Contents


Preface

Systemic sclerosis (scleroderma) is the most enigmatic and challenging of all rheumatic diseases. To date, it is considered incurable and carries the highest cause-specific mortality of all the connective tissue diseases. Despite the

scleroderma patients.

systemic sclerosis.

advancements in basic, translational, and clinical research that have been made in recent years, the etiology and pathophysiology of this complex and heterogeneous condition remain to be elucidated. The pathogenesis of scleroderma involves an interplay between genetic, epigenetic, and environmental factors that cause alterations in the immune and vascular systems and lead to progressive tissue fibrosis. The heterogeneity, highly variable clinical presentations, multisystemic manifestations, natural history, response to treatment, low prevalence, and level of public awareness or government investment represent some of the reasons for slow progress in the field of systemic sclerosis. Despite the large numbers of clinical trials and the progress made in their design over the last decade, no approved disease-modifying therapies exist for scleroderma to date. Currently available pharmacological therapies predominantly target inflammatory and vascular pathways, have variable and unpredictable clinical efficacy, usually undesirable safety profiles, and only a modest effect on long-term survival. All of these factors contribute to the significant impact of this challenging disease on the quality of life of patients with scleroderma and their families. Nevertheless, life expectancy of scleroderma patients has improved over recent years, mainly because of better disease management and efficacy of available treatment modalities for organ manifestations and complications. However, there is a large unmet need for increasing the awareness of systemic sclerosis among patients, physicians, and allied health professionals, and for comprehensive multidisciplinary care, which should include physical and occupational therapy, and optimization of nutrition, sleep, and sexual or psychological aspects to reduce suffering and disability of

This book covers novel insights into the pathogenetic mechanisms, classification, differential diagnosis, diagnostic methods, clinical management, and available treatment approaches of a number of major organ manifestations of scleroderma, including pulmonary hypertension and gastrointestinal and renal involvement. Furthermore, it provides a comprehensive overview of often neglected aspects such as scleroderma-like syndromes, and the impact of sexual and biopsychosocial issues on the ability of patients to participate in society and to work. Contributors include well-established scleroderma experts, rheumatologists, internal medicine

specialists, a pathologist and dermatologist, young research fellows, and

experienced physiotherapists with an interest in basic, translational, and clinical research in systemic sclerosis. I am indebted to my colleagues for their dedication, expertise, and timely submissions and believe that their contributions reflect the progress made over the last couple of years in understanding and managing

I would like to express my gratitude to all colleagues, research collaborators, my students and mentors in rheumatology, and to all the patients who have taught me

about scleroderma and were sources of inspiration over the years.
