Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults
- Christopher P Denton, MD
Christopher P Denton, MD
- Professor of Experimental Rheumatology
- Royal Free Hospital, London
Scleroderma encompasses a spectrum of related disorders, most of which share a characteristic clinical feature of skin thickening due to an excess of collagen fibers. The simplest division of the scleroderma-related disorders is into localized (table 1) and systemic forms (table 2) (see "Overview and classification of scleroderma disorders"). Patients with the systemic forms are more likely to have internal organ involvement, which is usually the target of therapy.
Differentiating between localized and systemic disease and understanding of the systemic sclerosis (SSc) subsets, disease staging, and organ involvement are needed to guide effective use of available therapies.
The etiology and pathogenesis of the scleroderma disorders are poorly understood. As a result, treatment of these conditions is difficult, incomplete, and not curative. (See "Risk factors for and possible causes of systemic sclerosis (scleroderma)" and "Pathogenesis of systemic sclerosis (scleroderma)".)
An overview of the management of adults with SSc will be discussed here. Pretreatment evaluation, systemic immunologic modulation, potentially antifibrotic approaches, and localized and systemic scleroderma disorders in children are discussed separately. (See "Pretreatment evaluation of adults with systemic sclerosis (scleroderma)" and "Immunomodulatory and antifibrotic approaches to the treatment of systemic sclerosis (scleroderma)" and "Localized scleroderma in childhood" and "Juvenile systemic sclerosis (scleroderma)".)
OVERVIEW OF PRETREATMENT EVALUATION
An organ-based pretreatment evaluation is presented separately; however, a few basic principles are discussed below. (See "Pretreatment evaluation of adults with systemic sclerosis (scleroderma)".)
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- OVERVIEW OF PRETREATMENT EVALUATION
- Determining disease subset
- Assessing disease severity
- GENERAL PRINCIPLES OF MANAGEMENT
- ORGAN-BASED TREATMENT
- - Localized scleroderma (morphea)
- - Diffuse skin sclerosis
- - Other cutaneous manifestations
- Calcinosis cutis
- Raynaud phenomenon
- - Arthralgia and/or arthritis
- - Inflammatory myopathy
- - Pericarditis
- - Myocardial fibrosis and myocarditis
- - Erectile dysfunction
- Risk factors for increased mortality
- SUMMARY AND RECOMMENDATIONS