Abstract
Systemic sclerosis (SSc) can be divided into two main phenotypes: diffuse and limited variants. The terms diffuse and limited refer to the extent of skin involvement. Limited SSc is what was formerly known as the CREST (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias) syndrome. There are several illnesses that might mimic SSc like morphea, nephrogenic systemic fibrosis, and eosinophilic fasciitis. This is a case based review of SSc, diffuse and limited phenotypes.
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References
Steen VD, Powell DL, Medsger Jr TA. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum. 1988;31(2):196–203.
Masi AT, Rodnan GP, Medsger Jr TA, et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980;23:581–90.
Blockmans D, Beyens G, Verhaeghe R. Predictive value of nailfold capillaroscopy in the diagnosis of connective tissue diseases. Clin Rheum. 1996;15(2):148–53.
Reimer G, et al. Correlates between autoantibodies to nucleolar antigens and clinical features in patients with systemic sclerosis (scleroderma). Arthritis Rheum. 1988;31(4):525–32.
Steen VD, et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum. 1994;37(9):1283–9.
Steen VD, Medsger Jr TA. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41:1613–9.
Taylor MH, McFadden JA, Bolster MB, Silver R. Ulnar Artery Involvement in Systemic Sclerosis (scleroderma). J Rheumatol. 2002;29(1):102–6.
Catterall M, Rowell NR. Respiratory function in progressive systemic sclerosis. Thorax. 1963;18:10–5.
Galie N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34:1219.
Clements PJ, Becvar R, Drosos AA, et al. Assessment of gastrointestinal involvement. Clin Exp Rheumatol. 2003;21:S15–18.
Steen VD, Medsger Jr TA. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43:2437–44.
Steen VD, et al. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med. 1984;76:779–86.
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Hatfield, C.M., Bolster, M.B. (2013). Systemic Sclerosis. In: Mahmoudi, M. (eds) Challenging Cases in Rheumatology and Diseases of the Immune System. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5088-7_8
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DOI: https://doi.org/10.1007/978-1-4614-5088-7_8
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