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Harrison's Principles of Internal Medicine, 18e
| Part 15. Disorders of the Joints and Adjacent Tissues > Section 2. Disorders of Immune-Mediated Injury > |
Chapter 323. Systemic Sclerosis (Scleroderma) and Related Disorders
Sections: Definition, Epidemiology, Environmental and Occupational Risk Factors, Pathogenesis, Pathology, Clinical Features, Organ Involvement, Course, Prognosis, Mixed Connective Tissue Disease, Eosinophilic Fasciitis, Further Readings.
Topics Discussed: systemic scleroderma.
Excerpt:"Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology, heterogeneous clinical manifestations, and chronic and often progressive course. The diffuse cutaneous form of SSc (dcSSc) is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs, most notably the lungs, gastrointestinal tract, heart, and kidneys. The early stage of the disease is associated with prominent inflammatory features. Over time, patients develop functional and structural alterations in multiple vascular beds and progressive visceral organ dysfunction due to fibrosis. Although the presence of thickened skin (scleroderma) distinguishes SSc from other connective tissue diseases, scleroderma-like skin induration can occur in localized forms of scleroderma and other disorders (Table 323-1). Patients can be classified into two principal subsets defined largely by the pattern of skin involvement, as well as clinical and laboratory manifestations (Table 323-2). Diffuse cutaneous SSc is associated with progressive skin induration, starting in the fingers and ascending from distal to proximal extremities, the face, and the trunk. These patients are at risk for early pulmonary fibrosis and acute renal involvement. Patients with limited cutaneous..."
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