Preface

Systemic sclerosis, or scleroderma, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases. Systemic sclerosis is a chronic connective tis‐ sue disease of unknown etiology that causes widespread microvascular damage and exces‐ sive deposition of collagen in the skin and internal organs. Raynaud phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. Since systemic sclerosis presents with symptoms similar to other autoimmune diseases, diagnosis is difficult. The typical patient is a young or middle-aged woman with a history of Raynaud phenomenon who presents with skin induration and internal organ dysfunction. Clinical evaluation and laboratory testing, along with pulmonary function testing, Doppler echocardiography, vid‐ eo-capillaroscopy, and high-resolution computed tomography of the chest, establish the di‐ agnosis and detect visceral involvement. Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involve‐ ment, autoantibody production, and survival. Prognosis is determined by the degree of in‐ ternal organ involvement. Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-spe‐ cific manifestations have improved substantially.

This book covers new insights into genetics of systemic sclerosis; animal models of systemic sclerosis; survival, causes of death, and factors of poor outcome; pulmonary hypertension in systemic sclerosis; cardiovascular risk in systemic sclerosis; possible treatments in systemic sclerosis; video-capillaroscopy in systemic sclerosis; and many more. Contributors include systemic sclerosis experts from all over the world reflecting the collaborative international effort for improved understanding and management of this disease. I am indebted to my colleagues for their dedication, expertise, and timely submissions. Each author must be cer‐ tain that his or her efforts will benefit to all patients suffering from this serious disease such as systemic sclerosis. It is an honor to be the editor of this book.

I am grateful to Ana Pantar for this kind invitation to be an editor of this book. Furthermore, this book could not have been edited without the dedicated technical assistance of the pub‐ lishing process manager Edi Lipovic; I would like to thank him for his patience and willing‐ ness.

*Systemic Sclerosis* book is meant to provide the interested clinician and researcher with the most current understanding of this complex and fascinating disease.

### **Mislav Radić, MD, PhD**

Deputy Head of the Center of Excellence for Systemic Sclerosis, Ministry of Health of the Republic of Croatia, Division of Rheumatology and Clinical Immunology, Internal Clinic of Medical School University of Split, Split, Croatia

**Provisional chapter**
