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Abstract Background Scleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made on the basis of clinical findings and skin biopsy. Case presentation Here, we describe a 14-year-old Iranian girl presenting with non-pitting edema and woody thickening of the skin that progressed within a month. She was evaluated for possible underlying malignancy or connective tissue disorders, which were excluded by multiple laboratory workups. She underwent a skin biopsy which confirmed the diagnosis of scleredema, and she was successfully treated with intravenous immunoglobulin and mycophenolate mofetil. Conclusion While scleredema adultorum of Buschke is a rare disease with no definite treatment, our effort through this report was to highlight the possible benefits of treatment by intravenous immunoglobulin and mycophenolate mofetil.

Abstract Objectives The dysregulated immune response is one of the cardinal features of severe coronavirus disease 2019 (COVID‐19). This study was conducted to clarify the occurrence of autoantibodies (AABs) associated with systemic autoimmune rheumatic diseases (SARDs) in hospitalized patients with a moderate, severe, and critical form of COVID‐19. Methods The serum samples obtained from 176 hospitalized COVID‐19 patients were investigated in this study, including patients with moderate (N = 90), severe (N = 50), and critical (N = 36) forms of COVID‐19. Also, the serum samples collected from healthy subjects before the COVID‐19 pandemic were used as controls (N = 176). The antinuclear antibodies (ANAs), antidouble‐stranded DNA (anti‐dsDNA), cytoplasmic‐anti neutrophil cytoplasmic antibody (c‐ANCA), perinuclear ANCA (p‐ANCA), antiphospholipid antibodies (aPLs), and anticyclic citrullinated peptide (anti‐CCP) occurrence was evaluated using a solid‐phase enzyme‐linked immunosorbent assay (ELISA). Results The results showed that the occurrence of ANAs, anti‐dsDNA, anti‐CCP, c‐ANCA, and p‐ANCA was significantly higher in the COVID‐19 patients compared to serum obtained from healthy subjects (p

Abstract Objectives The significance of T helper 17 (Th17) cells in the pathogenesis of rheumatoid arthritis (RA) has recently been demonstrated in many studies. Retinoic acid receptor‐related orphan receptor γt (RORγt) is a transcription factor that is specifically involved in the generation of Th17 cells. Besides, the chemokine receptor CCR6, the receptor for CCL20, is characteristically expressed by these cells. Considering the pivotal roles of Th17 cells in RA pathogenesis, in this study, we assessed the gene expression of CCR6 and RORγt in the peripheral blood leukocytes of new case RA patients. Also, we evaluated their association with anticyclic citrullinated peptide (anti‐CCP) antibodies and disease activity. Methods Forty‐five new case RA patients and 45 healthy persons have been recruited in this investigation. The gene expression of CCR6 and RORγt was evaluated by quantitative real‐time PCR (qRT‐PCR), and anti‐CCP antibodies plasma levels were measured using the enzyme‐linked immunosorbent assay (ELISA) technique. Disease activity was measured according to the disease activity score‐28 (DAS‐28) formula. Results The gene expression of CCR6 and RORγt increased remarkably in new case RA patients compared to healthy controls (p

Abstract Background Aqua Pilates and Aqua Stretch exercises are different and new methods for the rehabilitation of musculoskeletal disorders. This study aimed to compare the effectiveness of Aqua Stretch and Aqua Pilates interventions in the treatment of pain, function, and posture of the spine in ankylosing spondylitis (AS) patients. Methods Forty patients participated in this study who were randomly allocated into Aqua Stretch, aqua Pilates, and control. The experimental groups received four 60-min training sessions each week for six weeks. However, the control group had only its routine drug treatment (NSAIDs & Anti TNF). Pain with Visual Analog Scale (VAS), function with Bath Ankylosing Spondylitis Functional Index (BASFI) and 40-m walking test (MWT), quality of life with ankylosing spondylitis quality of life (ASQoL), and posture of the spine with the Spinal Mouse were evaluated. Evaluations were performed before and after the interventions. Repeated measure ANOVA was employed to determine the main and interaction effects. Results Aqua Stretch and Aqua Pilates had a significant effect on pain (Aqua-Pilates: P = 0.0001; Aqua-Stretch: P = 0.0001), BASFI (Aqua-Pilates: P = 0.01; Aqua-Stretch: P = 0.02), 40-MWT (Aqua-Pilates: P = 0.006; Aqua-Stretch: P = 0.0001) and ASQoL (Aqua-Pilates: P = 0.01; Aqua-Stretch: P = 0.001), spinal range of motion (ROM) (Aqua-Pilates: P = 0.0001; Aqua-Stretch: P = 0.0001) at a similar ratio. However, the control group did not present any improvement in these factors (P > 0.05). Moreover, the minimal clinically important difference (MCID) revealed that the Aqua Stretch group performed better than the Aqua Pilates group in terms of VAS, ASQOL, and 40-MWT factors. Conclusions Aqua Stretch and Aqua Pilates had statistically the same effect on improving pain, function, quality of life, and spinal ROM, while MCID results revealed that the Aqua Stretch group performed better than the Aqua Pilates in terms of VAS-ASQOL-40-MWT. Trial registration It is notable that local ethics committee approval was obtained (IR.KUMS.REC.1399.1137), and the study was registered in Iranian Registry of Clinical Trials (IRCT; IRCT20190426043377N3; registered on 22/05/2021, https://fa.irct.ir/user/trial/56058/view ) and patient recruitments were started on 06/07/2021.

Abstract Background Angiosarcoma is a malignant rare tumor that originates from vascular endothelial cells that cover lymphatic or blood vessels. Cardiac angiosarcoma is the most prevalent sarcoma entail the heart. It has low incidence rate and poor prognosis. Our effort through this report was raising awareness of uncommon manifestations of this disease and showing the importance of appropriate diagnosis and treatment. Case presentation We present a case of cardiac angiosarcoma in a young female whose symptoms included dyspnea and hemoptysis with a history of pericardial effusion and a past history of cardiac surgery for suspected atrial Myxoma. She had history of several hospitalizations and relapse of symptoms a few months after each hospital discharge. Conclusions The unspecific symptoms of cardiac angiosarcoma made it difficult to make in time diagnose and appropriate treatment. Awareness of unspecific presentations of cardiac angiosarcoma is necessary for proper diagnosis and treatment while delayed diagnosis may worsen the prognosis and even lead to death.

Abstract Leukocytoclastic vasculitis could be a possible adverse event of different SARS‐CoV‐2 vaccines. Clinicians and manufacturers should be aware of this adverse event for appropriate diagnosis and treatment.

Takayasu arteritis (TA) is a chronic inflammatory disorder characterized by vascular damage and fibrosis in the intima that commonly occurs in the aorta. In many damaged sites in TA patients, natural killer (NK) cells have been shown to be hyperactivated and produce inflammatory cytokines and toxic components. Killer cell immunoglobulin-like receptors (KIRs) are found on NK cells and interact with human leukocyte antigen (HLA) class I ligands to activate or suppress NK cells. The present study assessed the possible role of KIR and their HLA ligand genes in susceptibility to TA in Iranian patients. This case-control study included 50 TA patients and 50 healthy subjects. DNA was extracted from whole peripheral blood samples, and polymerase chain reaction with sequence-specific primers (PCR-SSP) was performed to recognize the presence or absence of polymorphism in 17 KIR genes and 5 HLA class I ligands in each participant. Among the KIR and HLA genes, a significant decrease was detected in the frequency of 2DS4 (full allele) in TA patients (38%) compared with healthy controls (82%) (OR=0.13, 95% CI=0.05–0.34). However, none of the KIR and HLA genotypes or the interactions between these genes were associated with susceptibility to TA. The KIR2DS4 gene might be involved in the regulation of activation as well as the production of cytotoxic mediators of NK cells in patients with TA.

Background: Rheumatoid Arthritis patients are evaluated during treatment for various inflammatory factors such as C-reactive protein, Erythrocyte Sedimentation Rate, and Disease Activity Score, and other immune system-related factors. In the follow-up of patients with rheumatoid arthritis, hematologic factors associated with the immune system especially Platelet to Lymphocyte Ratio and Neutrophil to Lymphocyte Ratio are important. In this study, platelet to lymphocyte ratio and Neutrophil to lymphocyte ratio were compared in two groups of patients with and without ocular complications. Methods: This cross-sectional study was performed on 246 patients with rheumatoid arthritis who were referred to the rheumatology clinic of Kermanshah from December 2018 to May 2019. This study was carried out in accordance with the approval of the ethics committee (IR.KUMS.REC1397.311) at Kermanshah University of Medical Sciences. Of these patients, 191 had no ocular complications and 55 patients had ocular complications and were matched for age and sex. The blood samples were taken from patients and blood cell count was measured by Sysmex KX-21 hematology analyzer. The Spearman correlation test was used to evaluate the relationship between platelet to lymphocyte ratio and neutrophil to lymphocyte ratio in both groups of patients without ocular complications and with ocular complications. The Disease Activity Score was compared between the two groups using the Mann-Whitney test. Results: The results of this study showed no significant difference between NLR and PLR levels in both groups of patients without ocular complications and with ocular complications. But the results showed that DAS-28 was significantly lower in the group with ocular complications (P

Background: Interstitial lung disease (ILD) is a severe systemic sclerosis (SSc) complication with no current approved or golden standard treatment. This report aims to investigate the effectiveness of treatment with placental mesenchymal stromal cell (MSC) extracellular vesicles (EVs) in a patient with ILD due to SSc. Case presentation: The patient was a 55-year-old woman with a ten years history of SSc complicated by severe ILD. Over time, her lung disease progressed to interstitial fibrosis despite being treated with mycophenolate mofetil and monthly pulses of cyclophosphamide. Thus, she was treated with eight doses of placenta MSC-EVs. Four weeks after the third dose (Day 31 after the first dose), she reported marked improvement in her clinical symptoms, such as dyspnea and cough. Also, chest computed tomography (CT) scans demonstrated a significant reduction in ground glass consolidations and fibrotic changes. The patient was subsequently followed for twelve months, with findings showing significant improvement in exercise tolerance and reduced supplemental oxygen need. Conclusion: In this single case, placental MSC-EVs were seen to provide a potentially efficient treatment for SSc-related ILD; however, further investigation and clinical trials are necessary.

Autoimmune disease, caused by unwanted immune responses to self-antigens, affects millions of people each year and poses a great social and economic burden to individuals and communities. In the course of autoimmune disorders, including rheumatoid arthritis, systemic lupus erythematosus, type 1 diabetes mellitus, and multiple sclerosis, disturbances in the balance between the immune response against harmful agents and tolerance towards self-antigens lead to an immune response against self-tissues. In recent years, various regulatory immune cells have been identified. Disruptions in the quality, quantity, and function of these cells have been implicated in autoimmune disease development. Therefore, targeting or engineering these cells is a promising therapeutic for different autoimmune diseases. Regulatory T cells, regulatory B cells, regulatory dendritic cells, myeloid suppressor cells, and some subsets of innate lymphoid cells are arising as important players among this class of cells. Here, we review the roles of each suppressive cell type in the immune system during homeostasis and in the development of autoimmunity. Moreover, we discuss the current and future therapeutic potential of each one of these cell types for autoimmune diseases.