Treatment of Vascular Complications in Systemic Sclerosis
Vasculopathy as exemplified by scleroderma renal crisis (SRC) and digital ulcers (DUs) is a cardinal feature of systemic sclerosis (SSc) and is associated with significant morbidity, including in patients with early disease. Prompt recognition and management is required to alleviate potentially irreversible damage from SSc-associated vasculopathy. Both SRC and DUs share many etiopathogenic drivers which inform the therapeutic strategy. The aim of our review was to describe the diagnosis and management of SRC and DUs in SSc, and to discuss unmet needs for future research. (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Michael Hughes, Ariane L. Herrick, Marie Hudson Source Type: research

Interstitial Lung Disease
Systemic sclerosis-interstitial lung disease (SSc-ILD) is a major complication of SSc resulting in important morbidity and mortality. Next to cyclophosphamide and mycophenolate mofetil, tocilizumab and nintedanib have proven efficacy in the treatment of SSc-ILD. The highly variable course of SSc-ILD, the complexity in determining and predicting the progression of SSc-ILD, and the diversity of treatment options for SSc-ILD, pose many challenges for everyday clinical practice. In this review, currently available evidence for monitoring and treatment of SSc-ILD is summarized and areas where additional evidence is highly desir...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Cosimo Bruni, Corrado Campochiaro, Jeska K. de Vries-Bouwstra Source Type: research

Gastrointestinal Tract Considerations: Part II
Lower gastrointestinal (GI) symptoms are a frequently encountered problem for clinicians managing patients with systemic sclerosis. The current management practices are focused on the treatment of symptoms with little information available on how to use GI investigations in daily practice. This review demonstrates how to integrate the objective assessment of common lower GI symptoms into clinical care with the aim of guiding clinical decision making. Understanding the type of abnormal GI function that is affecting a patient and determining which parts of the gut are impacted can help clinicians to target therapy more preci...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Alannah Quinlivan, Zsuzsanna H. McMahan, Eun Bong Lee, Mandana Nikpour Source Type: research

Preventative Care in Scleroderma
Systemic sclerosis (SSc) is a rare multisystem autoimmune disease characterized by fibrosis, vasculopathy, and autoimmunity. Lesser known complications inherent to SSc, such as malignancies and osteoporosis, can lead to decreased quality of life and increased morbidity and mortality. Patients with SSc have a greater risk of developing malignancies than the general population. In addition, they are more likely to be vitamin D deficient and are at great risk of osteoporosis-related fractures. However, these complications can be addressed through preventative measures. The purpose of this review is to provide clinicians with ...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Leonardo Martin Calderon, Robyn T. Domsic, Ami A. Shah, Janet E. Pope Source Type: research

Preventative Care in Scleroderma
Systemic sclerosis (SSc) is a rare multisystem autoimmune disease characterized by fibrosis, vasculopathy, and autoimmunity. There are multiple complications inherent to SSc and its management. One of these complications is increased infection risk, which can lead to decreased quality of life and increased morbidity and mortality. Patients with SSc have lower vaccination rates and decreased vaccine seroconversion secondary to immunosuppressive medications compared with the general population. The purpose of this review is to provide clinicians with an approach to vaccinations in SSc. (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Leonardo Martin Calderon, Janet E. Pope, Ami A. Shah, Robyn T. Domsic Source Type: research

Training the Next Generation of Rheumatologists
Rheumatology is rich in educational opportunities, learning about a variety of diseases. Rheumatology subspecialty training is a time of unparalleled learning, and within the curriculum of a training program, the connective tissue diseases (CTDs) represent a unique challenge to the fellows. The challenge therein lies in the multisystem presentations they are faced with mastering. Scleroderma, as a rare and life-threatening CTD, remains one of the most difficult conditions to manage and treat. In this article, the authors focus on an approach to training the next generation of rheumatologists to take care of patients with s...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Tatiana S. Rodr íguez-Reyna, Faye N. Hant, Maurizio Cutolo, Vanessa Smith Source Type: research

Pulmonary Hypertension
Pulmonary hypertension (PH) is a leading cause of morbidity and mortality in systemic sclerosis (SSc). PH is a heterogenous condition and several different forms of PH are associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to interstitial lung disease, PH due to left heart disease, and PH due to thromboembolic disease. Extensive research has led to an improved understanding of the mediators involved in the pathogenesis of SSc-PH. Initial combination therapy is the preferred treatment approach for SSc-PAH and requires coordinated care with a multidis...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Benjamin D. Korman, Daniel J. Lachant, Flavia V. Castelino Source Type: research

Patient Experience of Systemic Sclerosis –Related Calcinosis
Systemic sclerosis (SSc) -related calcinosis can be a debilitating, constantly painful, poorly understood vascular complication of calcium hydroxyapatite deposition in soft tissue structures that affects approximately 40% of both limited and diffuse cutaneous SSc subtypes. This publication describes the iterative and multitiered international qualitative investigations that yielded remarkable insights into natural history, daily experience, and complications of SSc-calcinosis providing pivotal information for health management. Patient-driven question development and field testing, according to Food and Drug Administration...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Lesley Ann Saketkoo, Jessica K. Gordon, Kim Fligelstone, Anne Mawdsley, Humza A. Chaudhry, Antonia Valenzuela, Angela Christensen, Samara M. Khalique, Kelly Jensen, Sophia C. Weinmann, Evan Busman, Lorinda Chung, Vivien M. Hsu, Anne-Marie Russell, Virgini Source Type: research

Scleroderma: Best Approaches to Patient Care
This issue on systemic sclerosis is quite unlike anything that has come before, and the kudos have to go to Tracy Frech, who orchestrated the effort and selected the topics. For the very first time, we have a scholarly review of systemic sclerosis that is patient centered, comprehensive, and focused on clinical care that also addresses mental health, physical therapy, occupational therapy, and preventive care alongside organ system approaches. It has long been recognized that subsetting systemic sclerosis patients is critical for prognosis; now with modern tools and increased knowledge of therapeutic pathways, we can appro...
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Michael H. Weisman Tags: Foreword Source Type: research

Scleroderma: Best Approaches to Patient Care
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Authors: Tracy M. Frech Source Type: research

Copyright
ELSEVIER (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Source Type: research

Contributors
MICHAEL H. WEISMAN, MD (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Source Type: research

Contents
Michael H. Weisman (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Source Type: research

Forthcoming Issues
Vasculitis (Source: Rheumatic Disease Clinics of North America)
Source: Rheumatic Disease Clinics of North America - April 6, 2023 Category: Rheumatology Source Type: research

Kawasaki Disease and Multisystem Inflammatory Syndrome in Children
Multisystem inflammatory syndrome in children (MIS-C) is a delayed postinflammatory 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 2 entities differ in their epidemiological, clinical, immunological, and pathological features. MIS-C clinical and laboratory characteristics are more closely related to toxic shock syndrome...
Source: Rheumatic Disease Clinics of North America - March 16, 2023 Category: Rheumatology Authors: Magali Noval Rivas, Moshe Arditi Source Type: research