Abstract
Systemic sclerosis (scleroderma) is classified based on the extent of skin involvement as either limited cutaneous or diffuse cutaneous scleroderma. Patients with the limited cutaneous form of systemic sclerosis, once known as the CREST syndrome (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias), often report a long history of Raynaud’s phenomenon preceding other clinical manifestations. Current classification criteria lack sensitivity for this subset of scleroderma, as it may take years or even decades before these patients fulfill classification criteria. Certain clinical and laboratory findings can facilitate earlier diagnosis, particularly the presence of digital pitted scars, telangiectasias, abnormal nailfold capillary morphology, and the presence of anticentromere antibodies (ACA). One should consider the diagnosis of limited cutaneous systemic sclerosis in any patient who gives a history of Raynaud’s phenomenon with one or more of these clinical and laboratory features, or with signs and symptoms suggesting visceral organ involvement. Although limited in the extent of skin involvement, these patients must be monitored for the development of internal organ complications such as pulmonary arterial hypertension, pulmonary fibrosis, and a variety of gastrointestinal complications.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Leroy EC, Medsger TA Jr, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202-205.
Masi AT, Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980;23:581-590.
Maricq HR, Valter I. A working classification of scleroderma spectrum disorders: a proposal and the results of testing on a sample of patients. Clin Exp Rheumatol. 2004;22(3 suppl 33):S5-S13.
Hudson M, Fritzler MJ, Baron M, Canadian Scleroderma Research Group (CSRG). Systemic sclerosis: establishing diagnostic criteria. Medicine (Baltimore). 2010;89:159-165.
Steen VD. Epidemiology and classification of scleroderma. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 4th ed. Amsterdam: Mosby Elsevier; 2008:1361-1368.
Gitzelmann MM, Koppensteiner R, Amann-Vesti BR. Predictive value of nailfold capillaroscopy in patients with Raynaud’s phenomenon. Clin Rheumatol. 2006;25:153-158.
Murray AK, Moore TL, Manning JB, Taylor C, Griffiths CE, Herrick AL. Noninvasive imaging techniques in the assessment of scleroderma spectrum disorders. Arthritis Rheum. 2009;61:1103-1111.
Duchini A, Sessoms SL. Gastrointestinal hemorrhage in patients with systemic sclerosis and CREST syndrome. Am J Gastroenterol. 1998;93:1453-1456.
Shah AA, Wigley FM, Hummers LK. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. J Rheumatol. 2010;37:98-104.
Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 2009;48(Suppl 3):iii36-iii39.
Franck-Larsson K, Graf W, Rönnblom A. Lower gastrointestinal symptoms and quality of life in patients with systemic sclerosis: a population-based study. Eur J Gastroenterol Hepatol. 2009;21:176-182.
Sugimoto T, Soumura M, Danno K, et al. Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis. Mod Rheumatol. 2006;16:309-311.
Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655-2666.
McLaughlin V, Humbert M, Coghlan G, Nash P, Steen V. Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis. Rheumatology (Oxford). 2009;48(Suppl 3):iii25-iii31.
Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CP. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103:109-115.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2011 Springer-Verlag London Limited
About this chapter
Cite this chapter
Silver, R.M. (2011). A 35-Year-Old Woman with Puffy Hands, Raynaud’s Phenomenon, and Positive Antinuclear Antibody Test. In: Silver, R., Denton, C. (eds) Case Studies in Systemic Sclerosis. Springer, London. https://doi.org/10.1007/978-0-85729-641-2_1
Download citation
DOI: https://doi.org/10.1007/978-0-85729-641-2_1
Published:
Publisher Name: Springer, London
Print ISBN: 978-0-85729-640-5
Online ISBN: 978-0-85729-641-2
eBook Packages: MedicineMedicine (R0)