Common Rheumatologic Conditions Research Articles

Prevalence of Latent Tuberculosis Infection Among Rheumatology Patients and Management Practices in the United Arab Emirates: A Single-Center Retrospective Cohort Study.

Introduction Prior to immunosuppression, rheumatology patients are routinely screened for latent tuberculosis (TB) infection using interferon-gamma release assays (IGRAs). Variability in the management of latent and indeterminate IGRA results across institutionslimited long-term outcome data. A retrospective study was conducted at Tawam Hospital, United Arab Emirates, to investigate the incidence and management protocols associated with positive and indeterminate IGRAresults, as well as TBinfection, among patients with rheumatic conditions. Methods A single-center retrospective observational study was performed at Tawam Hospital, Abu Dhabi, UAE. Ethical approval for this study was obtained from the Tawam Human Research Ethics Committee. Laboratory records and the hospital's electronic medical system were used to obtain information about IGRA results over a 12-year period (April 2010-April 2022). The hospital's electronic medical system was used to obtain patient information and subsequent management approaches of positive and indeterminate IGRAs.Moreover, long-term follow-up data were collected to determine the risk of TB reactivation in the cohort. Results We found a total of 1,012 positive and 223 indeterminate IGRA test results within the 12-year period. Within the rheumatology department, 123 positive and 39 indeterminate IGRA results were identified. In the indeterminate IGRA group, the majority were women (n = 24, 61.5%) and UAE nationals (n = 22, 56.4%), and their mean age was 38.6 years. Systemic lupus erythematosus was the most prevalent rheumatologic condition (n = 21, 53.8%). Thirteen (33.3%) were on disease-modifying anti-rheumatic drugs (DMARDs) and 26 (66.7%) were on corticosteroids during IGRA testing. A total of eight patients (20.5%) received anti-TB medications. In the positive IGRA group, the mean age was 55.7 years and the female-to-male ratio was 3:1. The most common rheumatologic condition was rheumatoid arthritis (n = 69, 56%). Sixty-five (52.8%) patients were on conventional DMARDs, 43 (34.9%) were on corticosteroids during IGRA testing, and 74 (60%) received anti-TB medications. Two cases (1.6%) of active TB infections were detected among patients with positive IGRA tests, both of whom were receiving anti-tumor necrosis factor alpha inhibitor treatment in combination with methotrexate. No cases of active TB infection were observed in the indeterminate IGRA group. Conclusion Long-term data on the risk of TB activation in positive and indeterminate IGRA results for rheumatological conditions are low. It is recommended to reassess the choice of using anti-TNF-α, with a positive IGRA result if no other feasible alternatives can be offered. Our findings stress the importance of age, underlying diseases, and immunosuppressive treatments in interpreting IGRA results and guiding patient management. A large multicenter study is needed to understand the differences and outcomes of such patients in TB endemic and nonendemic geographical areas.

Abstract 27 — Prevalence and Management Approach of Latent Tuberculosis Infection in Rheumatology Patients

Prior to immunosuppression, rheumatology patients are routinely screened for latent tuberculosis infection (LTBI) using Interferon-Gamma Release Assays (IGRA). The management of latent and indeterminate IGRA results varied among institutions and long-term outcome data is lacking. We conducted a retrospective study at Tawam Hospital, United Arab Emirates, to examine the frequency and management of positive and indeterminate IGRA results and tuberculosis infection in rheumatic patients. Methods Laboratory records and hospital electronic medical system were used to obtain information about IGRA results over a 12 years period (April 2010-April 2022). Results In our hospital record over a 12-year period, we found a total of 1012 positive and 223 indeterminate IGRA test results. Within the rheumatology department, 123 positive and 39 indeterminate IGRA results were identified. In the indeterminate IGRA group, the majority were female (n=24, 61.5%), UAE nationals (n=22, 56.4%), and had a mean age of 38.6 years. Systemic lupus erythematosus (SLE) was the most prevalent rheumatologic condition (n=21, 53.8%). 33.3% (n=13) were on disease modifying anti-rheumatic drugs (DMARDs) and 66.7% (n=26) were on corticosteroid during IGRA testing. A total of 8 patients (20.5%) received anti-TB medications. In a positive IGRA group, the mean age of 55.7 years and a female-to-male ratio of 3:1. The most common rheumatologic conditions were RA (n=67, 54.4%). Approximately 52.8% (n=65) of patients were on conventional DMARDs, 66.7% (n=26) were on corticosteroids during IGRA testing, and 60% (n=74) received anti-TB medications. Two cases (1.6%) of active TB infections were detected among patients with positive IGRA tests, both of whom were receiving anti-TNFa inhibitor treatment in combination with methotrexate. No cases of active TB infection were observed in the indeterminate IGRA group. Conclusion Tuberculosis risk in positive and indeterminate IGRA results for rheumatological conditions is low. Further multicenter studies are required to analyze patient outcomes and differences in TB-endemic and non-endemic regions.

Audiovestibular manifestations in patients with rheumatologic diseases: a review

Rheumatologic disorders are chronic and systemic disorders with an estimated global prevalence of 3,200 per 100,000 inhabitants. Several clinical manifestations are known and may lead to physical disability and altered quality of life. The most common audio vestibular manifestations commonly reported are hearing loss, tinnitus, vertigo, and fullness, however, there are some more still unregistered. The aim of this review is to describe the most common audio vestibular features in three of the most common rheumatologic conditions such as rheumatoid arthritis (RA), systemic erythematous lupus and systemic sclerosis (SSc). A literature search was performed with articles published between 2000 to 2021. In this literature review an increased prevalence of hearing loss for RA of 40% and 70% for systemic erythematous lupus was observed. In SSc, due to its early onset and progression, there is no estimated prevalence, however, a notorious hearing impairment in early stages of the disease was described. Vertigo was also described in these disorders, affecting more than 60% of patients. Further studies are in need to describe the affection on early stages of all disorders as well as its progression.

P152 Allele-associated chromatin interactions in primary immune cells uncover mechanisms of gene regulation

Abstract Background/Aims Genome-wide association studies studies have identified many genetic variants associated with the risk of developing common rheumatological conditions. These variants primarily affect regulatory elements of the genome which can affect the expression of genes located far away from their genomic location through chromatin interaction mechanisms. Furthermore, common genetic variants have been associated with specific changes in chromatin interactions in cell lines. Studying chromatin interactions can improve our understanding of these diseases. Here, we aim to study chromatin interactions and how they are affected by genetic variation in primary immune cells that are relevant for rheumatological conditions. Methods We generated promoter Capture Hi-C (pCHi-C) data for primary CD4+ T cells and CD14+ monocytes isolated from peripheral blood of 10 systemic sclerosis patients and 5 healthy controls. We developed a new computational pipeline to explore how genetic variation affects chromatin interactions. Briefly, genotypes were called from the pCHi-C data using a modified GLIMPSE pipeline. Genotypes were phased using the integrated phasing pipeline which combined population haplotype information with the pCHi-C data. Reads were then assigned to the correct allele using SNPsplit and assigned to chromatin loops (CHiCAGO score > 5). A test for allelic imbalance was carried out using a binomial test, and results were consolidated using Fisher’s method. Results We identified 171 and 139 allele-associated loops (FDR < 0.05) in CD4+ T cells and CD14+ monocytes respectively. These interactions are cell-type specific and highly associated with expression quantitative trait loci (eQTLs). We found that 80% of the variants involved in allele-associated loops were eQTLs for at least one gene. These interactions link to the promoters of 62 and 57 distal genes in CD4+ T cells and CD14+ monocytes respectively. Of these, 38 and 31 respectively were also eQTL for those genes. Many of these allele-associated interactions affect genomic regions that are highly significant for the function of these cell populations. For example, a region with variants affecting T cell-specific chromatin interactions is the TRAF3IP3/IRF6/UTP25 locus. In this region there are separate groups of variants which affect gene expression with a complex mechanism. A group of variants overlapping the UTP25 promoter increases interaction strength with the TRAF3IP3 and IRF6 gene bodies. Interestingly, these are also associated with an increase in UTP25 expression but a decrease in TRAF3IP3 and IRF6 expression. Another group of variants located near the IRF6 promoter is associated with increased interactions with the UTP25 promoter. In contrast, these variants are associated with a reduction of UTP25 expression but an increase in TRAF3IP3 and IRF6 expression. Conclusion We created a new pipeline that allows the identification of allele-associated chromatin interactions in primary cells. These new data will be helpful in uncovering mechanisms of gene regulation in loci highly significant to autoimmune conditions. Disclosure D. González-Serna: None. C. Shi: None. M. Kerick: None. J. Hankinson: None. J. Ding: None. A. McGovern: None. M. Tutino: None. G. Villanueva Martin: None. N. Ortego-Centeno: None. J. Luis Callejas: None. J. Martin: None. G. Orozco: None.

Secukinumab Therapy in Refractory Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA), the most common chronic rheumatologic condition in childhood, remains a cause of significant morbidity, particularly in those with spondyloarthropathy, including psoriatic arthritis (PsA) and enthesitis-related arthritis (ERA). While secukinumab was recently approved for the treatment of children and adolescents with ERA and PsA, there is limited published data on its use in JIA, particularly in refractory cases, despite its efficacy in the treatment of adult arthritis. We aim to examine the use of this therapy in JIA in a single pediatric rheumatology center. A retrospective chart review was performed and 10 JIA patients who received treatment with secukinumab were identified. Data extracted included disease activity, patient demographics, comorbidities, medications, and laboratory data. Seven ERA, 2 PsA, and 1 poly JIA patient were treated with secukinumab at our center between April 2011 and July 2021. These patients had notably resistant disease, with a mean disease-modifying antirheumatic drug (DMARD) failure rate of 3.8. One hundred percent of patients who underwent magnetic resonance imaging (MRI) after being on at least 3 months of secukinumab therapy demonstrated improvement in their MRI findings. One patient developed a flare of uveitis while on secukinumab therapy, with no other adverse events recorded in our patients. Secukinumab therapy was recently approved for children and adolescents with ERA and PsA, and may offer an efficacious option given its demonstrated improvement in imaging and joint examination, as well as qualitative reports of pain, even in those who have failed other therapies. However, caution may be warranted in those with a history of uveitis and warrants further study.

002 Paediatric rheumatology training: a naive subject to medical trainees in Tanzania

Abstract Background Pediatric rheumatic disorders are common and result in significant impairment in the quality of life, morbidity and mortality. Tanzania with a population of over 60 million out which children and youth (0–17 years) comprise of > 50% has only one paediatric rheumatologist. Given the low number of trained experts in this sub specialty, it is expected training of medical students and residents will provide adequate training in paediatric rheumatology. This survey was aimed at determining paediatric rheumatology training among medical students and residents in Tanzania. Methods This was a survey conducted among senior medical undergraduate students who had completed paediatric rotations and final year residents from medical schools in Tanzania. Survey questionnaire was administered through interview to final year medical students and final year paediatric resident’s medical schools, the questions asked included duration of rotation in paediatric for undergraduate students and duration of paediatric residency programs. Results There are 11 universities in Tanzania that offer undergraduate (UG) medical degree, and 5 of them offer residency programs for pediatrics and child health. The duration for undergraduate rotation in pediatrics varied among universities with the longest being 12 weeks and the shortest being 7 weeks. The duration for paediatric residency programs is 3 years in all the universities with an exception of one university which the program for 4 years. There were only two universities that reported to train pediatric rheumatology to undergraduate students in the country, and the topics taught to both of the universities were juvenile idiopathic arthritis. Five universities that provide undergraduate training were excluded due to absence of final year UG students. Final year residents in three out of five universities which offer paediatric residency programs reported to have trained in pediatric rheumatology. One university did not have any class session on pediatric rheumatology, and the other was excluded since there were no final year residents. Common paediatric rheumatology topics covered for residents in the three universities were juvenile idiopathic arthritis and systemic lupus erythematosus. Only one of the three universities had an extra class on dermatomyositis. Conclusion and Recommendations Pediatric rheumatology is not adequately taught to university trainees at both undergraduates and paediatric residency levels. There is a need to put emphasis on exposing all trainees in pediatric to catall common pediatric rheumatology conditions because of the severe shortage of rheumatology trained practitioners in Tanzania.

Pulmonary Manifestations in Rheumatological Diseases.

Pulmonary involvement complicates the various aspects of care in patients suffering from autoimmune disorders. The epidemiological data generated over the last 10 to 15 years have improved the overall understanding of the risk factors and pathophysiological mechanisms involved in pulmonary involvement in rheumatological conditions. Recent advances in genetics have provided superior insight into the pathogenesis of autoimmune diseases and the underlying pulmonary involvement. This review article provides a concise overview of the four most common rheumatological conditions associated with pulmonary involvement: systemic lupus erythematosus (SLE), dermatomyositis/polymyositis, rheumatoid arthritis (RA), and systemic sclerosis (SSc). The clinical, epidemiological, and genetic aspects of these diseases are summarized in this article with particular emphasis on the characteristic patterns of pulmonary involvement in radiological imaging and various treatment options for each of these autoimmune diseases and their lung manifestations.

Pathogenesis and predisposing factors in drug-induced aseptic meningitis: A case study

Aseptic meningitis is the inflammation of the meningeal membranes in the absence of an identifiable pathogen. While viruses are the most common culprit, bacteria, fungi, rheumatological conditions, and neoplastic processes are also known causes. It is also an uncommon, yet increasingly reported, adverse effect of certain drugs. Due to its recurrent nature, association with a specific medication, and resolution upon drug withdrawal, DIAM is an important diagnosis of exclusion in prevention and management.

P019 Rheumatology Referrals to Tertiary Care Hospital over a Three Month Period During Covid-19 Pandemic: a Quality Improvement Project

Background/AimsDuring the COVID-19 pandemic, waiting times for routine new referrals to our tertiary rheumatology department was >20 weeks. Therefore, a quality improvement project (QIP) was undertaken to understand the nature of these referrals and develop an alternative option to rheumatology review. Our aim was to reduce waiting times and improve patient experience by better integrating primary, secondary and therapy services, as well as provide additional triage options.MethodsWe conducted a retrospective analysis of all routine referrals over a 3-month period (1st April to 30th June 2020). Urgent referrals including GCA, CTD and EIA were excluded.ResultsA total of 92/143 (64%) patients were referred, a more significant reduction than normal due to the pandemic. Median age [IQR] was 39.5 [28-66.25] years and most referrals (79%) were from primary care. Table 1 represents information included in the referrals. Thirty-one patients had previously undergone a rheumatology review, of which 11 (35%) were seen in our department. Of these, 18/31 (58%) patients had a diagnosis of Hypermobility Spectrum Disorder (HSD) or fibromyalgia. The commonest reason for re-referral was worsening of existing symptoms (n = 11, 35%), with no suggestive of an alternative diagnosis. P019 Table 1Information included in new routine referrals to rheumatologyTotal n = 92 Commonest presenting complaints (n, %) • Polyathralgia51 (55)• Connective tissue disease/vasculitis symptoms13 (14)• Myalgia12 (13)Number of referrals stating joint swelling4 (4%) Working diagnosis • Hypermobility spectrum disorder26 (28)• Fibromyalgia23 (25)• Connective tissue disease21 (23)• Inflammatory arthritis21 (23)• Osteoarthritis1 (1) Laboratory tests performed (n = 62, %) • Inflammatory markers checked49 (79)o Patients with raised inflammatory markers19 (39)• Antibodies (RhF, Anti-CCP, ANA/ENA)36 (58)o ANA/ENA in conjunction with RhF25 (69)o Anti-CCP in conjunction with RhF5 (14) Patients with polyathralgia and/or myalgia (n = 62, %) • Inflammatory markers checked28 (45)• RhF +/- Anti-CCP antibody21 (33)• ANA/ENA12 (19) Imaging (n = 13, %) • X-ray7 (54)• MRI3 (23)• CT3 (23) Treatment documented (n = 37, %) • Paracetamol9 (24)• Non-steroidal anti-inflammatory therapies10 (27)• Opiates17 (46)• Neuropathic agents11 (30)• Combination of opiates and neuropathic agents5 (14)• Immunosuppression (including steroids)6 (7) MDT input (n = 20, %) • Physiotherapy19 (95)• Occupational therapy1 (5)• Mental health services1 (5)ConclusionOur QIP identified a variation in the quality of referrals and that a high proportion of referrals concerned HSD and fibromyalgia, with many re-referred due to exacerbation of their existing disease. Based on this, we conducted a regional GP trainee educational session and highlighted: i) key features in investigation and management of common rheumatological conditions ii) vital information to include in referrals based on presenting complaint(s) and working diagnosis. We developed a pathway for patients previously diagnosed with fibromyalgia or HSD in our department and re-referred with worsening symptoms, to be triaged into a newly set up weekly specialist MSK physiotherapy-led clinic with rheumatology supervision. Future work will involve re-assessing routine new-referral waiting times and evaluating pre- and post-physiotherapy intervention MSK and quality of life scores, with the aim of formulating a business case to conduct this clinic on a permanent basis. We hope incorporating this pathway will lead to improved patient outcomes and ease some departmental management decisions.Disclosure B. Khan: None. T. David: None. C. Clegg: None. B. Parker: None.

Considerations for the Primary Care Provider Caring for Patients with Juvenile Idiopathic Arthritis Receiving Biologic Therapy.

Juvenile idiopathic arthritis (JIA) is the most common rheumatologic condition in children. JIA is a chronic autoimmune disease with no known cure affecting more than 1 in 1,000 children. With the development of targeted biologic therapies for JIA in the late 1990s, the outlook for children with JIA dramatically improved. Since that time, many children have received biologic therapies, allowing for better disease control and even disease remission. Given the frequency of JIA and increasing use of biologic medications, primary care providers require a basic understanding about these medications and their side effects as well as how the primary care provider can best support the patients who are receiving biologic therapy. [Pediatr Ann. 2022;51(2):e54-e56.].

Positron emission tomography/magnetic resonance imaging in musculoskeletal benign conditions.

A wide spectrum of benign musculoskeletal (orthopedic and rheumatological) conditions affect the general population. Collectively, these are common, and they can inflict significant morbidity with resultant negative impact on the quality of life of patients. For many of these conditions, there is established evidence for research and clinical use of PETCT and MRI for assessment of disease. Introduction of integrated PET/MRI around a decade ago brought optimism that combining the strength of PET and MRI techniques on a single platform could have synergistic effect to benefit imaging assessment of patients, including in the context of benign musculoskeletal conditions. This review specifically focuses on the progress that has been made. This aims to showcase clinical studies derived primarily from the integrated PET/MRI platforms for the evaluation of common orthopedic and rheumatological conditions. Despite enthusiasm and progress by early adopters of the PET/MRI technology, significant barriers are present for its wider adoption, validation, and translation to routine clinical practice. Attenuation correction is a particular challenge which affects regions close to the skeleton and impacts PET/MRI assessment of musculoskeletal disorders. Continued effort on research and validation, as well as promotion of its multimodal multiparametric capability to clinical and pharmaceutical stakeholders, and increased availability through increased adoption of PET/MRI scanners internationally, may accelerate its translation into routine clinical practice in this domain.

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.

CONTEMPORARY UNDERSTANDING OF THE PATHOGENESIS OF RHEUMATOID ARTHRITIS.

Rheumatoid Arthritis (RA) is one of the most common rheumatologic conditions. Dell et al (2013) denes RA as a systemic autoimmune polyarticular arthritis, which can also have extra-articular manifestations that can lead to various systemic complications,. It is more common in women and can present at any age, however, the peak age of onset is the fth decade. RA mainly involves the synovial membrane leading to synovial inammation, proliferation, pannus formation and destruction of the articular cartilage, peri- articular bone and soft tissues.

Management of inflammatory temporomandibular joint collapse in children

Juvenile idiopathic arthritis is the most common pediatric rheumatologic condition.( Abramowicz et al., 2016 Jul) 2 The etiology and pathogenesis have not been fully elucidated; a combination of environmental and certain immunogenic factors is suspected. This review will provide current knowledge and concepts of diagnosis and management of children with JIA and TMJ arthritis.

Comparing quality and readability of online English language information to patient use and perspectives for common rheumatologic conditions.

Evaluate quality and readability of online information for common rheumatologic diseases. Compare rheumatology patients' internet use and preferences to an objective evaluation of internet quality and readability. Five common rheumatologic diseases were searched on the web browser Google using English language. The first twenty websites from each of the five searches were evaluated for internet quality (e.g. content that is current, balanced, has specific aims, and is appropriately cited) using the DISCERN criteria and readability using the Flesch-Kincaid Grade Level (FKGL). The results were contrasted with a survey sent to patients with rheumatic disease. The survey measured patient likeliness to use and trust identified websites. Internet quality was similar (good) for all five diseases searched while readability was poor. There was an inverse relationship between internet quality and readability. Internet quality significantly differed across website sponsor, and readability significantly differed across disease and website sponsor. Common medical website sponsors with the highest combined quality and readability scores were Mayo Clinic and Web MD. Eight hundred and fifty-eight patients were sent a survey, of which 147 (17%) completed. Patients indicated they were most likely to use and trust a Mayo Clinic-sponsored website when compared to other common sponsored websites from our evaluation, followed by the American College of Rheumatology. Although we found good-quality information, all websites evaluated had readability levels above the recommended sixth-grade reading level. The website sponsor with the highest combined readability and quality score was also the most used and trusted by patients. Patients would like more information about credible and trusted websites from their medical providers.

Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic.

Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH) is an acute and rapidly progressive systemic inflammatory disorder characterized by cytopenia, excessive cytokine production, and hyperferritinemia. Common clinical manifestations of HLH are acute unremitting fever, lymphadenopathy, hepatosplenomegaly, and multiorgan failure. Due to a massive cytokine release, this clinical condition is considered as a cytokine storm syndrome. HPS has primary and acquired (secondary, reactive) forms. Its primary form is mostly seen in childhood and caused by various mutations with genetic inheritance and, therefore, is called familial HLH. Secondary HLH may be caused in the presence of an underlying disorder, that is, secondary to a malignant, infectious, or autoimmune/autoinflammatory stimulus. This paper aims to review the pathogenesis and the clinical picture of HLH, and its severe complication, the cytokine storm, with a special emphasis on the developed classification criteria sets for rheumatologists, since COVID-19 infection has clinical symptoms resembling those of the common rheumatologic conditions and possibly triggers HLH. MED-LINE/Pubmed was searched from inception to April 2020, and the following terms were used for data searching: “hemophagocytic syndrome” OR “macrophage activation syndrome” OR “hemophagocytic lymphohistiocytosis”, OR “cytokine storm”. Finally, AND “COVID-19” was included in this algorithm. The selection is restricted to the past 5 years and limited numbers of earlier key references were manually selected. Only full-text manuscripts, published in an English language peer-reviewed journal were included. Manuscript selection procedure and numbers are given in Fig. 2. Briefly, the database search with the following terms of “Hemophagocytic syndrome” OR “Macrophage activation syndrome” OR “Hemophagocytic lymphohistiocytosis” OR “Cytokine storm” yielded 6744 results from inception to April 2020. The selection is restricted to the past 5 years and only limited numbers of earlier key references were selected, and this algorithm resulted in 3080 manuscripts. The addition of (AND “COVID-19”) resulted in 115 publications of which 47 studies, together with four sections of an online book were used in the final review. No statistical method was used. HLH is triggered by genetic conditions, infections, malignancies, autoimmune-autoinflammatory diseases, and some drugs. In COVID-19 patients, secondary HLH and cytokine storm may be responsible for unexplained progressive fever, cytopenia, ARDS, neurological and renal impairment. Differentiation between the primary and secondary forms of HLH is utterly important, since primary form of HLH requires complicated treatments such as hematopoietic stem cell transplantation. Further studies addressing the performance of HScore and other recommendations in the classification of these patients is necessary.

Cardiogenic shock in autoimmune rheumatologic diseases: an insight on etiologies, management, and treatment outcomes.

Autoimmune rheumatological disorders are known to have an increased risk for cardiovascular diseases including coronary artery disease (CAD), myocarditis, pericarditis, valvulopathy, and in consequence cardiogenic shock. Data on cardiogenic shock in rheumatological diseases are scarce; however, several reports have highlighted this specific entity. We sought to review the available literature and highlight major outcomes and the management approaches in each disease. Systematic literature search, including PubMed, Ovid/Medline, Cochrane Library, and Web of Science, was conducted between January 2000 and December 2009. We reviewed all cases reporting cardiogenic shock with rheumatologic conditions, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Takayasu's arteritis (TA), granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA), and antiphospholipid syndrome (APS). We selected 45 papers reporting a total of 48 cases. Mean age was 39 ± 7.3years and 68.8% were females. Most common rheumatologic conditions associated with cardiogenic shock were SLE (31%), GPA (23%), TA (14.6%), APA (10.4%), and RA (8.3%). Cardiogenic shock was found to be caused by eosinophilic myocarditis in 58% of cases, CAD in 19% of cases, and valvulopathy in 6% of cases. Most patient required high-dose steroids and second immunosuppressant therapy. Mechanical circulatory supported was required in 23 cases, IABP in 16 cases, and ECMO in 12 cases. Complete recovery occurred in 37 patients while 9 patients died and 2 required heart transplant. Responsible for two-thirds of cases, eosinophilic myocarditis should be suspected in young cardiogenic shock patients with underlying rheumatologic conditions. Lupus and GPA are the two most common conditions.

Polymyalgia Rheumatica: a Common Disease in Seniors.

Polymyalgia rheumatica (PMR) is one of the most common inflammatory rheumatologic condition occurring in older adults. It is characterized by proximal pain and stiffness in the shoulders, neck, and/or pelvic girdle in individuals over 50years of age along with evidence of an intense systemic inflammatory response. Although the above clinical symptoms are very characteristic for the condition, it can be mimicked by other autoimmune, infectious, malignant, and endocrine disorders chief among which are giant cell arteritis (GCA) and elderly-onset rheumatoid arthritis (EORA). Recently, PMR was reported in relation to treatment with immune checkpoint inhibitors. Current treatment of PMR consists of low-to-medium doses of glucocorticosteroids (GC) with variable response rates and disease recurrence estimated to occur in 50% of patients while tapering down GC doses. In addition, GC-based regimens cause much of the morbidity associated with PMR in older adults, requiring close monitoring for GC-induced toxicity during therapy and highlighting the need for novel therapeutic strategies. Here, we review the latest findings in the field regarding specific etiologic factors, genetic associations, diagnostic methods, and advancements in treatment strategies and disease monitoring indices. Recent discoveries involving novel therapeutic targets in GCA have accelerated the study of PMR pathophysiology and have advanced treatment strategies in PMR management leading to current trials in IL-6 blocking agents. PMR remains an enigmatic inflammatory condition affecting older adults, with current treatment approach causing much morbidity in this patient population. Advancements in our understanding of novel immunopathologic targets can serve as a solid foundation for future treatment strategies in the field.

Pediatric rheumatology in Turkey.

Pediatric Rheumatology is an emerging specialty in Turkey with increasing number of available centers and specialists all over the country. In this paper, we sought to provide an overview on pediatric rheumatology service in our country, as well as to assess the principle published literature from Turkey in this field. A systematic literature search has been performed to achieve the significance and the impact of this manuscript. The most relevant used databases (PubMed/MEDLINE, Web of Science, SCOPUS) for peer-reviewed studies and reviews in English language published during the last 5years were screened. In the first part of the manuscript, we tried to give more details on the history of pediatric rheumatology in Turkey. In further text, we put an accent over the most common rheumatologic conditions among children in Turkey, including Familial Mediterranean fever, juvenile idiopathic arthritis, juvenile spondyloarthropathies, and childhood vasculitides. Despite the considerable literature from Turkey on pediatric rheumatic diseases, a need for unique strategies that would guide the management of rheumatic diseases in childhood remains open. The cultural and historical inheritance together with geographical position make the Turkey a suitable ground for investigations in filed of auto-inflammation and all other inflammatory conditions. Prospective, multicentric studies especially among rheumatologic conditions common in this part of the world would give us more relevant data and open new horizons in diseases' management. International collaborations and databases should be highly encouraged and supported, to make the care of pediatric rheumatic disease uniform.

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.