Common Rheumatologic Disease Research Articles

Genetic association of antinuclear antibodies with HLA in JIA patients: a Swedish cohort study

BackgroundJuvenile Idiopathic Arthritis (JIA) is a complex autoimmune disease and the most common chronic rheumatological disease affecting children under the age of 16. The etiology of JIA remains poorly understood, but evidence suggests a significant genetic predisposition.MethodsWe analyzed a Swedish cohort of 329 JIA patients and 728 healthy adult controls using the Illumina OmniExpress array for genotyping. HLA alleles were imputed from GWAS data using the SNP2HLA algorithm.ResultsCase–control analysis yielded 12 SNPs with genome-wide significant association to JIA, all located on chromosome 6 within the MHC class II gene region. Notably, the top SNP (rs28421666) was located adjacent to HLA-DQA1 and HLA-DRB1. HLA-DRB1*08:01, HLA-DQA1*04:01, and HLA-DQB1*04:02 were the haplotypes most strongly associated with an increased risk of JIA in the overall cohort. When analyzing disease specific subtypes, these alleles were associated with oligoarthritis and RF-negative polyarthritis. Within the complex linkage disequilibrium of the HLA-DRB1-DQA1-DQB1 haplotype, our analysis suggests that HLA-DRB1*08 might be the primary allele linked to JIA susceptibility. The HLA-DRB1*11 allele group was also independently associated with JIA and specifically enriched in the oligoarthritis patient group. Additionally, our study revealed a significant correlation between antinuclear antibody (ANA) positivity and specific HLA alleles. The ANA-positive JIA group showed stronger associations with the HLA-DRB1-DQA1-DQB1 haplotype, HLA-DRB1*11, and HLA-DPB1*02, suggesting a potential connection between genetic factors and ANA production in JIA. Furthermore, logistic regression analysis reaffirmed the effects of HLA alleles, female sex, and lower age at onset on ANA positivity.ConclusionsThis study identified distinct genetic associations between HLA alleles and JIA subtypes, particularly in ANA-positive patients. These findings contribute to a better understanding of the genetic basis of JIA and provide insights into the genetic control of autoantibody production in ANA-positive JIA patients. This may inform future classification and personalized treatment approaches for JIA, ultimately improving patient outcomes and management of this disease.

Effects of Electro-Muscle Stimulation Exercise Combined with Mat Pilates on Pain, Anxiety, and Strength in Sedentary Females with Fibromyalgia: A Single-Blind Randomized Controlled Trial.

Fibromyalgia syndrome (FM) is a chronic pain disorder that is ranked as one of the four most common rheumatological diseases in the world. This study aims to investigate the effects of an eight-week mat Pilates and electro-muscle stimulation (EMS) with combined mat Pilates exercises on pain, depression, anxiety, and strength in sedentary women. This study is a single-blind randomized controlled trial. A total of 30 sedentary female patients (Pilates (n = 15), EMS (n = 15)) diagnosed with FM were included in the study. The patients were subjected to Beck Depression (BDIs) and Anxiety Inventories (BAIs); a Fibromyalgia Impact Questionnaire (FIQ); five different Single-Leg Hop Tests (SLHTs); modified push-up (MPU), Handgrip Strength (HGS), Deep Squat (DSQ), V-Sit Flexor, bent-arm hang (BA), sit-up and Biering-Sørensen tests; and anthropometric tests before and after the 8-week exercise program. The eight weeks of mat Pilates exercises combined with mat Pilates and EMS revealed significant results (p < 0.05) in anthropometric data (abdomen, lower abdomen, hips) (p < 0.05) except for the results of chest circumference measurements (p > 0.05). In addition, there were statistically significant positive results in BDIs, BAIs, FIQs, lower extremity (all SLHTs and DSQ), upper extremity (MPU, HGS, BA), and core (V-SIT, sit-up, Biering-Sørensen test) strength test findings (p < 0.05). Combining the mat Pilates exercises with EMS is an effective and reliable method to improve the pain, anxiety, depression, and strength of female patients diagnosed with FM.

Network pharmacology and molecular docking to explore the treatment potential and molecular mechanism of Si-Miao decoction against gouty arthritis.

Gouty arthritis (GA) is a common metabolic rheumatological disease. Si-Miao decoction has therapeutic effects on GA. In our study, we investigated the mechanism of Si-Miao decoction against GA using network pharmacology and molecular docking analytical methods. The Traditional Chinese Medicine Systems Pharmacology Database was used as the basis for screening the main targets and agents of the Si-Miao decoction, and the Genecards, OMIM, and Drugbank databases were used to screen GA-related targets. They were analyzed using Venn with the drug targets to obtain the intersection targets. We used Cytoscape 3.9.1 to draw the "Drugs-Compounds-Targets" network and the String database for creative protein-protein interaction networks of target genes and filtered core targets. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes were used to analyze the core targets. Molecular docking was performed using AutoDockTools to predict the binding capacity between nuclear targets and active components in the Si-Miao decoction. A total of 50 chemically active components containing 53 common targets of Si-Miao decoction anti-GA and 53 potential drug target proteins were identified. Core targets, namely, TNF, STAT3, SRC, PPARG, TLR4, PTGS2, MMP9, RELA, TGFB1, and SIRT1, were obtained through PPI network analysis. GO and KEGG analyses showed that the mechanism of anti-GA in Si-Miao decoction may proceed by regulating biological processes such as inflammatory factor levels, cell proliferation, apoptosis, and lipid and glucose metabolism, and modulating the NOD-like receptor signaling pathway, IL-17 signaling pathway, TNF signaling pathway, NF-kappa B signaling pathway, and Toll-like receptor signaling pathway. We further screened the core targets, including PTGS2, MMP9, and PPAGR, as receptor proteins based on their degree value and molecular docking with the main active compounds in Si-Miao decoction, and found that baicalein had high affinity. In conclusion, Si-Miao decoction, through anti-inflammatory, apoptosis-regulating, and anti-oxidative stress action mechanisms in the treatment of GA.

Renal disease in pediatric rheumatology.

This review will provide updates in the outcomes in the common rheumatologic diseases with kidney involvement. Covered are also advances in therapeutics for the use of pediatric rheumatologic diseases with kidney involvement, as well as the potential kidney complications from other rheumatologic diseases and their medications. Two of the more common rheumatologic diseases with kidney involvement, lupus and vasculitis, continue to show inadequate response to initial therapy of renal disease and practice continues to be driven by results of adult studies. There is a continued need for pediatric specific studies in rheumatologic diseases with kidney involvement as outcomes continue to be inadequate. Despite recently approved treatments for adults with rheumatic diseases and kidney involvement, therapeutic options in pediatrics remain limited, contributing to the overall morbidity and mortality.

Analysis of the Relationship of Serum Ferritin, Urate, and Some Hematological Parameters with Disease Activity in Gout Patients

Background and objectives: Gout is a type of arthritis. Acute gout is a common rheumatological disease that mostly affects first metatarsopharyngeal joint. Chronic gout is the condition that characterized by repeated episodes of acute gout. The objective of this study was to further explore the relationship between iron, ferritin, and other hematological markers with serum urate and gout. Methods: This cross sectional study was conducted in the Department of Rheumatology of Rizgary Teaching Hospital, Erbil, and Northern Iraqi Kurdistan. A total of 85 cases were enrolled in this study, 76 males and 9 females. There mean age (SD) was 46.6 (±11.2) years. All participants were known to have gout and fulfilled the criteria for diagnosis of gout. Serum ferritin, C-reactive protein, Serum iron, total iron binding capacity, and serum urate level were measured in addition to renal function tests and liver function tests were measured. Results: The mean (SD) for urate was 9.04 (±1.16) mg/dL, 76 (89.4%) patients with high serum uric acid had high level of ferritin in the blood meanwhile; 9 patients have normal ferritin level. There was a significant positive correlation (76 out of 85 patients) between serum ferritin level and serum urate (p&lt;0.005 and rho=0.237). A positive correlation was found between C reactive protein and urate level, in contrast there was no correlation of iron level and urate level. Conclusion: The ferritin and C reactive protein levels were significantly high among gouty patients.

Safety and Efficacy of Golimumab in Indian Patients with Active Spondyloarthritis of Ankylosing Spondylitis or Psoriatic Arthritis: A Multicenter, Noncomparative, Open-Label, Real-World Study.

The safety and efficacy of tumor necrosis factor-α (TNF-α) inhibitor therapy for most common rheumatological diseases, ankylosing spondylitis (AS), and psoriatic arthritis (PsA) in controlled clinical trials is well-studied. This study evaluated subcutaneous (SC) golimumab in Indian patients with active spondyloarthritis (SpA) of AS or PsA in a real-world setting. This phase 4, multicenter, prospective, non-comparative, interventional, 24-week study was performed in patients (age ≥18 years) with active SpA of AS or PsA (NCT03733925). Golimumab 50 mg was given subcutaneously to the patients every 4 weeks. Safety was assessed. The proportion of patients with AS and PsA achieving ≥20% improvement in the Assessment of SpA International Society 20 (ASAS20) criteria and American College of Rheumatology 20 (ACR20) responses, respectively, at weeks 14 and 24 were efficacy endpoints. Of the 100 patients enrolled (men: 78 [78.0%]; mean age: 36.7 [12.02] years), 94 (94.0%) patients completed the study. Treatment-emergent adverse events with golimumab were observed in 29/100 (29.0%) patients, and nasopharyngitis and upper respiratory tract infection (5.0% each) were the most common (≥5%). Deaths were not reported. At week 14, 74.5% (95% confidence interval [CI]: 59.7; 86.1%) of patients with AS and 84.6% (95% CI: 69.5; 94.1%) of patients with PsA achieved ASAS20 and ACR20 responses, which were sustained at week 24 (ASAS20: 66.0% [95% CI: 50.7, 79.1%]; ACR20: 93.2% [95% CI: 81.3, 98.6%]), respectively. Golimumab (50 mg) administered subcutaneously was safe and effective in Indian patients with active SpA of AS or PsA during the 24-week study period with no new safety signals.

THE UTILIZATION OF THE SPIKES PROTOCOL WITHIN RHEUMATOLOGY PRACTICE

Introduction. The beginning of the patient-physician relationship often involves the delivery of bad news. The work of rheumatologists is unique in that they have to form strong relationships with their patients while delivering bad news. Many rheumatology patients stay with their doctors for their entire lives, which makes it essential to create a positive and caring experience for them. This depends on the physician's professional skills, ability to understand the patient, and engage in respectful and understandable communication when discussing complicated information. Currently, no specific recommendations exist for constructing an appropriate approach for every patient, especially in the case of rheumatology patients. This paper will adapt the SPIKES protocol for rheumatology practice. Methods. The literature search was done via MEDLINE/PubMed, Scopus, and Google Scholar. Search terms included “Ethics”, “Rheumatology”, “Bad news”, “SPIKES” and “Difficult patient”. These keywords were used in different combinations, using a filter box with an option for the recent 5 years. Firstly, articles’ abstracts were analyzed, and only articles that met inclusion criteria were included. Also, references from the selected articles were analyzed, except for the older publications and those irrelevant to the specific topic. Since there was not enough information for these requests, we tried to narrow the search by combining the names of various (most common) rheumatological diseases and using them along with the main purpose of the search. Then, we focused on the publications that met criteria and analyzed the sources cited in them. The 'Related Citations' function was also used in the search strategy. In the literature review process, we included 10 MEDLINE/PubMed articles, 4 Scopus articles, and 2 Google Scholar articles. Aim. This paper analyzes utilization of the SPIKES protocol in rheumatology practice. Results. Currently, no studies have been conducted on the utilization of these approaches in rheumatology practice. There is insufficient data regarding comparison of the effectiveness of different protocols in enhancing communication between the healthcare provider (physician or nurse) and the patient. Effective communication between physicians and patients can be achieved by following the six-step SPIKES approach, which involves specific communication skills. Conclusion. Delivering bad news requires different approaches, but none of them have been specifically tailored for rheumatic disease patients. These patients have unique behavior patterns due to the nature of their illnesses, which can lead to a decrease in their quality of life, limited activity, and painful symptoms. As a result, they often turn to their doctors in a state of personal and mental suffering. Thus, the SPIKES protocol is a useful tool for rheumatology practice

Lymphadenopathy in the rheumatology practice: a pragmatic approach.

Lymphadenopathy is a common clinical finding and diagnostic challenge within general medicine and rheumatology practice. It may represent a primary manifestation of an underlying immune-mediated disease or indicate an infectious or neoplastic complication requiring differing management. Evaluating lymphadenopathy is of particular relevance in rheumatology, given that lymph node enlargement is a common finding within the clinical spectrum of several well-known rheumatologic disorders including RA, SLE and SS. In addition, lymphadenopathy represents a hallmark manifestation of rare immunological diseases such as Castleman disease and IgG4-related disease that must be considered in the differential diagnosis because effective targeted treatments can now impact the prognosis of these conditions. In this review we present an overview of the clinical significance of lymphadenopathy in common and rare rheumatologic diseases and propose a practical approach to lymphadenopathy in the rheumatology practice. Differential diagnosis of Castleman disease and therapeutic options for this condition of increasing rheumatologic interest will be discussed in detail.

Rheumatoid arthritis and cardiovascular comorbidities

Rheumatoid arthritis (RA) is the most common rheumatologic disease characterized by inflammation with a definite relationship with heart disease. Impaired immunity, chronic inflammation, genetic susceptibility, autonomic nervous system (ANS) dysfunction, altered metabolic profile have been blamed for ischemic and non-ischemic heart diseases in RA patients. Medications used in RA treatment can also modify the risk of heart diseases by different mechanisms. Understanding the pathogenesis is essential to prevent early cardiac dysfunction in RA patients. Fundamental cellular and molecular mechanisms of pathogenesis await further elucidation. Disease management is of great importance since the cardiovascular (CV) events are known to be reduced with low disease activity. Discovery of new mechanisms will pave the way for the development of novel treatment modalities. This review highlights the epidemiology, pathogenesis, risk factors, diagnosis and screening methods and management of CV comorbidities in RA patients. Besides impact of RA medications and exercise on CV risk are summarized.

Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) comprises a group of heterogenous disorders characterized by childhood-onset chronic joint inflammation. It is the most common rheumatologic disease in the pediatric population and an important cause of chronic illness in children. Early recognition and treatment are vital to prevent sequelae of uncontrolled inflammation on the developing skeleton. JIA can have significant complications that general pediatricians should be aware of, especially uveitis, which can be insidious and asymptomatic in very young children, and macrophage activation syndrome, which can be life-threatening if not recognized and appropriately treated. Although advances have been made in the past few decades, the etiology of JIA remains incompletely understood. Efforts are underway to refine the classification of JIA. The currently accepted classification scheme identifies subsets of JIA that are important clinically in terms of prognosis and tailoring treatment approaches. However, it is limited in identifying homogenous groups of children with early childhood onset and antinuclear antibody positivity, which may have different pathogenic mechanisms that could be important in developing more targeted and effective treatment approaches in the future. Treatment strategies for JIA have changed significantly in recent years with the availability of multiple newer targeted therapies, often modeled after medications used in adult-onset forms of arthritis. These treatments, and likely many others to come, have markedly improved symptom control and reduced complications in patients with JIA.

A case report on sero-negative juvenile idiopathic arthritis progressing to polyarthritis

A group of inflammatory arthritic disorders known as juvenile idiopathic arthritis (JIA) are characterized by arthritis of uncertain origin that first manifests in a child under the age of 16. This is the most common chronic rheumatological disease of childhood. The JIA is present in every country and has an incidence of 1 in 100,000. Despite the absence of a documented etiology, there are a number of genetic and environmental risk factors as well as immunologic abnormalities that can be seen and used to infer information about the pathophysiology of the illness. Although there is no cure, modern therapy can frequently reduce the underlying inflammation and enhance function and symptoms. In this case patient showed complete resolution of symptoms with no signs of recurrence. As a result, it helps patient to live a healthy life.

Ocular manifestations in Iranian patients referred to rheumatology clinics from 2018 to 2020.

Autoimmune diseases are presented with many signs and symptoms. Eyes are commonly involved in these diseases. This study aimed to estimate the prevalence of different ophthalmological complications in patients with and without immune-mediated rheumatological diseases. Patients who were referred to Kermanshah's rheumatologic clinics by an ophthalmologist from 2018 to 2020 for a rheumatologist visit were included. A checklist for extracting data from medical files; containing symptoms, organ involvement, ocular diseases diagnosed by an ophthalmologist, rheumatologic diseases diagnosed by a rheumatologist, lab tests, and disease progression was created. After we evaluated the medical data, we found that 54 patients out of 106 were diagnosed to have immune-mediated rheumatological diseases. Patients were divided into two groups; the first group included patients with diagnosed immune-mediated rheumatologic disease and ophthalmic complications; patients with no known immune-mediated rheumatological disease were considered the second group. The obtained information was analyzed using statistical tests. One hundred and six patients participated in this study, 67% of whom were females. The most common ocular symptom was blurred vision (49%). Involvement of both eyes (43.4%) was more common than single left or right eye involvement. The most common ophthalmic disease was anterior uveitis (35.8%). The most common rheumatologic disease was Behçet's disease (21.7%). Hypertension and hypothyroidism were the most common comorbidities; 36.7% of the patients had skin and mucous involvement, and 37.7% had joint involvement. In follow-up of the ophthalmic symptoms, most patients were controlled partially. Ophthalmic diseases, laboratory tests, joint involvement, skin and mucous involvement, and lung involvement were associated with rheumatologic diseases. Early diagnosis of ocular involvement in rheumatologic diseases is crucial to prevent adverse complications. The results can be beneficial for a better perception of ophthalmic symptoms and diseases among patients with autoimmune diseases.

Levels of antibodies to adalimumab in children with juvenile idiopathic arthritis at different stages of treatment

Background. Juvenile idiopathic arthritis (JIA) is one of the most common rheumatological diseases of childhood. The central place in the problem of JIA belongs to the question of treatment the timeliness and adequacy of which determine the disease prognosis and, in fact, the entire future of the child. Immunobiological therapy can cause stable clinical and laboratory remission, as well as stop the further progression of structural changes, affecting the pathogenetic link of idiopathic arthritis. But the lack of response to therapy or a decrease in its effectiveness remains a fairly common problem and, in many cases, can be caused by the immunogenicity of immunobiological drugs, especially in case of treatment with tumor necrosis factor inhibitors. Aim of the work: to study the level of antibodies to adalimumab in children with juvenile idiopathic arthritis at different stages of treatment for analysis of immunogenicity. Materials and methods. The concentration of antibodies to adalimumab in 80 serum samples from patients with JIA was studied and evaluated, treatment effectiveness and adverse events were analyzed in 56 patients with JIA at different stages of therapy. Two groups were identified. The first one included 24 patients who had at least a 6-month break in adalimumab administration for non-medical reasons during which treatment was continued with methotrexate with periodic intra-articular injection of glucocorticoids. The level of antibodies to adalimumab was evaluated before the break and 1 month after the reinitiation of adalimumab administration. The second group consisted of 32 children who continued adalimumab without a break during treatment. Disease activity was measured using JADAS-27. Antibodies to adalimumab were detected by enzyme-linked immunosorbent assay. Results. During the examination, an elevated level of antibodies to adalimumab was detected in 10 of 24 serum samples (42 %) before non-medical withdrawal in group I. Among the results of group II, elevated levels of antibodies to adalimumab were found in 12 samples, which was 38 %. The correlation analysis revealed direct statistically significant relationships of moderate strength between the level of antibodies to adalimumab and the indicator of inflammatory activity on JADAS-27 (Spearman’s r = 0.39, p &lt; 0.05), as well as between the level of antibodies and disease duration (Spearman’s r = 0.32, p &lt; 0.05). Conclusions. Monitoring serum antibodies to adalimumab is informative for the correct interpretation of treatment effectiveness and the course of the disease with immunobiological treatment, as it may improve understanding of the clinical consequences of continued therapy, help prevent adalimumab immunogenicity, develop follow-up strategies and, as a result, can affect a long-term outcome of treatment for JIA.

Sick Leave Patterns in Common Rheumatological Diseases.

Objectives The study aims to analyze the patterns and determinants of sick leaves (SL) associated with the most common rheumatological diseases and estimate the associated productivity loss cost (PLC). Method A retrospective study reviewed all SLs that were issued from a rheumatology outpatient clinic between 2016 and 2019 for the following diagnoses: low back pain (LBP), knee osteoarthritis (OA), rheumatoid arthritis (RA), and disc disorders. The duration of each sick leave was captured and analyzed by patients' age category, gender, and diagnosis. The human capital method was used to estimate the PLC. Result One thousand and two SLs have been issued during the study period, for a cumulative 4,649 days. The majority of the patients were female (86.3%), and the mean (SD) age was 42.01 (10.71) years. SL durations ranged from 2 to 14 days. The most frequent diagnosis was RA (34.3%), followed by LBP (30.1%). Disc disorder, knee OA, and RA were independently associated with 2.01 (p=0.014), 9.07 (p<0.001), and 7.75 (p<0.001) odd ratios for long SL (≥7 days), by reference to LBP. The average PLC was estimated at $235.29 per day of sick leave, for a total cumulative cost of $235,755.30. Conclusion Rheumatological diseases are responsible for approximately 4.5 days of SL prescribing per day in our clinic, with an average yearly cost of $58,938.83. Monitoring the pattern of sick leave and identifying the interplay between their cofactors are essential to developing a comprehensive approach to enable evidence-based clinical practices along with advancements in work-based protective measures and policies.

Adaptive capabilities of the cardiovascular system, taking into account regulation systems, in adolescents with juvenile idiopathic arthritis

Background. Juvenile idiopathic arthritis (JIA) is the most common rheumatological disease of childhood (1 : 1,000 children), which involves many organs and systems, primarily cardiovascular, with the gradual development of chronic heart failure. As you know, when the functional capacity of the heart decreases, a cascade of reactions of the neurohumoral regulation system is triggered and, first of all, the sympathoadrenal system is activated with an increase in the catecholamine production, the development of tachycardia. Due to this, the level of brain natriuretic peptide in the blood is increased. In children, chronic heart failure is asymptomatic. Therefore, determining tolerance to physical exertion plays an important role in its diagnosis. The purpose was to determine the functional state of the cardiovascular system and the tolerance to physical exertion in adolescents with JIA, taking into account the level of catecholamine excretion in the daily urine and the level of natriuretic peptide in the blood. Materials and methods. Fifty-six patients with JIA aged 12–17 years (13.28 ± 0.38 years) were examined. The control group consisted of 46 apparently healthy peers of the corresponding age. A heart ultrasound was performed to establish the functional state of the myocardium. To study tolerance to physical exertion, a six-minute walk test was performed with calculation of Kerdo index before and after the test. The level of catecholamine excretion in the urine and the level of natriuretic peptide in the blood were studied. Results. Patients with juvenile idiopathic arthritis had early signs of right ventricular myocardial systolic dysfunction (a decrease in the right ventricular ejection fraction). At the same time, we detected a decrease in the catecholamine level and an increase in the content of natriuretic peptide, which has a neuromodulatory effect at different levels of the sympathoadrenal system. Conclusions. Activation of the sympathetic division of the autonomic nervous system (increased Kerdo index) in patients with juvenile idiopathic arthritis does not ensure the effectiveness of minimal physical exertion in a six-minute walk test.

Multimodality Evaluation of Aortic Insufficiency and Aortitis in Rheumatologic Diseases.

Aortic insufficiency is commonly observed in rheumatologic diseases such as ankylosing spondylitis, systemic lupus erythematosus, antiphospholipid syndrome, Behçet's disease, granulomatosis with polyangiitis, and Takayasu arteritis. Aortic insufficiency with an underlying rheumatologic disease may be caused by a primary valve pathology (leaflet destruction, prolapse or restriction), annular dilatation due to associated aortitis or a combination of both. Early recognition of characteristic valve and aorta morphology on cardiac imaging has both diagnostic and prognostic importance. Currently, echocardiography remains the primary diagnostic tool for aortic insufficiency. Complementary use of computed tomography, cardiac magnetic resonance imaging and positron emission tomography in these systemic conditions may augment the assessment of underlying mechanism, disease severity and identification of relevant non-valvular/extracardiac pathology. We aim to review common rheumatologic diseases associated with aortic insufficiency and describe their imaging findings that have been reported in the literature.

Rheumatologic diseases in patients with inborn errors of immunity in the USIDNET registry.

There is a gap in clinical knowledge regarding associations between specific inborn errors of immunity (IEIs) and rheumatologic diseases. This study reports the frequency of rheumatologic conditions in a large cohort of patients with IEI using the USIDNET (United States Immunodeficiency Network) registry. We used the USIDNET registry to conduct the analysis. We included all IEI patients within the registry for whom a diagnosed rheumatologic disease was reported. The total number of patients with IEI in our query was 5058. Among those, 278 (5.49%) patients had a diagnosis of rheumatologic disease. This cohort included 172 (61.8%) female and 106 (38.2%) male patients. Rheumatologic complications were highest in the interferonopathies (66.6%), autoimmune lymphoproliferative syndrome (ALPS) (13.7%), and immunoglobulin G subclass deficiency (IgGSD) (11.11%). Additionally, disease patterns were noted to be different in various IEI disease groups. Inflammatory myopathies were the most common rheumatologic condition in patients with X-linked agammaglobulinemia (1.65%), Sjogren's syndrome was the most common rheumatologic disease reported in ALPS patients (6.85%), and systemic lupus erythematosus was the most common rheumatologic disease in patients with chronic mucocutaneous candidiasis (CMC) (7.41%). Rheumatoid arthritis (RA) report rate was highest in patients with IgGSD (3.70%), specific antibody deficiency (SAD) (3.66%), and ALPS (2.74%). This study reports that rheumatologic diseases are frequently observed in patients with IEI. The frequency of different rheumatologic conditions was variable based on the underlying diagnosis. Clinicians caring for patients with IEI should be vigilant to monitor for rheumatologic complications. Key Points • The rates of reported rheumatologic diseases in the USIDNET registry are different in individual IEIs. • Further studies are needed to guide clinicians for detecting rheumatologic conditions earlier in patients with IEI.

Surgical Management of Juvenile Idiopathic Arthritis Related Dentofacial Deformities

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease of childhood and frequently affects the temporomandibular joints (TMJ). JIA TMJ management requires a team approach. Initially, TMJ JIA involvement is managed with systemic therapy or intra-articular medications to treat symptoms and limit the growth deformity, however may later require surgical intervention. This case series describes 4 patients with different presentations and treatments of juvenile idiopathic arthritis affecting the temporomandibular joints to illustrate a proposed treatment algorithm. This algorithm is not designed to be an absolute treatment regimen but a framework to help clarify the presenting problems and interventions that may be considered to treat JIA associated temporomandibular dysfunction and dentofacial deformity. This case series represents the variety of JIA temporomandibular joint disease and offers a graduated appropriate treatment algorithm.

Effects of Dry Needling on Pain in Patients with Knee Osteoarthritis: A Preliminary Study.

Background:Osteoarthritis is one of the most common rheumatological diseases and is also considered as one of the causes of myofascial trigger points (MtrPs). Dry needling (DN) is one of the methods used for the treatment of the MtrPs. The aim of the current study was to investigate the effects of DN on pain in participants with knee osteoarthritis (KOA).Materials and Methods:In this before and after preliminary clinical trial study, patients with mild to moderate KOA were enrolled. In one session, after determining the location of trigger points in quadriceps and gastrocnemius muscles, the patients underwent DN. The pain was evaluated at baseline, by passing 4 days and 1 month from the intervention using the Visual Analog Scale (VAS).Results:Sixteen patients with a mean age of 56.5 (4.53) years old have completed the study and follow-up period. According to the ANOVA analysis, VAS values indicated a significant decrease in pain score at the 4th and 1 month after the intervention compared to baseline (P < 0.001). There was no significant difference between VAS at the 4th day of intervention and also 1 month later of intervention (P = 0.087).Conclusion:The application of one session DN can lead to improvement in pain intensity in participants with mild to moderate KOA.

Osteoporose e comprometimento laríngeo secundários à artrite idiopática juvenil: relato de caso

OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a systemic inflammatory condition and the most common chronic rheumatological disease in childhood. Among complications, patients may have osteoporosis induced by treatment with glucocorticoids or the evolution of the disease. Another rare complication is the involvement of laryngeal cartilage, which may present with stridor or respiratory failure. The purpose of this report is to present a case of JIA with osteoporosis and laryngeal manifestations. CASE REPORT: Female, 15 years old, diagnosed with JIA 12 years ago with irregular treatment that evolved with severe and limiting disease. The patient had stridor and dyspnoea. Laryngoscopy showed involvement of arytenoid cartilage requiring tracheostomy. In addition, the patient was diagnosed with osteoporosis and bilateral coxarthrosis, requiring bilateral total hip arthroplasty.