Abstract
Systemic sclerosis is a prototypic fibrotic disease characterised by accumulation of excessive extracellular matrix in affected tissues, including skin, lung, and walls of blood vessels, that can lead to pulmonary hypertension, scleroderma renal crisis, or digital ischaemia. The constellation of clinical manifestations makes systemic sclerosis one of the most challenging of the immune-mediated rheumatic diseases to treat. One of the hallmarks of the disease is its clinical diversity, and the natural history of progression, improvement, or stability in the skin and lung is increasingly appreciated as a predictor of outcome and disease burden.
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