Pathology of systemic sclerosis

Autopsy studies played a key role in initially characterizing the protean manifestations of systemic sclerosis. Today, the pathologic evaluation of tissue from affected patients is still vital to the diagnosis and management of this disease. Scleroderma is associated with a wide range of distinctive pathologic findings, including morpheaform skin thickening, pulmonary interstitial fibrosis and hypertension, renal thrombotic microangiopathy, and myocardial fibrosis. Microscopic examination of such lesions allows direct visualization of the classic triad of vascular damage, immune dysfunction, and tissue remodeling that is fundamental to disease pathogenesis. Additionally, systemic sclerosis has a wide range of associated complications that can also be seen on pathologic examination of the skin, lungs, kidneys, cardiovascular system, gastrointestinal tract, musculoskeletal system, and placenta. Pathologic recognition of all of these features continues to guide clinical evaluation of the disease process in individual scleroderma patients as well as contributing to a deeper understanding of this devastating condition in general.