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Kawasaki Disease and Multisystem Inflammatory Syndrome in Children: common inflammatory pathways of two distinct diseases
Multisystem inflammatory syndrome in children (MIS-C) is a delayed post-inflammatory disorder associated with the previous infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Initially, MIS-C was described as highly similar to Kawasaki disease (KD), a pediatric febrile systemic vasculitis that can lead to the development of coronary artery aneurysms (CAAs). While KD and MIS-C are both inflammatory disorders, the two entities differ in their epidemiological, clinical, immunological, and pathological features. MIS-C clinical and laboratory characteristics are more closely related to toxic shock syndr...
Source: Rheumatic Disease Clinics of North America - March 16, 2023 Category: Rheumatology Authors: Magali Noval Rivas, Moshe Arditi Source Type: research

Managing Immunosuppression in Vasculitis Patients in Times of Coronavirus Disease 2019
The coronavirus disease 2019 (COVID-19) pandemic has led to the emergence of multiple challenges in the care of patients with systemic rheumatic diseases. Patients with vasculitis represent a group of particular concern due to existing risk factors which include a higher burden of comorbidities and specific immunosuppressive therapies used for treatment. Vaccination and the use of other risk mitigation strategies are crucial for the care of these patients. This review provides an overview of existing evidence to contribute to the understanding and specific requirements of the treatment and management of patients with vascu...
Source: Rheumatic Disease Clinics of North America - March 13, 2023 Category: Rheumatology Authors: Sebastian E. Sattui, Zachary S. Wallace Source Type: research

Managing immunosuppression in vasculitis patients in times of COVID-19
The coronavirus disease 2019 (COVID-19) pandemic has led to the emergence of multiple challenges in the care of patients with systemic rheumatic diseases. Patients with vasculitis represent a group of particular concern due to existing risk factors which include a higher burden of comorbidities and specific immunosuppressive therapies used for treatment. Vaccination and the use of other risk mitigation strategies are crucial for the care of these patients. This review provides an overview of existing evidence to contribute to the understanding and specific requirements of the treatment and management of patients with vascu...
Source: Rheumatic Disease Clinics of North America - March 13, 2023 Category: Rheumatology Authors: Sebastian E. Sattui, Zachary R. Wallace Source Type: research

Approach to Systemic Sclerosis Patient Assessment
Systemic sclerosis (SSc) is a heterogeneous disease comprising of a wide spectrum of ages of onset, sex-based differences, ethnic variations, disease manifestations, differential serologic profiles, and variable response to therapy resulting in reduced health-related quality of life, disability, and survival. The ability to subset groups of patients with SSc can assist with refining the diagnosis, guide appropriate monitoring, inform aggressiveness of immunosuppression, and predict prognosis. The ability to subset patients with SSc has several important practical implications for patient care. (Source: Rheumatic Disease Cl...
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Sindhu R. Johnson, Ivan Foeldvari Source Type: research

Treatment of Inflammatory Arthritis in Systemic Sclerosis
Joint involvement, including arthralgia, inflammatory arthritis, joint contractures and overlapping with rheumatoid arthritis, is a common manifestation and is associated with impared quality of life in systemic sclerosis (SSc). Few studies have evaluated the treatment of arthritis in SSc. Pharmacological approach includes low-dose corticosteroids, methotrexate, and hydroxychloroquine. Non-tumor necrosis factor biologics, especially rituximab and tocilizumab, may be a promising option for refractory cases. (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Cristiane Kayser, Lucas Vict ória de Oliveira Martins Source Type: research

Expanding the Treatment Team
The optimal systemic sclerosis (SSc) care plan includes an occupational therapist and physical therapist as well as wound care experts and a registered dietitian if indicated. Screening instruments for functional and work disability, hand and mouth limitations, malnutrition, and dietary intake can identify the need for ancillary support services. Telemedicine can assist in developing effective ancillary treatment plans. Reimbursement for services may limit access for patients with SSc to expand their care team but a focus on prevention rather than management of damage is recognized as an important unmet need in SSc. In thi...
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Tracy M. Frech, Janet L. Poole, Maureen Murtaugh, Marco Matucci-Cerinic Source Type: research

Mental Health Considerations in Chronic Disease
Each person who presents for scleroderma-focused care not only has their own psychosocial stressors in their day-to-day life but they also have scleroderma symptom-specific stressors as well as their own mental health reactions throughout their journey with this disease course. There are many actions patients can take to help and support themselves when they are faced with any of the mental health and social determinants of health stressors associated with this rare, chronic illness. Using the scleroderma specialty providers to inform, discuss, and address these areas with their patients can assist with more effective symp...
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Nancy Lazar, Virginia D. Steen Source Type: research

The Exciting Future for Scleroderma
This article reviews recent progress in understanding how fibrosis becomes profibrotic and how the immune system, vascular, and mesenchymal compartment affect disease development. Early phase trials are informing about pathogenic mechanisms in  vivo and reverse translation for observational and randomized trials is allowing hypotheses to be developed and tested. In addition to repurposing already available drugs, these studies are paving the way for the next generation of targeted therapeutics. (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: J örg H.W. Distler, Gabriela Riemekasten, Christopher P. Denton Source Type: research

Health Care Utilization
Systemic sclerosis (SSc), also known as scleroderma, is a chronic autoimmune connective tissue disease and is associated with a significant economic burden resulting from health care utilization costs in addition to indirect costs attributable to SSc resulting from early retirement and lost productivity in those that remain in employment. (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Kathleen Morrisroe, Nora Sandorfi, Murray Barron Source Type: research

Primary Cardiac Involvement in Systemic Sclerosis
Primary cardiac involvement in systemic sclerosis (SSc) is an important cause of morbidity and mortality. Abnormalities of cardiac structure and function can be detected on routine cardiopulmonary screening that is the standard of care for SSc monitoring. Cardiovascular magnetic resonance-extracellular volume (indicating diffuse fibrosis) and cardiac biomarkers may identify at-risk patients who would benefit from further evaluation including screening for atrial and ventricular arrhythmias with implantable loop recorders. The role of algorithm-based cardiac evaluation both before and after therapeutic initiation is one of ...
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Erin Chew, Vineet Agrawal, Tracy Frech Source Type: research

Best Approaches to the Care of Systemic Sclerosis Patients
In this issue, the world ’s leading experts review the best approaches to patient care for patients with systemic sclerosis. Systemic sclerosis is an autoimmune disease with severe multiorgan system manifestations with heterogeneous rates of progression, which occurs in children and adults. This comprehensive series cover s a thoughtful approach to assessing an individual patient and applying scientific principles of vasculopathy, fibrosis, and immune dysfunction to treatment decisions. Organ-specific manifestations and treatments, including skin, joint, heart, lung, and kidney, are reviewed by expert rheumatologists . (...
Source: Rheumatic Disease Clinics of North America - March 1, 2023 Category: Rheumatology Authors: Tracy M. Frech Tags: Preface Source Type: research

Gastrointestinal Tract Considerations Part I
The upper gastrointestinal (GI) tract is frequently involved in  systemic sclerosis (SSc) and may impact quality of life, physical function and survival. Although we are currently very proactive in terms of screening for heart and lung involvement, patients with SSc are not routinely screened for GI involvement. This review details the available investigations for common upper GI symptoms in SSc, including dysphagia, reflux and bloating and provides advice as to how to integrate these investigations into current clinical care. (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - February 28, 2023 Category: Rheumatology Authors: Alannah Quinlivan, Zsuzsanna H. McMahan, Eun Bong Lee, Mandana Nikpour Source Type: research

Time Trends of Cardiovascular Disease in the General Population and Inflammatory Arthritis
Cardiovascular diseases (CVDs) are the leading causes of death in the world, but declining trends for cardiovascular (CV) mortality and morbidity have been observed during the last decades. Reports on secular trends regarding the excess CV mortality and morbidity in rheumatoid arthritis show diverging results. Data support that also patients with inflammatory arthritis have benefited from improved treatment and prevention for CVD, which can be observed, for example, in decreased case fatality after CV event. However, several recent studies indicate a remaining excess CV risk in patients with inflammatory arthritis. (Source...
Source: Rheumatic Disease Clinics of North America - November 22, 2022 Category: Rheumatology Authors: Anna S ödergren, Solbritt Rantapää-Dahlqvist, Lotta Ljung Source Type: research

Atherosclerotic Cardiovascular Risk Stratification in the Rheumatic Diseases:
Cardiovascular disease (CVD) risk is increased in most inflammatory rheumatic diseases (IRDs), reiterating the role of inflammation in the initiation and progression of atherosclerosis. An inverse association of CVD risk with body weight and lipid levels has been described in IRDs. Coronary artery calcium scores, plaque burden and characteristics, and carotid plaques on ultrasound optimize CVD risk estimate in IRDs. Biomarkers of cardiac injury, autoantibodies, lipid biomarkers, and cytokines also improve risk assessment in IRDs. Machine learning and deep learning algorithms for phenotype and image analysis hold promise to...
Source: Rheumatic Disease Clinics of North America - November 22, 2022 Category: Rheumatology Authors: Durga Prasanna Misra, Ellen M. Hauge, Cynthia S. Crowson, George D. Kitas, Sarah R. Ormseth, George A. Karpouzas Source Type: research

Lessons from Cardiac and Vascular Biopsies from Patients with and without Inflammatory Rheumatic Diseases
Feiring Heart Biopsy Study enables searching for potential pathogenetic mechanisms, therapeutic targets, and biomarkers through the assessment of clinical data and multiple blood and tissue samples from patients with and without inflammatory rheumatic diseases (IRDs), undergoing coronary artery bypass grafting. Some of our findings, for example, more inflammation (including the presence of immune cells and expression of proinflammatory cytokines) in vessels and the heart, and the presence of certain bacteria and autoantigens in vessels, could contribute to the increased risk of ischemia, aneurysms, and/or cardiac dysfuncti...
Source: Rheumatic Disease Clinics of North America - November 22, 2022 Category: Rheumatology Authors: Ivana Hollan Source Type: research