A multicenter study of long-term outcomes of relapsing polychondritis in Iran.

Relapsing polychondritis (RP) is a systemic immune mediated disease characterized by recurrent episodes of inflammation in various cartilage-rich areas. RP may cause extensive tissue destruction and is associated with significant morbidity and mortality. In this multicenter study, we considered the remission status and long-term outcomes of RP in patients who were followed-up in six referral rheumatology centers in Iran. Outcomes of disease was assessed by remission status and RP induced damage. A total of 29 patients with RP were examined for enrollment in the study, and 26 patients with a minimum follow-up period of 6 months were included in the RP outcome analysis. Median time to control of symptoms and sustained remission were 5 and 23 weeks, respectively. Prednisolone was discontinued in 8 (30.8%) patients and medication-free remission was achieved in 7 (23.1%) patients. Regarding the disease course, 34.6% of patients had a relapsing-remitting course, 42.3% had a monophasic course, and 23.1% had an always-active course. Despite extensive treatment with immunosuppressive medications, RP induced damage was developed in 21 (80.8%) patients. Ear deformity and osteoporosis were the most common RP induced damage. Long-term remission and medications-free remission in RP is accessible. However, RP related damage occur in majority of patients.

Predictors of medications-free and long-term remission in anti-neutrophil cytoplasmic antibody-associated vasculitis: Real-world evidence.

BACKGROUND AND AIM: In this study, we report the outcomes of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in daily practice based on Connective Tissue Diseases Research Center-Vasculitis Registry (CTDRC-VR) data. METHODS: Patients were included if they were 18 years or older, had a diagnosis of the groups of AAV based on 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis, and were followed for a period longer than 2 years or were died. Complete clinical remission was defined as granulomatosis with polyangiitis (BVAS/GPA) of 0. Sustained remission was defined as a complete clinical remission for at least six months and tapering prednisolone dose to ≤ 7.5 mg/d. Long-term remission was defined as complete clinical remission for ≥ 5 years and tapering prednisolone dose to ≤ 7.5 mg/d. Medications-free remission was defined as complete clinical remission and discontinuation of glucocorticoids, cytotoxic medications and biologics. RESULTS: Sixty patients with AAV were enrolled in this study. Sustained and long-term remission were developed in 91.7 and 72.1 percent of patients, respectively. Relapse was developed in 27 (45%) patients. Medications-free remission was developed in 23 (33.3%) patients. Vasculitis induced damage was developed in 40 (66.7%) patients. Patients with damage had significantly lower age and higher BVAS at the baseline. Upper airway and renal involvement, and non-adherence in patients with damage was significantly more common. CONCLUSIONS: Induction therapy leads to long-term and medications-free remission in 72% and 38% of patients with AAV, respectively.

Cigarette smoking and risk of adult-onset Still disease: a propensity score matching analysis.

BACKGROUND: Environmental factors play an important role in the pathogenesis of rheumatic diseases. Smoking is thought to be a risk factor for autoimmune rheumatic diseases. AIMS: The purpose of the present study was to assess the association between smoking and adult-onset Still disease (AOSD) and the effect of smoking on outcomes of this disease. METHODS: In this case-control study, patients with AOSD who met the Yamaguchi criteria, were older than 16 years at the disease onset and were in follow-up for at least 12 months were consecutively enrolled in the study. The outcome of AOSD was assessed by acquiring remission on treatment, remission off treatment, time to remission and rate of flare. The smoking status of participants was defined by direct or phone interviews. Individuals who had smoked daily for at least 6 months were defined as a smoker. We performed propensity score matching analyses by using four parameters, including age, sex, educational status and marital status. RESULTS: Propensity score matching resulted in 72 patients with AOSD and 216 matched controls. The number of ever smokers in the AOSD and control groups were 11 (15.3%) and 25 (11.6%) respectively. There was no significant increase in the risk of AOSD in multivariate analysis after adjustment for age, sex, marital status and educational level. There were no significant differences in the outcomes of AOSD between ever and never smokers. CONCLUSIONS: Smoking probably is not a risk factor for AOSD and did not affect the response to treatment.

Serum salusin-ß levels in patients with systemic lupus erythematosus.

OBJECTIVE: Endothelial dysfunction (ED) has an important role in the pathogenesis of systemic lupus erythematosus (SLE). Studies on other inflammatory diseases show that salusin-ß with various mechanisms may play a role in the promotion of ED and inflammation. The aim of this study was to measure serum salusin-ß levels in SLE patients and evaluate it as a potential biomarker in assessing SLE activity and predicting organ involvement. METHODS: In a cross-sectional study, 60 patients diagnosed with SLE and 30 age- and sex-matched healthy controls were enrolled. Disease activity of SLE patients was assessed by the systemic lupus erythematosus disease activity index 2000 (SLEDAI-2 K). Serum levels of salusin-ß were measured using a human salusin-ß enzyme-linked immunosorbent assay kit. RESULTS: Serum salusin-ß levels in SLE and control groups were 474.2 ± 117.1 pg/ml and 157.7 ± 88.7 pg/ml, respectively. The difference was significant (P = 0.001). There was no significant correlation between serum salusin-ß levels with age (r = - 0.06, P = 0.632) and SLEDAI (r = - 0.185, P = 0.158). In patients with nephritis and thrombosis, serum salusin-ß was significantly higher. In addition, in patients with serositis, serum salusin-ß was significantly lower. Multiple linear regression analysis showed that serum salusin-ß levels retained a significant association with nephritis and thrombosis after model adjustment for serositis, nephritis, and thrombosis. CONCLUSIONS: Our findings showed that salusin-ß might have a possible role in the pathogenesis of SLE. Salusin-ß may be a potential biomarker for nephritis and thrombosis in SLE. Key Points • Serum salusin-ß levels were significantly higher in SLE patients than the control group. • There was no significant correlation between serum salusin-ß levels with age and SLEDAI. • Serum salusin-ß levels retained a significant association with nephritis and thrombosis.

Prevalence and predictors of long-term remission in rheumatoid arthritis in real-world practice: a longitudinal study.

OBJECTIVES: The aim of the present study was to provide real-world evidence for factors predicting long-term remission in a longitudinal study of rheumatoid arthritis (RA) patients. METHODS: Long-term remission was defined by meeting American Rheumatism Association (ARA) criteria for remission and prednisolone dose ≤ 5 mg/d for at least 5 years. Patients in this cohort were treated by tight control strategy using step-up combination therapy with conventional synthetic DMARDs (csDMARDs), biologic DMARDs. The parameters associated with long-term remission were subjected to univariate analysis, and parameters with P-values of < 0.1 in univariate analysis were included in a multivariate regression analysis. RESULTS: One thousand two hundred and eighty-six RA subjects were considered for eligibility, and finally, 499 patients were included in the study. Median duration of follow-up was 108 months. Long-term remission occurred in 157 (31.5%) patients. Median time to long-term remission was 8 (5, 41) months. Predictors of long-term remission were absence of flare during the course of disease, occurrence of sustained remission during 6 months after starting therapy, age at the disease onset > 60, being anti-citrullinated protein antibodies (ACPA) negative, and Disease Activity Score-28 (DAS28) at cohort entry ≤ 5.1. CONCLUSION: In real-world practice, long-term remission occurs in 31.5% of patients treated with a tight control strategy. Absence of flare during the course of disease, occurrence of sustained remission during 6 months after starting therapy, age at the disease onset > 60, being ACPA negative, and DAS28 at baseline ≤ 5.1 are independent predictors of long-term remission. Key Points • In real-world practice, long-term remission occurs in 31.5% of patients treated with a tight control strategy. • Median time to long-term remission was 8 months. • Absence of flare during the course of disease, occurrence of sustained remission during 6 months after starting therapy, age at the disease onset >60, being ACPA negative, and DAS28 at baseline ≤ 5.1 are independent predictors of long-term remission.

Survivin; a novel therapeutic target that correlates with survival of autoreactive T lymphocytes obtained from patients with ankylosing spondylitis.

Ankylosing spondylitis (AS) is progressive immune-mediated arthritis. Persistent autoreactivity of T cells with an up-regulated Survivin expression is strongly implicated in AS immunopathogenesis. Besides, Survivin can inhibit proapoptotic caspase 9 activations. Moreover, microRNAs are small non-coding RNAs that are dysregulated in various diseases, in which their altered expression could modulate Survivin expression. The primary goal of this study was to assess the role of Survivin and its-targeting microRNAs in the immunopathogenesis of AS disease. For this aim, peripheral blood mononuclear cells (PBMCs) were isolated from 15 patients with AS and healthy matched controls using Ficoll-Hypaque. T cells were obtained using the magnetic-activated cell sorting (MACS) method. After that, the expression levels of Survivin, Caspase 9, and specific miRNAs were determined using qT-qPCR. Also, the expression of Survivin and Caspase 9 at protein levels was determined by western blotting. Then, the isolated T cells were co-cultured with interleukin (IL)-2 and muromonab-CD3 (OKT-3) for active-induced cell death (AICD) induction, Survivin siRNA for inhibition of Survivin expression, and their combination to assess the implication of Survivin expression in autoreactive T lymphocytes' resistance to apoptosis by determining the rate of apoptosis by Flowcytometry assay. The results showed that Survivin was up-regulated while Caspase 9 was downregulated in patients with AS. It was also revealed that microRNAs that directly or indirectly target the Survivin mRNA were dysregulated in patients with AS. It was also revealed that T cells obtained from AS patients were more resistant to apoptosis induction than those obtained from healthy people. In summary, the results obtained from this study showed that dysregulation of Survivin and Survivin-targeting miRNAs in T lymphocytes obtained from AS patients contribute to their resistance to apoptosis, suggesting the future development of targeted therapies for AS.

The effect of osteoporotic and non-osteoporotic individuals' T cell-derived exosomes on osteoblast cells' bone remodeling related genes expression and alkaline phosphatase activity.

OBJECTIVES: Osteoporosis is a common skeletal disorder attributed to age and is defined as a systematic degradation of bone mass and the microarchitecture leading to bone fractures. Exosomes have been reported in almost all biological fluids and during the failure of bone remodeling. 20 ml of blood samples were obtained from osteoporotic and non-osteoporotic postmenopausal women. After the isolation of peripheral blood mononuclear cells (PBMCs), T cells were separated via the magnetic-activated cell sorting (MACS) technique. Exosomes were driven from T cells of non-osteoporotic and osteoporotic volunteers. Subsequently, normal osteoblasts were treated with obtained T cell exosomes to assess osteoblastic function and gene expression. RESULTS: Runx2, type I collagen, osteopontin, and osteocalcin expression decreased in osteoblasts treated by osteoporotic T cell exosomes. In contrast, an increased expression of the mentioned genes was observed following non-osteoporotic T cell exosome treatment. Additionally, osteoblast alkaline phosphatase (ALP) activity treated with non-osteoporotic T cell exosomes increased. However, this activity decreased in another group. Our data demonstrated that T cell exosomes obtained from osteoporotic and non-osteoporotic individuals could alter the osteoblastic function and gene expression by affecting the genes essential for bone remodeling.

Clinical course, chest computed tomography severity score and outcome of coronavirus disease 2019 (COVID-19) in patients with rheumatic diseases.

Aim of the work: To assess the clinical manifestations, imaging findings and outcomes of corona virus disease 2019 (COVID-19) in patients with rheumatic diseases. Patients and methods: In a three-center study, patients with rheumatic diseases who developed COVID-19 were included. Patients were classified into two groups, i) inflammatory arthritis including rheumatoid arthritis (RA), spondyloarthritis (SpA) and undifferentiated arthritis, ii) connective tissue diseases (CTDs) including systemic lupus erythematosus (SLE), vasculitis and others. COVID-19 outcomes were assessed based on chest computed tomography severity score (CT-ss), the level of care, the number of patients who died and flare of underlying rheumatic disease. Results: One hundred ninety-six patients with a mean age of 47.9 ± 15.1 years, 73.5% female, were included. Underlying rheumatic diseases were RA (57.7%), SLE and other CTDs (17.9%), SpA (11.2%), vasculitis (11.2%) and undifferentiated arthritis (2%). Myalgia, malaise and fever were the most common clinical manifestations of COVID-19. Pneumonia on computerized tomography (CT), hospitalization, admission in intensive care unit and need to mechanical ventilation were observed in 75.5, 37.2%, 10.7% and 6.6% of patients, respectively. Treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids, diabetes and underlying pulmonary disease were predictors of moderate to severe pneumonia and hospitalization. Fifteen (7.6%) patients died. Flare of underlying rheumatic disease occurred in 16.3% of patients. Flare of disease in patients with CTDs was significantly more than other rheumatic diseases. Conclusions: In rheumatic patients, treatment with NSAIDs or prednisolone, diabetes and pulmonary disease are risk factors of moderate to high CT-ss and hospitalization during COVID-19.

Nanocurcumin supplementation ameliorates Behcet's disease by modulating regulatory T cells: A randomized, double-blind, placebo-controlled trial.

Current research was designed to assess the effects of nanocurcumin supplementation on regulatory T (Treg) cells frequency and function in Behçet's disease (BD). In this randomized double-masked, placebo-controlled trial, 36 BD subjects were randomly put into two groups to take one 80 mg nanocurcumin capsule or placebo daily for 8 weeks. Before and after trial, disease activity, Treg cells frequency and expression of related immunologic parameters including forkhead box protein P3 (Foxp3) transcription factor messenger RNA (mRNA) and microRNAs (miRNAs) such as miRNA-25 and miRNA-106b as well as cytokines including transforming growth factor (TGF)-ß and interleukin (IL)-10 were studied. Thirty-two patients (17 in the nanocurcumin and 15 in the placebo groups) completed the trial. Treg cells frequency increased significantly in the nanocurcumin group compared with baseline (P < 0.001) and placebo group (P < 0.001). Moreover, FoxP3, TGF-ß, IL-10, miRNA-25, and miRNA-106b mRNA expression levels increased considerably in the nanocurcumin group compared to baseline (P < 0.001) and placebo group (P < 0.001, P < 0.001, P = 0.025, P = 0.011, and P < 0.001, respectively). Significant increases in serum TGF-ß and IL-10 were seen in nanocurcumin group compared with baseline (P < 0.001) and placebo group (P = 0.001 and P < 0.001, respectively). Significant decrease in disease activity was found in nanocurcumin group compared with placebo group (P = 0.044). Our study provided a promising view for desirable effects of nanocurcumin supplementation in improving immunological parameters and disease activity in BD.

Can treating rheumatoid arthritis with disease-modifying anti-rheumatic drugs at the window of opportunity with tight control strategy lead to long-term remission and medications free remission in real-world clinical practice? A cohort study.

OBJECTIVE: The aim of this retrospective study is to compare the results of starting rheumatoid arthritis (RA) treatment with tight control strategy in the window of opportunity and later phases of the disease in real-world clinical practice. METHODS: In this cohort, 609 RA patients were divided into three groups: (i) very early treatment (VET): ≤ 3 months; (ii) early treatment (ET): 3-12 months; and (iii) late treatment (LT) > 12 months after the onset of the disease. Four levels of remission were defined: (i) sustained remission on treatment, (ii) sustained glucocorticoids free remission, (iii) sustained disease-modifying anti-rheumatic drugs (DMARDs) free remission, and (iv) long-term remission. Outcome was assessed based on the number of patients in sustained or long-term remission and patients with poor joint outcome and systemic involvement. RESULTS: There were no significant differences in the remission rate between the groups. Time to sustained remission in VET group was shorter than ET and LT groups. There were no significant differences in the rate and duration of prednisolone discontinuation in the studied groups. DMARDs were discontinued in VET, ET, and LT groups in 8.7%, 10.2%, and 7% of the patients, respectively. Poor joint outcome occurred in 33.2%, 50.5%, and 59.4% of the patients in the VET, ET, and LT groups, respectively. Remission induction in the first year of the treatment was associated with long-term remission in the VET, ET, and LT groups. CONCLUSIONS: Medications free remission in RA is rare, and although treatment with DMARDs within 3 months of the onset of the disease can prevent joint damage, it cannot lead to long-term remission and discontinuation of medications. KEY POINTS: • Medications free remission in rheumatoid arthritis is rare. • Treatment with DMARDs within 3 months of the onset of the disease can prevent joint damage, but it cannot lead to long-term remission and discontinuation of medications.

Factors associated with COVID-19 and its outcome in patients with rheumatoid arthritis.

OBJECTIVE: We assessed the factors associated with COVID-19, clinical manifestations, and a 30-day-prognosis of COVID-19 in a cohort of rheumatoid arthritis (RA) patients compared with the index population. METHODS: In a cross-sectional study, RA patients were followed in rheumatology clinics of Tabriz University of Medical Sciences, and a group of patients diagnosed with COVID-19 from index population were recruited. Outcomes of COVID-19 were assessed by the hospitalization rate and need to intensive care unit (ICU) and mortality. During a period of 12 weeks, 128 RA patients diagnosed with COVID-19, 760 RA control group, and 92 COVID-19 patients from index population were enrolled. RESULTS: Being female, obese, and diabetic, having pulmonary disease and chronic kidney disease (CKD), and treatment with prednisolone > 5 mg/d and TNFα inhibitors (TNFis) were independent predictors of COVID-19 in RA patients. Dyspnea, anosmia, and taste loss were more common in RA patients compared with the index population. Admission in hospital, need to ICU care, and mortality occurred in 38, 11.9, and 8.6 percent of RA patients, respectively. Although hospitalization rate in RA patients was more than the index population, there were no significant differences in need to ICU care and mortality between the two groups. CONCLUSIONS: Treatment with prednisolone and TNFis and having comorbidities including obesity, diabetes, pulmonary disease, and CKD increase the risk of COVID-19 in RA patients. Although some differences exist in the clinical manifestations of COVID-19 in RA patients and index population, prognosis of COVID-19 in RA patients is not any worse. Key Points • Being female, obese and diabetic, having pulmonary disease, chronic kidney disease (CKD), treatment with prednisolone > 5 mg/d and TNFα inhibitors (TNFis) were independent predictors of COVID-19 in RA patients. • Dyspnea, anosmia and taste loss were more common in RA patients compared with the index population. • Although COVID-19 related hospitalization was higher in RA patients than in the index population, there was no significant differences in the need to ICU care and mortality between the two groups.

Efficacy of tight control strategy in the treatment of adult-onset Still disease.

BACKGROUND: Adult-onset Still's disease (AOSD) characterized by a high spiking fever, skin rash, arthritis, and leukocytosis. The aim of the present study was considering the long-term outcomes of patients with AOSD who were treated with tight control strategy with disease modifying anti-rheumatic drugs (DMARDs). METHODS: Fifty-six patients with AOSD treated with tight control strategy were included. Four levels of remission were defined. Remission on-treatment was defined as the clinical remission, patient global assessment (PGA) ≤ 1, and prednisolone dose ≤ 5 mg/day for at least 6 months. Remission off-treatment was defined as the clinical remission and PGA ≤ 1 for at least 6 months as well as discontinuation of prednisolone, DMARDs, and biologics. Sustained remission on-treatment was defined as the clinical remission, PGA ≤ 1, and prednisolone dose ≤ 5 mg/day for ≥ 5 years. Sustained remission off-treatment was defined as the clinical remission and PGA ≤ 1 for ≥ 5 years as well as discontinuation of prednisolone, DMARDs, and biologics. RESULTS: Throughout a median follow-up of 47 months, remission on-treatment and off-treatment were obtained in 94.6% and 44.6% of patients, respectively. Sustained remission on-treatment and off-treatment were obtained in 79.2 and 8.3% of patients, respectively. Glucocorticoids (GCs) and DMARDs were discontinued in 66.1% and 48.2% of the patients, respectively. Apart from the older age of the patients in the on-GCs group, no significant differences were observed between the groups. CONCLUSION: Our study showed that using DMARDs with tight control strategy at the presentation of AOSD may control disease activity successfully. Key Points • Using DMARDs with tight control strategy at the presentation of adult-onset Still's disease may control disease activity successfully.

Effects of melatonin supplementation on serum oxidative stress markers and disease activity in systemic lupus erythematosus patients: A randomised, double-blind, placebo-controlled trial.

BACKGROUND: Considering pathological significance of oxidative stress in systemic lupus erythematosus (SLE), current research aimed to evaluate the effects of melatonin supplementation on oxidative stress markers and disease activity in SLE. METHOD: In this randomised double-blind, placebo-controlled trial, 32 SLE females were selected and randomly assigned into two groups to take 10 mg/day melatonin or placebo for 12 weeks. Before and after trial, serum malondialdehyde (MDA) and total antioxidant capacity (TAC) were measured and disease activity was determined by Systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K). RESULTS: Twenty-five patients (13 in the melatonin and 12 in the placebo groups) completed the trial. Melatonin supplementation caused significant reduction in serum MDA compared with baseline (P = .003) and placebo group (P = .004). Serum TAC level did not change significantly in the melatonin group compared with baseline and placebo group (P > .05). Furthermore, melatonin supplementation did not cause significant change in disease activity compared to baseline and placebo group (P > .05). CONCLUSION: This study demonstrated affirmative effects of melatonin in decreasing oxidative stress in SLE patients without any effect on disease activity. Further investigations are required to affirm these primitive findings and to achieve concise conclusions.What's known Free radical damage and oxidative stress has a remarkable function in systemic lupus erythematosus (SLE) pathogenesis. Products derived from oxidative modification cascades are found in biological fluids and their redundancy has a correlation with disease activity and organ damage in SLE. Dietary supplements, which decrease oxidative stress, would be useful in managing SLE. Melatonin is a potent antioxidant and has anti-inflammatory and immunomodulatory characteristics. Limited in vitro and animal studies are available indicating desirable effects of melatonin in preventing from SLE organ damage, thereby opening a new area of investigation that can contribute to using melatonin as a therapy or co-therapy for SLE. What's new Melatonin supplementation caused significant reduction in serum MDA compared with baseline and placebo group. Serum TAC level did not change significantly in the melatonin group compared with baseline and placebo group. Furthermore, melatonin supplementation did not cause significant change in disease activity compared to baseline and placebo group.

Long-term outcome and predictors of remission in Behçet's disease in daily practice.

BACKGROUND: Many factors can influence the response to treatment and prognosis of Behçet's disease (BD). Identifying the predictors of response to treatment can improve the quality and decrease the cost of medical care. This analytical study was performed to identify factors affecting the remission and outcome in BD patients with long-term follow-up. METHODS: A total of 245 BD patients aged over 16 years were followed for at least 12 months and visited at least three times a year were included. The outcome was assessed by the number of patients who were in sustained and long-term remission, had lost the primary criteria of BD for at least 12 months, were asymptomatic, and developed the sequela of disease or deceased. Sustained remission was defined as being in remission for at least six months. Long-term remission was defined as remission for ≥ 5 years. RESULTS: Mean age and mean duration of follow-up were 35.1 ± 10.7 years and 92.3 months, respectively. At the end of follow-up, 63.2% of the patients lost the criteria of BD, 51.8% of the cases were in sustained remission, and 36.2% of them were asymptomatic. Predictors of sustained remission were adherence to therapy and treatment for more than six years. Having genital ulcers and treatment with methotrexate were associated with non-remission. Predictor of long-term remission was remission induction in the first two years of the treatment. Treatment with methotrexate was associated with non-remission. Poor outcome was observed in 31.8% of patients. Male sex, obesity, and having severe disease were the risk factors of poor outcome. CONCLUSION: Achieving remission in BD is not inaccessible. Treatment with conventional and biologic disease-modifying antirheumatic drugs may cause sustained and long-term remission. Adherence to treatment, remission induction during the two years after the diagnosis and treatment for at least six years have significant role.

Is Palindromic Rheumatism a Pre-rheumatoid Arthritis Condition? Low Incidence of Rheumatoid Arthritis in Palindromic Rheumatism Patients Treated with Tight Control Strategy / ¿Es el reumatismo palindrómico una condición de artritis pre-reumatoide? Baja incidencia de artritis reumatoide en pacientes con reumatismo palindrómico tratados con estrategia de control estricta

Objectives: Palindromic rheumatism (PR) is characterized by repetitive, afebrile episodes of acute arthritis and peri-arthritis. The aim of this study was considering the long-term outcomes of patients with PR who were treated with tight control strategy using Disease-modifying anti-rheumatic drugs (DMARDs). Methods: We reviewed the charts of 106 patients diagnosed with PR who were referred to the Connective Tissue Diseases Research Center (CTDRC). We recruited all the patients diagnosed with PR according to the criteria of Hannonen. They visited the CTDRC clinic regularly and were treated with hydroxychloroquine and low dose prednisolone because of active episodes of PR. In cases that the attacks did not come under control in 3–6 months, methotrexate was added or replaced and the dose was increased up to 25mg/week. In resistant cases, sulfasalazine was added, followed by the addition of leflunomide and then azathioprine. Disease outcome was evaluated by getting complete or partial remission and prevention of disease evolution to rheumatoid arthritis (RA) or other inflammatory connective tissue diseases. Results: This study included 92 patients with PR who were treated with DMARDs. Attacks were controlled completely or partially in 76 (82.6%) patients. Medications free remission was obtained in 16.3% of the patients. RA developed in 8.7% of the patients. By multivariate logistic regression analysis, age ≤40 at disease presentation, non-adherence to therapy and PIP joints involvement were the only factors which independently predicted the risk of treatment failure. Conclusions: Tight control strategy by using DMARDs may control PR and prevent disease progression to RA.(AU)

Objetivos: El reumatismo palindrómico (PR) se caracteriza por episodios repetitivos y afebriles de artritis aguda y periartritis. El objetivo de este estudio fue considerar los resultados a largo plazo de los pacientes con PR que fueron tratados con una estrategia de control estricta utilizando fármacos antirreumáticos modificadores de la enfermedad (DMARD). Métodos: Revisamos los cuadros de 106 pacientes diagnosticados con PR que fueron remitidos al Centro de Investigación de Enfermedades de Tejido Conectivo (CTDRC). Reclutamos a todos los pacientes diagnosticados con PR según los criterios de Hannonen. Visitaron la clínica de CTDRC regularmente y fueron tratados con hidroxicloroquina y prednisolona a dosis bajas debido a episodios activos de PR. En los casos en que los ataques no se controlaron en 3 a 6 meses, se agregó o reemplazó metotrexato y la dosis se aumentó hasta 25mg/semana. En casos resistentes, se añadió sulfasalazina, seguido de la adición de leflunomida y luego azatioprina. El resultado de la enfermedad se evaluó obteniendo la remisión completa o parcial y la prevención de la evolución de la enfermedad a la artritis reumatoide (AR) u otras enfermedades inflamatorias del tejido conectivo. Resultados: Este estudio incluyó 92 pacientes con PR que fueron tratados con DMARD. Los ataques fueron controlados total o parcialmente en 76 (82,6%) pacientes. La remisión libre de medicamentos se obtuvo en el 16,3% de los pacientes. La AR se desarrolló en el 8,7% de los pacientes. Mediante el análisis de regresión logística multivariante, la edad ≤40 en la presentación de la enfermedad, la no adhesión al tratamiento y la afectación de las articulaciones PIP fueron los únicos factores que predijeron de forma independiente el riesgo de fracaso del tratamiento. Conclusiones: Una estrategia de control estricta mediante el uso de DMARD puede controlar la RP y prevenir la progresión de la enfermedad a AR.(AU)

Is Palindromic Rheumatism a Pre-rheumatoid Arthritis Condition? Low Incidence of Rheumatoid Arthritis in Palindromic Rheumatism Patients Treated with Tight Control Strategy.

OBJECTIVES: Palindromic rheumatism (PR) is characterized by repetitive, afebrile episodes of acute arthritis and peri-arthritis. The aim of this study was considering the long-term outcomes of patients with PR who were treated with tight control strategy using Disease-modifying anti-rheumatic drugs (DMARDs). METHODS: We reviewed the charts of 106 patients diagnosed with PR who were referred to the Connective Tissue Diseases Research Center (CTDRC). We recruited all the patients diagnosed with PR according to the criteria of Hannonen. They visited the CTDRC clinic regularly and were treated with hydroxychloroquine and low dose prednisolone because of active episodes of PR. In cases that the attacks did not come under control in 3-6 months, methotrexate was added or replaced and the dose was increased up to 25mg/week. In resistant cases, sulfasalazine was added, followed by the addition of leflunomide and then azathioprine. Disease outcome was evaluated by getting complete or partial remission and prevention of disease evolution to rheumatoid arthritis (RA) or other inflammatory connective tissue diseases. RESULTS: This study included 92 patients with PR who were treated with DMARDs. Attacks were controlled completely or partially in 76 (82.6%) patients. Medications free remission was obtained in 16.3% of the patients. RA developed in 8.7% of the patients. By multivariate logistic regression analysis, age ≤40 at disease presentation, non-adherence to therapy and PIP joints involvement were the only factors which independently predicted the risk of treatment failure. CONCLUSIONS: Tight control strategy by using DMARDs may control PR and prevent disease progression to RA.

Long-term remission in patients with systemic lupus erythematosus.

BACKGROUND: Remission has been introduced as a desirable outcome and the primary target of treatment in systemic lupus erythematosus (SLE). The purpose of this study was to identify the number of patients in remission and the long-term outcome of the disease and their predictors. METHOD: Of the 379 patients in our SLE Database, a total of 193 patients fulfilled the inclusion criteria. Remission was definition according to the definitions of remission in SLE. Three levels of remission were defined, including remission on-treatment, remission off-treatment and complete remission. In addition, we have defined a sustained remission for each level of remission in which the remission should last at least 5 years. RESULTS: During a median follow-up of 96 months, remission on-treatment and off-treatment, and complete remission were obtained in 49.2%, 38.9% and 19.2% of patients, respectively. Predictors of remission on-treatment in multivariate regression analysis were adherence to therapy and remission induction during 6 months after treatment. Predictors of remission off-treatment were age ≥40 at the time of analysis and remission induction during 6 months after treatment. Poor outcome (SLE Damage Index ≥1) was observed in 28% of the patients. Age at disease onset <30, kidney and nervous system involvement and SLEDAI-2K ≥ 11 at the cohort entry were the risk factors of poor outcome in multivariate analysis. However, sustained remission on-treatment had a negative association with poor outcome. CONCLUSION: Treatment with glucocorticoids, antimalarials, immunosuppressants and biologics in sequential or in combination may cause durable remission. Patients with durable remission have significantly lower organ damage.

Clinical characteristics and long-term outcome of Takayasu arteritis in Iran: a multicentre study

Background/aim: This study aimed to evaluate the demographic, clinical, angiographic and prognostic characteristics of Takayasu arteritis (TA) in Iran. Materials and methods: A total of 75 patients with TA based on the American College of Rheumatology 1990 criteria for TA classification referred to the Rheumatology Centres, were followed-up from 1989 to 2019. Demographic, clinical, angiographic and prognostic characteristics were collected at baseline and last visit. Results: The mean age was 31.9 ± 9.8 years at the disease onset. Female to male ratio was 14. The median latency in diagnosis was 24 months. Pulse discrepancy in the arms, blood pressure discrepancy in the arms, limb claudication, hypertension and constitutional symptoms were the most common clinical features. The most common angiographic type at the time of diagnosis was Type I (42.7%). The most frequent arterial lesion was stenosis (89.4%). Subclavian, carotid and aortic arteries were the most commonly involved arteries. New lesions developed in 28.6% of patients during the 5.25-year follow-up. Vasculitis-induced chronic damage was observed in all patients. Disease activity decreased and vascular damage remained stable throughout the follow-up period. Conclusions: The clinical features and angiographic type of TA in Iran are different from most Asian countries. Differences in angiographic and clinical features may lead to delayed diagnosis. The issue of delay in diagnosis should create awareness among health care providers that TA is not a very rare disease in Iranians and failure to pay attention to warning symptoms may delay the diagnosis.