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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 SSc (lcSSc) generally have long-standing Raynaud's phenomenon before other manifestations of SSc appear. Skin involvement in lcSSc is slowly progressive and remains limited to the fingers (sclerodactyly), distal extremities, and face, but the trunk is not affected. A subset of patients with lcSSc have prominent calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, a constellation termed CRESTsyndrome. However, these features may also be seen in patients with dcSSc. Visceral organ involvement in lcSSc tends to show insidious progression. Although the long-term prognosis of lcSSc is better than that of dcSSc, pulmonary arterial hypertension (PAH), interstitial lung disease, hypothyroidism, and primary biliary cirrhosis may develop in the late stage of lcSSc. In some patients, Raynaud's phenomenon and other typical features of SSc occur in the absence of detectable skin thickening. This syndrome has been termed SSc sine scleroderma.

Table 323-1 Conditions Associated with Scleroderma-Like Induration
Table 323-2 Subsets of Systemic Sclerosis (SSc): Limited Cutaneous SSc versus Diffuse Cutaneous SSc

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