 1530-1601-4 G; Euroimmun, Germany). Data were analyzed using the SPSS software (v22; SPSS Inc. Chicago, IL, USA)., Results: The mean age of the patients was 46.18 ± 12.95 years and male/female ratio was 28.8/71.2. Autoantibodies were positive in 63.46% of myositis patients. Interestingly, anti-TIF1γ and anti-PL7 were significantly associated with malignancy (P < 0.001, P = 0.008; respectively). The existence of autoantibody and anti-Jo1 had significant relation with interstitial lung disease (ILD) (P = 0.034, P = 0.006; respectively). Joint involvements including arthritis and arthralgia were significantly associated with anti-Ro52 and anti-Jo1 (P = 0.04, P = 0.02; respectively)., Conclusion: Taken together, it can be concluded that certain myositis autoantibodies present clinical significance which is in line with the literature. The use of these autoantibodies as biomarkers by line blotting along with indirect immunofluorescence facilitates diagnosis of inflammatory myopathies and makes it more accurate as well as better management of myositis patients if used based on a science-based manner. Key Points • Identification of MSAs and MAAs facilitates the diagnosis of inflammatory myopathies and provides better myositis patient's management if used according to a science-based manner. • Anti-rod and ring antibody was detected in a patient with ovarian cancer-induced dermatomyositis. • Malignancy and ILD are integrated parts of myositis which can be associated with MSAs and MAAs., (© 2021. International League of Associations for Rheumatology (ILAR).)

Background: The purpose of this study was to determine the prevalence of musculoskeletal complaints, rheumatologic diseases, and disability among the Zoroastrian population in Iran., Methods: The city of Yazd, in central Iran was selected for this study, with the highest population of Zoroastrians in Iran. Subjects were selected by cluster sampling of 9 neighborhoods populated with Zoroastrians. Subjects ≥15 years old were interviewed by trained interviewers in their houses. The validated Farsi translation of Community Oriented Program for the Control of Rheumatic Disease (COPCORD) Core Questionnaire (CCQ) was used for this study. Subjects with musculoskeletal complaints (pain, stiffness and/or swelling) were examined by a rheumatologist. Laboratory tests and radiographic exams were performed when deemed necessary., Results: Two-thousand subjects were interviewed during a 12-month period, of which 956 were male, and 1044 were female. The mean age was 41.1 ± 18.3 years (95%CI: 40.3-41.9). 36.9% of the subjects had university-level education. In the 7 days prior to the interview, 27.6% of the subjects had musculoskeletal complaints, with the knee, dorsolumbar spine, and shoulder being the most common sites of complaints. The most common rheumatologic diagnoses were osteoarthritis (21.5%) and low back pain (10.3%). Rheumatoid arthritis was diagnosed in 1.2% of the subjects., Conclusions: The epidemiology of musculoskeletal complaints and rheumatologic disorders was inconsistent with previous COPCORD studies in Iran, with a lower prevalence of musculoskeletal complaints in general, lower rates of Behçet and lupus, and a higher prevalence of rheumatoid arthritis. The findings of this study can be for development of better prevention, screening, and treatment programs for the vulnerable population of Zoroastrians in Iran., (© 2021. The Author(s).)

Background: Ocular manifestations are common in systemic lupus erythematosus (SLE). Retinopathy has previously been linked to disease severity and might have a significant impact on the patient's quality of life and has also been associated with a poor prognosis in SLE. This study aimed to determine the prevalence of retinopathy among patients who are newly diagnosed with SLE., Methods: In a cross-sectional study, patients diagnosed with SLE at a tertiary referral clinic were assessed for inclusion between March 2016 and March 2017. Patients who had received treatment for SLE at any time were excluded, as well as patients with hypertension, diabetes mellitus, and coagulopathy. Clinical findings and laboratory test results were recorded, and patients were examined by an ophthalmologist for evidence of retinal pathologies. SLE disease activity index was also calculated for all patients., Results: With 114 patients included in the final analysis, we found a prevalence of 15.8% for retinopathy among newly-diagnosed SLE patients. Cotton-wool spots were the most common finding (78%). Patients with retinopathy had significantly lower hemoglobin levels, C3 and C4 concentrations, and higher ANA and Anti-dsDNA levels. Also, patients with retinopathy had a significantly higher SLE DAI score., Conclusions: We found a relatively high rate of retinopathy in SLE patients at the time of their initial diagnosis. Our findings suggest that retinopathy is an early manifestation of the disease. Ophthalmologic screening might be considered for SLE patients at the time of diagnosis, especially for those with severe disease. We also encourage researchers to further evaluate the correlation between retinopathy and disease activity, and the prognosis of ocular involvement., (© 2021. The Author(s).)

Behçet's disease (BD) is a chronic disorder that involves multiple organs and is pathologically considered as a form of vasculitis. The current study aims to assess the metric properties of platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) in assessing BD disease activity. Three-hundred-nineteen patients with BD were enrolled in this cross-sectional study. Demographic and epidemiological data, including IBDDAM, time since the onset, and medication and manifestation history were recorded. Complete blood counts (CBC), NLR, and PLR were assessed by analyzing blood samples. On the last visit, patients were assessed for active manifestations of disease. IBDDAM and ocular IBDAAM scores were calculated for activity of disease in each patient. Both PLR and NLR were higher in patients with active BD (Mann-Whitney U test, p-value < 0.05). Patients with active ocular manifestation had significantly higher NLR and PLR (Mann-Whitney U test, p-value < 0.05). These ratios, however, were not associated with other active BD manifestations. A value of NLR > 2.58 had 46% sensitivity and 85% specificity for the diagnosis of active ocular manifestations (AUC: 0.690). NLR had a significant, though, weak positive correlation with IBDDAM (Spearman's rho = 0.162; p-value < 0.05) and ocular IBDDAM (Spearman's rho = 0.159; p-value < 0.05). Active Behçet's presented with higher NLR and PLR ratios; however, there was only a modest correlation between NLR and BD activity (IBDDAM score). Also, NLR and PLR have significant relationship with ocular features of BD patients., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background : Behcet's disease (BD) is a vasculitis with multisystem and multiorgan involvement. Cardiac involvement in BD is a rare complication with a poor outcome that manifests itself in different forms. In this study, we aimed to investigate cardiac involvement in BD. Methods : This is a retrospective study based on cardiac manifestations in BD according to the data of the Behçet's Disease Unit, the Rheumatology Research Center, Tehran University of Medical Sciences, from registered patients from 1975 until June 2017. Cardiac manifestations consisted of pericarditis, myocardiopathy, myocardial infarction, stable ischemic heart disease, endomyocardial fibrosis, thrombosis, and valvular and coronary involvement. All the patients' baseline and demographic data were recorded in a designed questionnaire. The laboratory workups, imaging, and pathological tests were also performed. Results: We studied 7650 patients with BD, of whom 51% were male. In the entire study population, 47 patients manifested cardiac involvement: valvular involvement in 6.1%, myocardial infarction in 23.4%, stable ischemic heart disease in 20%, pericarditis in 21.3%, intracardiac thrombosis in 2.1%, coronary aneurysm in 2.1%, heart failure in 12.8%, and dilated cardiomyopathy in 4.3%. Conclusion : The prevalence of cardiac involvement in our patients with BD was 0.6%. A multidisciplinary approach can reduce mortality and morbidity rates. Consequently, we suggest that echocardiography and other cardiac diagnostic tests be routinely considered for early diagnosis and subsequent treatment., (Copyright © 2021 Tehran University of Medical Sciences. Published by Tehran University of Medical Sciences.)

Objective: The objective of this study was to evaluate the relationship of lifestyle with disease activity among patients with systemic lupus erythematosus., Methods: This cross-sectional descriptive-correlational study was conducted in 2019 on 209 patients with systemic lupus erythematosus. Data were collected using a demographic and clinical characteristics questionnaire, the Health-Promoting Lifestyle Profile II, and the systemic lupus erythematosus disease activity Index. The data were analysed through the mixed model and the logistic regression analyses., Results: In total, 67.5% of participants had active disease. The mean score of lifestyle was 2.49±0.30 and the lowest and the highest lifestyle dimensional mean scores were respectively related to the physical activity and the health responsibility dimensions (1.55±0.60 and 3.25±0.45). The analysis showed that each one point increase in the mean score of lifestyle was associated with 0.79 point decrease in the odds of disease activity (P = 0.006). Moreover, disease activity had significant positive relationship with body mass index (P = 0.015)., Conclusion: Interventions for promoting lifestyle among these patients and improving healthcare providers' knowledge about Systemic lupus erythematosus and lifestyle modification are recommended to reduce disease activity., (© 2021 The Mediterranean Journal of Rheumatology (MJR).)

Background: The diagnosis of Behçet disease (BD) is challenging in many cases. The purpose of this study was to describe the clinical characteristics of patients at a referral BD clinic., Methods: In a retrospective study, we collected data from patients at a national referral Behçet clinic from November 2018-August 2019. A BD diagnosis was confirmed (BD group) or ruled out (Non-BD group), and the two groups were compared for differences., Results: A total of 238 patients satisfied the inclusion criteria. Forty patients (16.8%) were finally diagnosed with BD. Ocular and genital lesions were significantly more prevalent in the BD group. A positive pathergy test and HLA-B51 were also significantly more common in BD. However, oral lesions, articular involvement, and gastrointestinal manifestations were similar between groups. Also, patients with BD were significantly more likely to have multi-organ (≥2 organ systems) involvement., Conclusions: Being the first study to evaluate the clinical characteristics of patients who are visited at a referral BD clinic and are believed to have a high probability of Behçet, the results of this study are important from an epidemiological standpoint. Also, the findings of this study could be used by referral Behçet clinics, which evaluate and diagnose patients with a high pretest probability and atypical presentations of BD on a daily basis. The alternative diagnoses established in this study could be used as the list of the most common differential diagnoses for Behçet's disease.

Objectives: Celiac disease (CD) is one of the most common chronic diseases. Celiac disease has been associated with several autoimmune disorders, but the association with systemic lupus erythematosus (SLE) as a systemic autoimmune disease is still controversial. In this study, we aimed to determine the prevalence of biopsy-proven CD in patients with SLE, and to determine the clinical symptoms and laboratory data in these patients., Material and Methods: In a cross-sectional study, SLE patients at a referral clinic were evaluated for gastrointestinal symptoms between March and December 2016. Patients were evaluated by a gastroenterologist, and upper gastrointestinal endoscopy with intestinal biopsy was performed if deemed necessary. The clinical symptoms, laboratory data, and endoscopy results were recorded and compared between groups., Results: In total, 130 patients were evaluated in this study. Gastrointestinal symptoms were present in 40% of the patients. Endoscopy was performed in all SLE patients with gastrointestinal symptoms. Four patients (3%) were diagnosed as having CD based on biopsy results and response to a gluten-free diet. Anti-endomysium antibody (AEA) was found to be 100% sensitive and 99.2% specific for the diagnosis of CD in SLE patients, and anti-gliadin antibody (AGA) had a 50% sensitivity and 98% specificity. Patients with comorbid CD and SLE were significantly more likely to have diarrhea, abdominal pain, nausea/vomiting, recurrent oral aphthosis, and anemia., Conclusions: The results of this study suggest that a significant association is present between CD and SLE. We found a prevalence of 3% for biopsy-proven CD in patients with SLE, which is five times the prevalence of CD in the general population., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2021 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie.)

Objectives: This study aims to explore the perceptions, concerns, expectations, and viewpoints of female patients with systemic lupus erythematosus (SLE) regarding reproductive health in Iran., Patients and Methods: This qualitative research was conducted using 27 semi-structured deep interviews with 19 married female patients (mean age 37.4±7.0 years; range, 15 to 49 years) suffering from SLE selected through purposive sampling in the Rheumatology Center of Shariati Hospital, Tehran, Iran between August 2017 and March 2018., Results: Patients' perceptions were classified into six categories and 14 subcategories: physical suffering (debilitating pain, gradual disability, and apparent transformation), psychological stress (family disturbance and sexual dissatisfaction), challenging fertility (bothersome pregnancy and impaired parenting), adaptation to the disease (self-care and lifestyle modification), need for a supporter (support from the spouse, support from the family and acquaintances, and lack of occupational and social support), and spirituality (forgiveness of sins and resorting to God)., Conclusion: The findings clearly suggested the negative effects of SLE on the reproductive health of these patients. This study emphasized the importance of care and support for female patients with SLE. We hope that this research enhances the awareness of care providers about physical, sexual, psychological, social, and spiritual factors, since taking care of these patients requires strategic and multidisciplinary management., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2020, Turkish League Against Rheumatism.)

Background: Low back pain (LBP) is a major contributor to chronic pain and disability. The purpose of this study was to evaluate health-care access and utilization among patients with LBP in Iran. We also sought to study the pattern and characteristics of care-utilization behavior in these patients., Methods: Data from the Community Oriented Program for Control of Rheumatic Diseases (COPCORD) were used for this study. Three cities (Zahedan, Sanandaj, Yazd) were selected to represent the Iranian population, with different socioeconomic status and ethnic, cultural, and religious background. Demographic data, acute or chronic LBP, disability index, and utilizing care from conventional medicine (CM), allied health providers (AHP), and complementary and alternative medicine (CAM) providers were recorded., Results: Of 9101 patients, 38.6% reported LBP. Only 3.3% did not utilize care of any kind, 66.7% referred to CM providers, 20.8% to AHP, and 9.2% to CAM care. Health-care utilization was higher in female patients, older age, higher education, and higher disability index., Conclusions: The findings of this study indicate a high rate of health-care utilization among patients with LBP in Iran. CM is the most prevalent health-care resource sought by patients. These findings could be used as a framework in developing more efficient health-care programs according to the needs of specific populations.

Background: Behçet's disease (BD) is a chronic multi-systemic vasculitis with a considerable prevalence in Asian countries. There are many genes associated with a higher risk of developing BD, one of which is endoplasmic reticulum aminopeptidase-1 (ERAP1). In this study, we aimed to investigate the interactions of ERAP1 single nucleotide polymorphisms (SNPs) using a novel data mining method called Model-based multifactor dimensionality reduction (MB-MDR)., Methods: We have included 748 BD patients and 776 healthy controls. A peripheral blood sample was collected, and eleven SNPs were assessed. Furthermore, we have applied the MB-MDR method to evaluate the interactions of ERAP1 gene polymorphisms., Results: The TT genotype of rs1065407 had a synergistic effect on BD susceptibility, considering the significant main effect. In the second order of interactions, CC genotype of rs2287987 and GG genotype of rs1065407 had the most prominent synergistic effect (β = 12.74). The mentioned genotypes also had significant interactions with CC genotype of rs26653 and TT genotype of rs30187 in the third-order (β = 12.74 and β = 12.73, respectively)., Conclusion: To the best of our knowledge, this is the first study investigating the interaction of a particular gene's SNPs in BD patients by applying a novel data mining method. However, future studies investigating the interactions of various genes could clarify this issue., Competing Interests: The authors have declared that no competing interests exist.

Objectives: Systemic lupus erythematosus (SLE) affects all body organs. This disease has marked physiological, mental, and cognitive effects on the patient and results in sexual dysfunction, depression, and decreased self-confidence. This study was conducted to compare sexual function, depression, anxiety, stress, and the related factors in women suffering from SLE., Material and Methods: In this cross-sectional study, 340 women (170 with SLE and 170 healthy) aged 18-49 years were investigated. Convenience sampling was used to select the participants. In this study, a demographic questionnaire, the Depression, Anxiety, and Stress Scales - 21 Items (DASS-21), and the Female Sexual Function Index (FSFI) were applied. Data were analyzed with SPSS 16 using Spearman's correlation, χ 2 , Mann-Whitney, covariance, and multiple regression statistics; p -values less than 0.05 were considered significant., Results: The results showed a significant difference in sexual function between the SLE and healthy groups. According to covariance analysis, the difference remained significant after controlling for confounding factors (stress, depression, anxiety). Moreover, the effect of SLE on the sexual function had a significant correlation with age, life status, number and age of children, economic status, menstruation, duration of marriage, age at diagnosis, disease duration and activity, stress, anxiety, and depression. The results of multiple regression analysis showed that age, disease activity, depression, and life status had the highest correlation with sexual dysfunction in women with SLE., Conclusions: The results of this study indicated that more attention should be paid to the sexual health of women suffering from SLE as a neglected aspect of their treatment, which requires an interdisciplinary approach to shift the care of these patients from a biomedical model to a biopsychosocial model., Competing Interests: The authors declare no conflict of interest.

Behçet's Disease (BD) pathogenesis remains unclear, but some genetic loci and environmental factors are proposed to play a role. Here, we investigate the association of the endoplasmic reticulum aminopeptidase-1 (ERAP1) gene variants and HLA-B*51 with BD susceptibility and clinical manifestations in Iranian patients. In the study, 748 BD patients and 776 healthy individuals were included. The MGB-TaqMan Allelic Discrimination method was used to genotype 10 common missense single nucleotide polymorphisms (SNPs) and one intronic SNP in the ERAP1 gene region. We found no significant association between the 11 SNPs and BD in allelic and genotypic association tests. However, rs30187 showed the strongest association with BD in the recessive genotype model of the risk T allele in HLA-B*51 carriers. Although this became insignificant after correcting for multiple comparisons, the homozygous rs30187 risk allele genotype (TT) increased disease susceptibility in HLA-B*51 carriers in epistasis analysis, and the rs30187 TT recessive genotype showed a significant association with risk of cardiac involvement in the all patients and articular involvements in HLA-B*51 positive patients. Our findings suggest that gene-gene interactions between HLA-B*51 and ERAP1 variants is important for BD development, however, ERAP1 variants which interact with HLA-B*51 may differ among disease phenotypes or populations.

Objectives: This study proposed to report the characteristics of paediatric Behçet's disease (PED-BD) in a cohort of patients from Iran's registry and compare them with different reports throughout the world., Methods: From a cohort of 7504 Iranian patients with Behçet's disease those diagnosed before the age of 16 years were included in this study. Data were collected on a standard protocol comprising 105 items, including demographic features, type of presentation, and different clinical and laboratory findings., Results: PED-BD was seen in 2.7% of patients. The male/female ratio was 1.02/1, and the mean age at onset was 10.5±3.4. Positive familial history was present in 9.9%. As a first manifestation, oral aphthosis was the most frequent (75%) followed by ocular lesions in 19.1%. The prevalence rates of various manifestations were as follows: oral aphthosis: 91.7%; genital ulcer: 42.2%; skin: 51.5% (pseudofolliculitis: 43.1%, erythema nodosum: 10.3%); ocular lesions: 66.2% (anterior uveitis 52%, posterior uveitis 58.3%, retinal vasculitis 39.7%); articular manifestations: 30.9%; neurological involvement: 4.9%; vascular involvement: 6.4% (venous 4.9%, arterial 2.5%); gastrointestinal manifestations: 5.9%; epididymo-orchitis: 8.7% (boys); high ESR (≥20): 50.8%; abnormal urine: 14.1%; positive pathergy test: 57%; HLA-B5/51: 48.7%. ICBD criteria have the highest sensitivity for the classification of PED-BD patients in Iran (91.7%)., Conclusions: The clinical spectrum of PED-BD in Iran in this study was similar to that of other reports; however, genital ulcers, skin lesions (notably erythema nodosum), and gastrointestinal involvement were noticed to occur less frequently, while ocular lesions were more frequent and more severe compared to other reports.

Introduction: Systemic lupus erythematosus (SLE) myocarditis occurs in between 5% and 10% of patients with lupus. Global longitudinal strain (GLS) via speckle tracking echocardiography can detect cardiac involvement in patients suffering from SLE. We decided to determine the echocardiographic features and subsequent early diagnosis of cardiac involvement in patients with SLE utilizing the GLS index via speckle tracking echocardiography. Methods: In this cross-sectional study, we compared female patients with SLE of at least 2 years' duration and healthy controls in terms of the left ventricular (LV) GLS via speckle tracking echocardiography. After data collection in both groups, the GLS index and the ejection fraction were evaluated. Results: We analyzed and compared the LV echocardiographic parameters of 33 patients with SLE (mean age=25.45±0.63 years) with those of 35 healthy controls (mean age=27±0.45 years). The apical 2-chamber view indicated a significant decrease in the LV GLS in the case group by comparison with the healthy controls ( P =0.005). The LV GLS in the apical 3-chamber view was significantly lower in the case group than in the control group ( P =0.006). The LV GLS in the apical 4-chamber view revealed no significant difference between the case and healthy control groups ( P =0.2). While there was a significant difference between the case and control groups visà- vis the LV GLS ( P =0.02), the LV ejection fraction measured with the Simpson method showed no significant difference between the 2 groups ( P =0.96). Conclusion: GLS speckle tracking echocardiography is a noninvasive method with diagnostic and prognostic values; it may, therefore, be a sensitive marker for the diagnosis of myocarditis and other cardiac involvements in patients with SLE.

Introduction: We evaluated the effects of implementing Team-Based Learning (TBL) on student engagement, accountability, satisfaction, and preference for lecture or team-based learning. Moreover, we assessed the effect of TBL on knowledge retention and application over time through short answer questions based on clinical scenarios addressing history taking and diagnosis skills in medical students., Methods: The study was conducted in a quasi-experimental design. The study population were all of the third-year medical students (n = 84) participating in a course of rheumatology in Shariati Hospital, which is a teaching hospital affiliated to Tehran University of Medical Sciences. We compared TBL with the conventional lecture-based method. The assessments were performed after implementation of TBL by the Classroom Engagement Survey (CES) and Team-Based Learning Student Assessment Instrument (TBL-SAI). The assessment for application of knowledge was conducted in 3 time-points through short answer questions on rheumatic diseases. The comparison of results was made by Student's t-test and repeated-measures analysis of variance (RM-ANOVA) using SPSS software, version 16., Results: The CES scores indicated a high level of engagement in TBL (Mean±SD=26.7±3.70, p=0.0001) but not in the lecture-based sessions (Mean±SD=23.80±4.35, p=0.09). The total mean score (SD) for TBL-SAI was 159.68 (14.14) for TBL sessions indicating a favorable outcome (p=0.0001). The student scores obtained from the short answer questions showed that over time the students' scores had declined significantly less for the TBL sessions in comparison to the lecture-based sessions, F (2, 166) = 4.624, p=0.011., Conclusion: The results indicated higher student engagement, satisfaction and long term learning by TBL., Competing Interests: Conflict of interests: None declared.

Purpose: The purpose of this study was to evaluate the effects of applying the continuous care model (CCM) on the knowledge level and health-related quality of life (HRQoL) of systemic lupus erythematosus (SLE) patients and their perceptions of family awareness about the disease., Design: The Continuous Care Model (CCM) was implemented for 3 months on 34 SLE patients, in a pretest-posttest quasiexperimental design., Methods: Two sets of questionnaires were designed for knowledge assessment. HRQoL was assessed by SF-36 questionnaires. Analysis was by paired t test and one-way ANOVA., Findings: The continuous care model significantly improved patients' knowledge level and their perceptions of their family members' awareness of their disease. HRQoL status in SLE patients was poorer than the general population in six of eight SF-36 scales (p < .05), except for Bodily Pain and Mental Health. Postintervention scores showed improvement in six SF-36 scales (p < .001), except for Bodily Pain and Social Functioning., Conclusions: Our results underlined the advantages of applying the CCM as a comprehensive method of self-care in SLE., Clinical Relevance: Despite many improvements in SLE care, the patients' quality of life is still much affected by SLE. Implementation of the CCM could lead to improvement in the knowledge and HRQoL of SLE patients.

Background: Systemic sclerosis (SSc) is a chronic multisystem connective tissue disorder with detrimental impact on quality of life. Patients with SSc face emotional distress and frequently meet criteria for a psychiatric disorder. However, the pattern of psychiatric manifestations may vary according to socioethnic background., Objectives: We investigated the prevalence of depressive and anxiety symptoms and examined their association with sociodemographic and clinical factors in Iranian SSc patients., Methods: Depressive and anxiety symptoms were evaluated by Beck Depression Inventory and Cattell questionnaire in 114 SSc patients. The associations between sociodemographic and clinical factors and depressive/anxiety symptoms were examined via multivariate analysis., Results: The prevalence of depressive symptoms was 68.4%. There was a significant association between depressive symptoms and pulmonary and gastrointestinal manifestations. Also, diffuse SSc patients were more prone to depressive symptoms. Mean Rodnan scores were significantly higher in patients with depressive symptoms in comparison with subjects with no depressive symptoms. The prevalence of anxiety symptoms was 23.6%. Anxiety symptoms were not associated with demographic characteristics, SSc subtype, disease duration, Rodnan score, other clinical features, and previous history of depression in the patients or their family. The coincidence of anxiety and depression was 82.8%., Conclusions: Depressive and anxiety symptoms are prevalent among Iranian SSc population. The depressive symptoms showed correlation with pulmonary and gastrointestinal involvement, as well as diffuse SSc subtype.

SLE is a common autoimmune disease with considerable morbidity. Ramadan fasting is a religious custom Muslims regularly practice. We aimed to evaluate the effect of Ramadan fasting on SLE patients' disease activity, health quality of life and lipid profile. We conducted this case control study as a pilot study in 40 quiescent SLE patients, 21 cases who decided to fast and 19 controls who decided not to have Ramadan fasting between August and November 2009 in lupus unit of Rheumatology Research Center in Tehran University of Medical Sciences, Iran. They were assessed for SLE Disease Activity Index, lipid profile and quality of life with Short-Form 36 (SF-36) Health Survey, 1 day before Ramadan, the day after and 3 months after Ramadan fasting. After 24.1 ± 5.4 (mean ± SD) days of fasting, anti-ds DNA increased for 0.34 ± 0.41 mmol/dL in cases versus 0.07 ± 0.31 in controls (P = 0.026). Likewise C3 increased more dramatically in cases (16.8 ± 17.5 vs. 2.3 ± 13.2 mg/dL, P = 0.006). Three months after fasting, anti-ds DNA was still increased 0.28 ± 0.46 mmol/dL in cases while a 0.02 ± 0.43 mmol/dL drop in controls was detected (P = 0.04). On the contrary, C3 returned to baseline. These changes were not accompanied with significant changes in disease activity and health quality of life. Ramadan fasting had no effect on lipid profile except for delayed total cholesterol decrease in cases in comparison with controls (16.4 ± 29.4 decrease vs. 4.6 ± 23.9 mg/dL decrease, P = 0.018). Ramadan fasting probably has no detrimental effect on SLE patients' disease activity and their quality of life in the quiescent phase of disease.

Objective: Avascular necrosis of bone (AVN) is an important complication of systemic lupus erythematosus (SLE). Corticosteroid therapy has been underlined as a main risk factor for osteonecrosis. However, AVN development in patients who have never received corticosteroid and the absence of AVN in the majority of the patients, who received corticosteroid, propose a role for non-corticosteroid risk factors in AVN development., Methods: This case-control study included two subsets: oral corticosteroid (66 AVN and 248 non-AVN patients) and pulse-therapy subset (39 AVN and 312 non-AVN patients) who have attended our Lupus clinic from 1979 to 2009. Patients received similar cumulative dose corticosteroid, equal maximum dose and 1-year maximum dose of corticosteroid. The demographic data (including sex, age of disease onset, age at the diagnosis of AVN), organs involvement, SLE Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology-Damage index (SLICC/ACR-DI), number of disease flare ups were compared between two subsets., Results: The mean age of SLE onset was younger (P value = 0.04) in the AVN patients. In oral corticosteroid subset, malar rash (P value < 0.001) and oral ulcer (P value = 0.003) were seen more frequently in non-AVN patients, whereas psychosis (P value = 0.03) was significantly more prevalent AVN subset in oral corticosteroid subset. In corticosteroid pulse subset, no significant difference in clinical features was noted., Conclusion: In oral corticosteroid subset, younger age of disease onset and psychosis were significantly associated with AVN, whereas malar rash and oral ulcer showed negative association AVN.

Objectives: We aimed to evaluate the prevalence of depression and anxiety in patients with systemic lupus erythematosus (SLE) and explore their association with demographic and clinical features., Methods: In this cross-sectional study, 166 SLE patients in rheumatology center of Shariati hospital, Tehran University of Medical Sciences were enrolled. SLE disease activity index (SLEDAI) and Beck and Cattell inventories for evaluation of depression and anxiety were completed for each patient., Results: The mean age of patients was 33.1 ± 11.1 years and 92.2% of them were female. Two patients (1.2%) had only depression (without anxiety), while 36 patients (21.6%) had only anxiety (without depression). Meanwhile, 105 patients (63.3%) had mixed depression-anxiety and 23 patients (13.8%) did not have either depression or anxiety. Mean daily dose of prednisolone and number of administered drugs did not show significant difference between different subgroups of patients. In assessment of clinical and therapeutic items, no significant correlation between severity of depression (P = 0.65) and anxiety (P = 0.36) with daily dose of prednisolone in SLE patients was observed. There was no significant association between SLEDAI and severity of depression or anxiety. Occupational status had significant correlation with severity of depression and anxiety (P = 0.005).On the contrary, no significant correlation between number of administered drugs and severity of depression and anxiety was present., Conclusion: This study indicated the high prevalence of depression and anxiety among SLE patients and reinforced the need of a comprehensive psychiatric work-up in SLE.

The Leeds Behçet's disease quality-of-life (BD-QoL) questionnaire is a specific and valid measure which is applied in English-speaking patients. We conducted Persian adaptation of BD-QoL questionnaire. Between June and December 2012, 220 Iranian patients fulfilling International Study Group criteria for the diagnosis of BD attending the rheumatology clinics at Tehran University of Medical Sciences were enrolled. Bilingual translators undertook the forward translation and cross-cultural adaptation of the BD-QoL questionnaire. Back-translation was conducted, and this version was sent to the designer of the questionnaire and revised accordingly. SF-36 health survey, Iranian Behçet's disease dynamic activity measure (IBDDAM), and Behçet's Disease Current Activity Form (BDCAF) were other administered measures. The Varimax rotation method with Kaiser normalization defined 5 factors with eigenvalues greater than 1.0. Studied cases were comprised of 118 males (53.6 %) and 102 females (46.4 %). Mean age of the patients was 38.3 ± 11.3 years (range 16-73). The mean BD-QoL score was 10.3 ± 8.8. Test-retest reliability was high, and two time points were significantly correlated (Spearman's correlation coefficient of 0.75-0.84). Cronbach's α coefficient of 0.949 demonstrated the excellent internal consistency. These factors cumulatively explained 58.74 % of total variance. The ratio of first to second eigenvalue was 7.08, which underlined the undimensionality. The results revealed adapted BD-QoL scores had significant correlation with IBDDAM (correlation coefficient = 0.19, P value = 0.005) and BDCAF (correlation coefficient = 0.21, P value = 0.002). Conversely, no significant correlation between BD-QoL and SF-36 results was detected (P value = 0.078). The Persian version of BD-QoL was shown to be unidimensional, highly reliable, and adequate construct validity.