Features of systemic sclerosis are –
**Core Concept**
Systemic sclerosis, also known as scleroderma, is a chronic autoimmune disease characterized by fibrosis and vascular alterations in the skin and internal organs. The pathophysiology involves immune system dysregulation, leading to the production of autoantibodies and the activation of fibroblasts.
**Why the Correct Answer is Right**
Systemic sclerosis is characterized by the deposition of collagen and other extracellular matrix proteins, leading to thickening of the skin and internal organs. This is mediated by the activation of fibroblasts, which produce excessive amounts of collagen, and the upregulation of transforming growth factor-beta (TGF-Ξ²). The disease also involves vascular alterations, including endothelial cell damage and the formation of microthrombi.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect as systemic sclerosis is not primarily characterized by inflammation, although some inflammation may be present.
**Option B:** This option is incorrect as systemic sclerosis is not primarily characterized by the presence of granulomas.
**Option C:** This option is incorrect as systemic sclerosis is not primarily characterized by the presence of malignant cells.
**Clinical Pearl / High-Yield Fact**
One key feature of systemic sclerosis is the presence of anti-Scl-70 antibodies, which are associated with a higher risk of pulmonary fibrosis and renal involvement. This is an important clinical correlation to remember when evaluating patients with systemic sclerosis.
**Correct Answer: C.** Systemic sclerosis is characterized by fibrosis and vascular alterations in the skin and internal organs.