Systemic Sclerosis (Scleroderma)

Scleroderma is an umbrella term that includes localized skin disease and systemic sclerosis. Localized morphoea and systemic sclerosis are not interchangeable diagnoses. Confirm the documented form before applying organ-based care because systemic sclerosis can affect blood vessels and internal organs as well as skin. The cause is not fully established, and the presentation and course vary substantially among individuals. Most management evidence concerns adults; juvenile-onset systemic sclerosis also requires coordinated specialist care. Systemic sclerosis is a chronic autoimmune connective-tissue disease characterized by interacting vasculopathy, immune dysregulation and fibrosis. Endothelial injury and vascular remodelling narrow small vessels and contribute to tissue ischemia; Raynaud phenomenon and digital ischemic lesions reflect this process. Immune activation and disease-associated autoantibodies accompany activation of fibroblasts and excessive extracellular-matrix deposition in skin and, in some people, the lungs, gastrointestinal tract, heart or kidneys. The RN connects skin and vascular findings with function and possible internal-organ involvement. A reassuring skin appearance does not establish that the lungs, pulmonary circulation, heart, digestive tract or kidneys are unaffected. Care therefore combines symptom assessment, planned organ screening, medication reconciliation, patient teaching...