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Rheumatology (Oxford) 2021 Jun 12. Epub 2021 Jun 12.

Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Adult-onset Still's disease (AOSD) is a rare, but characteristic non-familial, multi-genic systemic auto-inflammatory disorder, characterized by high spiking fever, salmon-like evanescent skin rash, polyarthritis, sore throat, hyperferritinemia, and leucocytosis. The hallmark of AOSD is a cytokine storm triggered by dysregulation of inflammation. Nowadays, with advances in anti-cytokine biologic agents, the treatment of AOSD is no longer limited to nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, or conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs). Read More

Joint Bone Spine 2021 Jun 8:105236. Epub 2021 Jun 8.

Department of Medicine, Universitat de Barcelona, Barcelona, Spain; Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Spain. Electronic address:

Objective: To analyze whether immune-mediated diseases (IMDs) occurs in sarcoidosis more commonly than expected in the general population, and how concomitant IMDs influence the clinical presentation of the disease.

Methods: We searched for coexisting IMDs in patients included in the SARCOGEAS-cohort, a multicenter nationwide database of consecutive patients diagnosed according to the ATS/ESC/WASOG criteria. Comparisons were made considering the presence or absence of IMD clustering, and odds ratios (OR) and their 95% confidence intervals (CI) were calculated as the ratio of observed cases of every IMD in the sarcoidosis cohort to the observed cases in the general population. Read More

PLoS Negl Trop Dis 2021 Jun 11;15(6):e0009399. Epub 2021 Jun 11.

Faculty of Health and Medical Sciences, University of Western Australia, Perth, Western Australia, Australia.

Introduction: Intramuscular benzathine penicillin G (BPG) injections are a cornerstone of secondary prophylaxis to prevent acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Uncertainties regarding inter-ethnic and preparation variability, and target exposure profiles of BPG injection are key knowledge gaps for RHD control.

Methods: To evaluate BPG pharmacokinetics (PK) in patients receiving 4-weekly doses in Ethiopia, we conducted a prospective cohort study of ARF/RHD patients attending cardiology outpatient clinics. Read More

JAMA Cardiol 2021 Jun 9. Epub 2021 Jun 9.

Department of Medicine, University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa.

Importance: Rheumatic heart disease (RHD), a sequela of rheumatic fever characterized by permanent heart valve damage, is the leading cause of cardiac surgery in Africa. However, its pathophysiologic characteristics and genetics are poorly understood. Understanding genetic susceptibility may aid in prevention, control, and interventions to eliminate RHD. Read More

Rheumatol Int 2021 Jun 5. Epub 2021 Jun 5.

Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, 45110, Ioannina, Greece.

Current data demonstrated that severe cases of coronavirus-disease-19 (COVID-19) require treatment with antiviral therapy, dexamethasone, supportive care, as well as some anti-rheumatic drugs, among them, cytokine inhibitors and colchicine. Colchicine is an anti-inflammatory drug that is being used in rheumatology for many years to treat mostly gout, calcium pyrophosphate deposition disease, and Familial Mediterranean Fever. Here, we present for the first time, two patients suffering from gout being treated with colchicine, who were affected from severe acute respiratory coronavirus-2 (SARS-CoV-2) syndrome. Read More

Int J Environ Res Public Health 2021 May 18;18(10). Epub 2021 May 18.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.

Fever of unknown origin (FUO) poses a diagnostic challenge, and 18-fluorodexoyglucose positron emission tomography with computed tomography (18FDG-PET/CT) may identify the source. We aimed to evaluate the diagnostic yield of 18FDG-PET/CT in the work-up of FUO. The records of patients admitted to Sheba Medical Center between January 2013 and January 2018 who underwent 18FDG-PET/CT for the evaluation of FUO were reviewed. Read More

Echo Res Pract 2021 May 1. Epub 2021 May 1.

B Rana, Cardiology, Hammersmith Hospitals NHS Trust, London, United Kingdom of Great Britain and Northern Ireland.

Mitral valve disease is common. Mitral regurgitation is the second most frequent indication for valve surgery in Europe and despite the decline of rheumatic fever in western societies, mitral stenosis of any aetiology is a regular finding in all echo departments. Mitral valve disease is therefore one of the most common pathologies encountered by echocardiographers, as both a primary indication for echocardiography and a secondary finding when investigating other cardiovascular disease processes. Read More

Circulation 2021 Jun 1;143(22):2127-2128. Epub 2021 Jun 1.

Adjunct Department of Physiology, University of Texas Southwestern Medical School, Dallas (K.A.T.).

Front Cardiovasc Med 2021 14;8:674805. Epub 2021 May 14.

School of Science and Technology, University of New England, Armidale, NSW, Australia.

Current diagnosis of Acute Rheumatic Fever and Rheumatic Heart Disease (ARF/RHD) relies on a battery of clinical observations aided by technologically advanced diagnostic tools and non-specific laboratory tests. The laboratory-based assays fall into two categories: those that (1) detect "evidence of preceding streptococcal infections" (ASOT, anti-DNAse B, isolation of the Group A from a throat swab) and (2) those that detect an ongoing inflammatory process (ESR and CRP). These laboratory tests are positive during any streptococcal infection and are non-specific for the diagnosis of ARF/RHD. Read More

Cureus 2021 Apr 24;13(4):e14670. Epub 2021 Apr 24.

Neurology, Icahn School of Medicine at Mount Sinai, New York, USA.

Introduction Approximately 5-10% of strokes occur in adults of less than 45 years of age. The rising prevalence of stroke risk factors may increase stroke rates in young adults (YA). We aimed to compare risk factors and outcomes of acute ischemic stroke (AIS) among YA. Read More

Front Cardiovasc Med 2021 11;8:643317. Epub 2021 May 11.

Heart Institute (InCor), School of Medicine, University of São Paulo, São Paulo, Brazil.

infections continue to be a worldwide public health problem, causing various diseases in humans, with rheumatic fever and rheumatic heart disease being the most harmful manifestations. Impetigo and post-streptococcal glomerulonephritis are also important sequelae of skin infections. We have developed a candidate vaccine epitope (StreptInCor) that presents promising results in diverse animal models. Read More

J Ethnopharmacol 2021 May 24;277:114216. Epub 2021 May 24.

State Key Laboratory Breeding Base of Dao-di Herbs, National Resource Center for Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, PR China; State Key Laboratory of Natural Medicines, China Pharmaceutical University, Nanjing, 210009, PR China. Electronic address:

Ethnopharmacological Relevance: Aconitum carmichaelii Debeaux, a famous traditional medicinal herb for collapse, rheumatic fever, and painful joints, always raises global concerns about its fatal toxicity from toxic alkaloids when improperly processed. Therefore, it is urgent to clarify the internal molecular mechanism of processing detoxification on Aconitum and develop simple and reliable approaches for clinical application, which is also of great significance to the rational medicinal use of Aconitum.

Aim Of The Study: The study aimed at developing a complete molecular mechanism exploration strategy in complex medicinal herb decocting system, clarifying the internal molecular mechanism of processing detoxification on Aconitum, and exploring valid approaches for detoxification. Read More

Pediatr Ann 2021 Mar 1;50(3):e98-e104. Epub 2021 Mar 1.

During the 1920s, acute rheumatic fever (ARF) was the leading cause of mortality in children in the United States. By the 1980s, many felt ARF had all but disappeared from the US. However, although ARF and rheumatic heart disease (RHD) rates remain low in the US today, disease burden is unequal and tracks along other disparities of cardiovascular health. Read More

Rheumatol Int 2021 May 25. Epub 2021 May 25.

Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Ankara University, Ankara, Turkey.

The study aimed to evaluate the impact of the coronavirus disease 2019 (COVID-19) in patients with familial Mediterranean fever (FMF) and to assess the relationships between FMF characteristics and severe COVID-19 outcomes such as hospitalization. The study was planned within a national network of 21 different centers. Demographics, FMF-related clinical and genetic characteristics, and COVID-19 outcomes were obtained. Read More

Aust N Z J Public Health 2021 May 24. Epub 2021 May 24.

Menzies School of Health Research, Northern Territory.

Objectives: To explore the acceptability of a novel, outreached-based approach to improve primary and primordial prevention of Strep A skin sores, sore throats and acute rheumatic fever in remote Aboriginal communities.

Methods: A comprehensive prevention program delivered by trained Aboriginal Community Workers was evaluated using approximately fortnightly household surveys about health and housing and clinical records.

Results: Twenty-seven primary participants from three remote Aboriginal communities in the Northern Territory consented, providing 37. Read More

Front Cardiovasc Med 2021 5;8:675339. Epub 2021 May 5.

School of Science and Technology, University of New England, Armidale, NSW, Australia.

The pathogenesis of Acute Rheumatic Fever/Rheumatic Heart Disease (ARF/RHD) and associated neurobehavioral complications including Sydenham's chorea (SC) is complex. Disease complications triggered by Group A streptococcal (GAS) infection are confined to human and determining the early events leading to pathology requires a robust animal model that reflects the hallmark features of the disease. However, modeling these conditions in a laboratory animal, of a uniquely human disease is challenging. Read More

Rheumatol Int 2021 May 20. Epub 2021 May 20.

Department of Rheumatology and Immunology, Hangzhou Normal University Affiliated Hospital, No. 126, Wenzhou Road, Hangzhou, 310015, China.

Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disease that is an exclusive diagnosis that needs to previously exclude infections, tumors, and rheumatic diseases. There are few reports on AOSD overlapping with other rheumatic diseases. We reported a 55-year-old male who presented with a high, daily spiking fever associated with an evanescent salmon-pink rash, arthralgia, and sore throat. Read More

N Z Med J 2021 05 21;134(1535):93-95. Epub 2021 May 21.

Paediatric Cardiologist, Starship Green Lane Paediatric and Congenital Cardiac Service, Auckland; Pū Manawa Aotearoa.

Medicine (Baltimore) 2021 May;100(20):e25954

The First Affiliated Hospital of Xinjiang Medical University, Urumchi, Xinjiang, China.

Introduction: Haploinsufficiency of A20 (HA20) is a novel genetic disease presented by Zhou et al in 2016. A20 is a protein encoded by TNFAIP3. Loss-of-function mutation in TNFAIP3 will trigger a new autoinflammatory disease: HA20. Read More

Lancet Glob Health 2021 May 10. Epub 2021 May 10.

Division of Global Health Equity, Brigham and Women's Hospital, Boston, MA, USA; Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, MA, USA; Program in Global Noncommunicable Disease and Social Change, Department of Global Health and Social Medicine, Harvard Medical School, Boston, MA, USA; Partners In Health, Boston, MA, USA. Electronic address:

Background: Despite declines in deaths from rheumatic heart disease (RHD) in Africa over the past 30 years, it remains a major cause of cardiovascular morbidity and mortality on the continent. We present an investment case for interventions to prevent and manage RHD in the African Union (AU).

Methods: We created a cohort state-transition model to estimate key outcomes in the disease process, including cases of pharyngitis from group A streptococcus, episodes of acute rheumatic fever (ARF), cases of RHD, heart failure, and deaths. Read More

Rheumatol Int 2021 07 12;41(7):1263-1271. Epub 2021 May 12.

Department of Medical Ecology and Hydroclimatology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

To describe the rheumatic and musculoskeletal symptoms at hospitalization as well as their persistence/severity after discharge with coronavirus disease 2019 (COVID-19) and to identify whether age, sex, body mass index (BMI), and length of hospital stay are associated with persistence of these symptoms. In this single-center cohort study, comprising 300 participants, two phone interviews were conducted (2-week and 1-month after hospitalization) and symptoms were queried with a standardized form. This form included musculoskeletal symptoms and other COVID-19 symptoms. Read More

Liver Int 2021 May 12. Epub 2021 May 12.

Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN, USA.

Background: Failure of immunologic homeostasis and resultant hepatocyte destruction in autoimmune hepatitis (AIH) is likely the result of environmental triggers within a permissive genetic architecture.

Aims: We aimed to identify risk factors associated with AIH in a well-phenotyped AIH cohort.

Methods: We prospectively collected environmental questionnaires from 358 AIH cases and 563 healthy controls. Read More

J Korean Med Sci 2021 May 10;36(18):e132. Epub 2021 May 10.

Department of Urology, Kyung Hee University School of Medicine, Seoul, Korea.

Background: Coronavirus disease 2019 (COVID-19) has spread around the globe, and it is important to determine the risk factors of death in the general population. Our study aimed to determine the risk factors of death and severe illness requiring supplemental oxygen therapy based on the demographic and clinical characteristics of COVID-19 patients in Korea.

Methods: In this study, we used data provided by the Korea Disease Control and Prevention Agency (KDCA) and analyzed a total of 5,068 patients with COVID-19, excluding 19 pregnant women and 544 individuals with missing data. Read More

Aust N Z J Public Health 2021 Jun 10;45(3):212-219. Epub 2021 May 10.

Menzies School of Health Research, Charles Darwin University, Northern Territory.

Objective: A high prevalence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) among Aboriginal children in northern Australia is coupled with low understanding among families. This has negative impacts on children's health, limits opportunities for prevention and suggests that better health communication is needed.

Methods: During an RHD echocardiography screening project, Aboriginal teachers in a remote community school created lessons to teach children about RHD in their home languages, drawing on principles of community-led development. Read More

Clin Rheumatol 2021 May 10. Epub 2021 May 10.

Connective Tissue Diseases Research Center, Imam-Reza Hospital, Tabriz University of Medical Sciences, Flat 1, Golgasht St, P.O. Box 5166614756, Tabriz, Iran.

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. Read More

Rheumatology (Oxford) 2021 May 7. Epub 2021 May 7.

Research Center of Systemic Auto inflammatory Diseases and Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.

Objectives: To investigate survival of interleukin (IL)-1 inhibitors in monogenic autoinflammatory disorders (mAID) through drug retention rate (DRR) and identify potential predictive factors of drug survival from a real-life perspective.

Patients And Methods: Multicentre retrospective study analyzing patients affected by the most common mAID treated with anakinra or canakinumab. Survival curves were analyzed with the Kaplan-Meier method. Read More

R Soc Open Sci 2021 Mar 10;8(3):201991. Epub 2021 Mar 10.

Division of Molecular Microbiology, School of Life Sciences, University of Dundee, Dow Street, Dundee, DD1 5EH, UK.

, also known as Group A Streptococcus (GAS), is a Gram-positive human-exclusive pathogen, responsible for more than 500 000 deaths annually worldwide. Upon infection, GAS commonly triggers mild symptoms such as pharyngitis, pyoderma and fever. However, recurrent infections or prolonged exposure to GAS might lead to life-threatening conditions. Read More

J Infect Dev Ctries 2021 Apr 30;15(4):566-572. Epub 2021 Apr 30.

Department of Microbiology, Kidwai Cancer Institute, Bangalore, India.

Introduction: Acute Rheumatic Fever/ Rheumatic Heart Disease (ARF/RHD), a sequel of group A streptococcal (GAS) infection, even today constitutes a public health issue in developing countries including India. Differences in the prevalence of ARF/RHD in countries with a similar prevalence of GAS infections indicate the role of other cofactors in pathogenesis of RHD.

Methodology: We investigated the prevalence of enterovirus (EV) in RHD by probing for both EV RNA and VP1 protein using Nonisotopic In Situ Hybridization (NISH) and Immunohistochemistry (IHC) respectively in 75 valvectomy specimens obtained from RHD cases. Read More

J Med Microbiol 2021 May;70(5)

Department of Microbiology, Seth G S Medical College and King Edward Memorial Hospital, Mumbai, India.

Group A streptococci can trigger autoimmune responses that lead to acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Some autoantibodies generated in ARF/RHD target antigens in the S2 subfragment region of cardiac myosin. However, little is known about the kinetics of these antibodies during the disease process. Read More

Pediatrics 2021 Jun 5;147(6). Epub 2021 May 5.

Department of Pediatrics, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii; and.

Acute rheumatic fever (ARF) is an acute inflammatory process resulting in rheumatic carditis, one of the most common acquired heart diseases in youth. Among the clinical manifestations of carditis, pathologic valve regurgitation and atrioventricular block are included in the criteria for the diagnosis of ARF. Besides atrioventricular block, ARF may often present with other arrhythmias, such as junctional tachycardia (JT). Read More