Abstract
Scleroderma has a median age of onset in the fifth decade of life; however, there are many individuals who develop scleroderma later or who are aging with this disease. It is important that clinicians are able to recognize features of scleroderma in the elderly and distinguish these from well-established imitators. The age of scleroderma onset can impact the course of disease and increase the risk for organ-specific complications such as pulmonary vascular disease. As individuals age with scleroderma, physicians should focus on careful monitoring of each organ system and comprehensive medical care. Treatment principles should be customized to an individual’s disease, and the importance of nutrition, mobility, and social support emphasized.
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
Wigley FM, Hummers LK. Clinical features of systemic sclerosis. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 3rd ed. New York: Mosby; 2003. p. 1463–79.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, et al. Scleroderma (systemic sclerosis): Classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5.
Medsger Jr TA, Masi AT, Rodnan GP, Benedek TG, Robinson H. Survival with systemic sclerosis (scleroderma). A life-table analysis of clinical and demographic factors in 309 patients. Ann Intern Med. 1971;75(3):369–76.
Jacobsen S, Halberg P, Ullman S. Mortality and causes of death of 344 danish patients with systemic sclerosis (scleroderma). Br J Rheumatol. 1998;37(7):750–5.
Mayes MD, Reveille JH. Epidemiology, demographics, and genetics. In: Clements PJ, Furst DE, editors. Systemic sclerosis. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2004.
Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino Jr AV, et al. Childhood onset systemic sclerosis: Classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006;33(5):1004–13.
Steen VD, Medsger Jr TA. Epidemiology and natural history of systemic sclerosis. Rheum Dis Clin North Am. 1990;16(1):1–10.
Derk CT, Artlett CM, Jimenez SA. Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: A nested case-control study. Clin Rheumatol. 2006;25(6):831–4.
Systemic sclerosis in old age. Br Med J. 1979 Nov 24;2(6201):1313–4.
Maricq HR, Carpentier PH, Weinrich MC, Keil JE, Franco A, Drouet P, et al. Geographic variation in the prevalence of raynaud’s phenomenon: Charleston, SC, USA, vs tarentaise, savoie, france. J Rheumatol. 1993;20(1):70–6.
Smolander J. Effect of cold exposure on older humans. Int J Sports Med. 2002;23(2):86–92.
Planchon B, Pistorius MA, Beurrier P, De Faucal P. Primary raynaud’s phenomenon. age of onset and pathogenesis in a prospective study of 424 patients. Angiology. 1994;45(8):677–86.
Kallenberg CG, Wouda AA, Hoet MH, van Venrooij WJ. Development of connective tissue disease in patients presenting with raynaud’s phenomenon: A six year follow up with emphasis on the predictive value of antinuclear antibodies as detected by immunoblotting. Ann Rheum Dis. 1988;47(8):634–41.
Kallenberg CG. Early detection of connective tissue disease in patients with raynaud’s phenomenon. Rheum Dis Clin North Am. 1990;16(1):11–30.
Ling SM, Wigley FM. Raynaud’s phenomenon in older adults: Diagnostic considerations and management. Drugs Aging. 1999;15(3):183–95.
Friedman EI, Taylor Jr LM, Porter JM. Late-onset raynaud’s syndrome: diagnostic and therapeutic considerations. Geriatrics. 1988;43(12):59,63, 67–70.
Grassi W, Medico PD, Izzo F, Cervini C. Microvascular involvement in systemic sclerosis: Capillaroscopic findings. Semin Arthritis Rheum. 2001;30(6):397–402.
Kabasakal Y, Elvins DM, Ring EF, McHugh NJ. Quantitative nailfold capillaroscopy findings in a population with connective tissue disease and in normal healthy controls. Ann Rheum Dis. 1996;55(8):507–12.
Ho M, Veale D, Eastmond C, Nuki G, Belch J. Macrovascular disease and systemic sclerosis. Ann Rheum Dis. 2000;59(1):39–43.
Stafford L, Englert H, Gover J, Bertouch J. Distribution of macrovascular disease in scleroderma. Ann Rheum Dis. 1998;57(8):476–9.
Wigley FM. Clinical practice. raynaud’s phenomenon. N Engl J Med. 2002;347(13):1001–8.
Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC. Age and risk of pulmonary arterial hypertension in scleroderma. Chest. 2003;124(6):2098–104.
Champion HC. The heart in scleroderma. Rheum Dis Clin North Am. 2008;34(1):181,90; viii.
Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. 2008;34(1):199,220; ix.
Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, et al. Scleroderma-like disorders. Autoimmun Rev. 2008;7(4):331–9.
Blum M, Wigley FM, Hummers LK. Scleromyxedema: A case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG). Medicine (Baltimore). 2008;87(1):10–20.
Gabriel SE, Perry HO, Oleson GB, Bowles CA. Scleromyxedema: A scleroderma-like disorder with systemic manifestations. Medicine (Baltimore). 1988;67(1):58–65.
Dziadzio M, Anastassiades CP, Hawkins PN, Potter M, Gabrielli A, Brough GM, et al. From scleredema to AL amyloidosis: Disease progression or coincidence? review of the literature. Clin Rheumatol. 2006;25(1):3–15.
Dispenzieri A. POEMS syndrome. Blood Rev. 2007;21(6):285–99.
Kyle RA, Rajkumar SV. Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. Hematol Oncol Clin North Am. 2007;21(6):1093,113, ix.
Naschitz JE, Rosner I, Rozenbaum M, Zuckerman E, Yeshurun D. Rheumatic syndromes: Clues to occult neoplasia. Semin Arthritis Rheum. 1999;29(1):43–55.
Haroon M, Phelan M. A paraneoplastic case of palmar fasciitis and polyarthritis syndrome. Nat Clin Pract Rheumatol. 2008;4(5):274–7.
Sheehy C, Ryan JG, Kelly M, Barry M. Palmar fasciitis and polyarthritis syndrome associated with non-small-cell lung carcinoma. Clin Rheumatol. 2007;26(11):1951–3.
Martorell EA, Murray PM, Peterson JJ, Menke DM, Calamia KT. Palmar fasciitis and arthritis syndrome associated with metastatic ovarian carcinoma: A report of four cases. J Hand Surg Am. 2004;29(4):654–60.
Naschitz JE, Misselevich I, Rosner I, Yeshurun D, Weiner P, Amar M, et al. Lymph-node-based malignant lymphoma and reactive lymphadenopathy in eosinophilic fasciitis. Am J Med Sci. 1999;318(5):343–9.
Khanna D, Verity A, Grossman JM. Eosinophilic fasciitis with multiple myeloma: A new haematological association. Ann Rheum Dis. 2002;61(12):1111–2.
Philpott H, Hissaria P, Warrren L, Singhal N, Brown M, Proudman S, et al. Eosinophilic fasciitis as a paraneoplastic phenomenon associated with metastatic colorectal carcinoma. Australas J Dermatol. 2008;49(1):27–9.
Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after – what do we really know? report of 11 patients and review of the literature. Dermatology. 2006;213(2):93–101.
Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: Clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988;17(4):221–31.
Naschitz JE, Boss JH, Misselevich I, Yeshurun D, Rosner I. The fasciitis-panniculitis syndromes. clinical and pathologic features. Medicine (Baltimore). 1996;75(1):6–16.
Baumann F, Bruhlmann P, Andreisek G, Michel BA, Marincek B, Weishaupt D. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. 2005;184(1):169–74.
Cowper SE. Nephrogenic systemic fibrosis: An overview. J Am Coll Radiol. 2008;5(1):23–8.
Ting WW, Stone MS, Madison KC, Kurtz K. Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol. 2003;139(7):903–6.
Prchal D, Holmes DT, Levin A. Nephrogenic systemic fibrosis: The story unfolds. Kidney Int. 2008;73(12):1335–7.
Information on gadolinium-containing contrast agents [homepage on the Internet]. Center for Drug Evaluation and Research, United States Food and Drug Administration. Available from: http://www.fda.gov/cder/drug/InfoSheets/HCP/gcca_200705.htm.
Krishnan E, Furst DE. Systemic sclerosis mortality in the united states: 1979–1998. Eur J Epidemiol. 2005;20(10):855–61.
Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR, et al. Predicting mortality in systemic sclerosis: Analysis of a cohort of 309 french canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore). 2002;81(2):154–67.
Chung L, Krishnan E, Chakravarty EF. Hospitalizations and mortality in systemic sclerosis: Results from the nationwide inpatient sample. Rheumatology (Oxford). 2007;46(12):1808–13.
Simeon CP, Armadans L, Fonollosa V, Solans R, Selva A, Villar M, et al. Mortality and prognostic factors in spanish patients with systemic sclerosis. Rheumatology (Oxford). 2003;42(1):71–5.
Mayes MD, Lacey Jr JV, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48(8):2246–55.
Czirjak L, Kumanovics G, Varju C, Nagy Z, Pakozdi A, Szekanecz Z, et al. Survival and causes of death in 366 hungarian patients with systemic sclerosis. Ann Rheum Dis. 2008;67(1):59–63.
Steen VD. The many faces of scleroderma. Rheum Dis Clin North Am. 2008;34(1):1,15; v.
Steen VD, Medsger Jr TA. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43(11):2437–44.
Benan M, Hande I, Gul O. The natural course of progressive systemic sclerosis patients with interstitial lung involvement. Clin Rheumatol. 2007;26(3):349–54.
Steen VD, Medsger Jr TA. Improvement in skin thickening in systemic sclerosis associated with improved survival. Arthritis Rheum. 2001;44(12):2828–35.
Shah AA, Wigley FM, Hummers LK. Telangiectases in scleroderma: A potential clinical marker of pulmonary arterial hypertension. J Rheumatol. 2010;37(1):98–104.
Walker UA, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR scleroderma trials and research group database. Ann Rheum Dis. 2007;66(6):754–63.
Chang B, Wigley FM, White B, Wise RA. Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol. 2003;30(11):2398–405.
Trad S, Amoura Z, Beigelman C, Haroche J, Costedoat N, le Boutin TH, et al. Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease. Arthritis Rheum. 2006;54(1):184–91.
Clements PJ, Lachenbruch PA, Furst DE, Paulus HE, Sterz MG. Cardiac score. A semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis. Arthritis Rheum. 1991;34(11):1371–80.
Satoh M, Tokuhira M, Hama N, Hirakata M, Kuwana M, Akizuki M, et al. Massive pericardial effusion in scleroderma: A review of five cases. Br J Rheumatol. 1995;34(6):564–7.
Rankin AC. Arrhythmias in systemic sclerosis and related disorders. Card Electrophysiol Rev. 2002;6(1–2):152–4.
Follansbee WP, Zerbe TR, Medsger Jr TA. Cardiac and skeletal muscle disease in systemic sclerosis (scleroderma): A high risk association. Am Heart J. 1993;125(1):194–203.
Akram MR, Handler CE, Williams M, Carulli MT, Andron M, Black CM, et al. Angiographically proven coronary artery disease in scleroderma. Rheumatology (Oxford). 2006;45(11):1395–8.
Alexander EL, Firestein GS, Weiss JL, Heuser RR, Leitl G, Wagner Jr HN, et al. Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis. Ann Intern Med. 1986;105(5):661–8.
Systemic sclerosis. 2nd ed. In: Clements PJ and Furst DE, editors. Philadelphia, PA: Lippincott Williams & Wilkins; 2004.
Wigley F, Hummers LK. Management: holistic approach to systemic sclerosis. In: Clements PJ, Furst DE, editors. Systemic sclerosis. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2004.
Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655–66.
Rubin LJ. Treatment of pulmonary arterial hypertension due to scleroderma: challenges for the future. Rheum Dis Clin North Am. 2008;34(1):191,7; viii.
Nihtyanova SI, Denton CP. Current approaches to the management of early active diffuse scleroderma skin disease. Rheum Dis Clin North Am. 2008;34(1):161,79; viii.
Gelber AC, Wigley FM. Treatment of scleroderma. Curr Opin Rheumatol. 1995;7(6):551–9.
Roca RP, Wigley FM, White B. Depressive symptoms associated with scleroderma. Arthritis Rheum. 1996;39(6):1035–40.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2011 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Manno, R.L., Wigley, F.M. (2011). Scleroderma in the Elderly Population. In: Nakasato, Y., Yung, R. (eds) Geriatric Rheumatology. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-5792-4_28
Download citation
DOI: https://doi.org/10.1007/978-1-4419-5792-4_28
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4419-5791-7
Online ISBN: 978-1-4419-5792-4
eBook Packages: MedicineMedicine (R0)