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Scleroderma: Pathogenesis, Clinical Features, and Management
Mikkel OstergaardScleroderma, also known as systemic sclerosis, is a complex autoimmune disorder characterized by progressive fibrosis and vascular abnormalities affecting multiple organs. This abstract provides an overview of the pathogenesis, clinical features, and management strategies for scleroderma.
The pathogenesis of scleroderma involves immune dysregulation, leading to excessive collagen deposition and fibrosis in the skin and internal organs. Genetic factors, environmental triggers, and aberrant immune responses contribute to the development of the disease. Dysregulated endothelial cell function and vascular damage further contribute to the pathogenesis, leading to tissue ischemia and organ dysfunction.
Clinical manifestations of scleroderma are diverse and can affect the skin, gastrointestinal tract, lungs, heart, kidneys, and musculoskeletal system. The hallmark feature is skin fibrosis, which can range from localized involvement to widespread systemic sclerosis. Other common clinical features include Raynaud's phenomenon, digital ulcers, gastroesophageal reflux disease, interstitial lung disease, pulmonary arterial hypertension, renal crisis, and musculoskeletal pain.
The management of scleroderma is multidisciplinary and tailored to the specific organ involvement and disease severity. Treatment goals include symptom control, prevention of complications, and preservation of organ function. Medications such as immunosuppressants, vasodilators, and antifibrotic agents may be used to target the underlying pathogenesis and manage specific manifestations. Physical and occupational therapy, along with lifestyle modifications, play an essential role in maintaining joint mobility, managing pain, and improving overall quality of life.
The abstract concludes by highlighting the importance of early diagnosis, close monitoring, and collaborative care involving rheumatologists, dermatologists, pulmonologists, cardiologists, and other specialists. Further research is needed to elucidate the underlying mechanisms of scleroderma and develop targeted therapies to improve outcomes for affected individuals.