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Pathology of Systemic Sclerosis

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Scleroderma

Abstract

Autopsy studies played a key role in initially characterizing the protean manifestations of systemic sclerosis. Today, 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 deeper understanding of this devastating condition in general.

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Authors and Affiliations

  1. Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA

    Lisa M. Rooper MD

  2. Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Frederic B. Askin MD

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  1. Lisa M. Rooper MD
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  2. Frederic B. Askin MD
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Correspondence to Frederic B. Askin MD .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA

    John Varga

  2. Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Hospital, London, United Kingdom

    Christopher P. Denton

  3. Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland, USA

    Fredrick M. Wigley

  4. Service de Rhumatologie A, INSERMU1016, Universit. Paris Descartes, Hôpital Cochin, Paris, France

    Yannick Allanore

  5. Department of Allergy and Rheumatology, Nippon Medical School, Graduate School of Medicine, Tokyo, Japan

    Masataka Kuwana

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Rooper, L.M., Askin, F.B. (2017). Pathology of Systemic Sclerosis. In: Varga, J., Denton, C., Wigley, F., Allanore, Y., Kuwana, M. (eds) Scleroderma. Springer, Cham. https://doi.org/10.1007/978-3-319-31407-5_12

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