Rheumatic Diseases Clinics of North America

No abstract text is available yet for this article.

No abstract text is available yet for this article.

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...

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...

Emerging evidence shows that a complex interplay between cells and mediators and extracellular matrix factors may underlie the development and persistence of fibrosis in systemic sclerosis. Similar processes may determine vasculopathy. 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...

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 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...

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...

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 symptom and disease self-management...

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...

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.

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 multidisciplinary team including rheumatology, pulmonology, and cardiology...

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.

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 precisely...

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...

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 desirable are discussed...

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...

As skin involvement is the hall mark of systemic sclerosis (SSc) and changes of skin involvement have shown to correlate with internal organ involvement, assessing the extend of skin involvement is key. Although the modified Rodnan skin score is a validated tool used to evaluate the skin in SSc, it has its drawbacks. Novel imagine methods are promising but should be further evaluated. As for molecule markers for skin progression there are conflicting data on the predictive significance of baseline SSc skin gene expression profiles, but immune cell type signature in SSc skin correlates with progression...

Systemic sclerosis (SSc) is a heterogenous systemic autoimmune disease of complex multi-organ manifestations with a disease-specific mortality of >50%. The patient journey is fraught with severe, diverse, and diffuse physical impairment, psychological burden, and diminishing health-related quality of life. SSc remains unfamiliar to many clinicians. Delayed/misdiagnosis, inadequate screening, and attention for common complications with potentially preventable disability/death contribute to patients feeling isolated and unsupported...

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...