系统性硬化症（硬皮病）

Harris ED Jr, Budd RC, Firestein GS, et al. Kelley's textbook of rheumatology. 7th Ed. Elsevier Saunders; 2005.

Leroy EC, Medsger TA. Raynaud's phenomenon: a proposal for classification. Clin Exp Rheumatol 1992;10:485-488.

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Wigley FM. Clinical practice: Raynaud's phenomenon. N Eng J Med. 2002;347:1001-1008.

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Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655-2666.

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Baron M, Bernier P, Côté LF, et al. Screening and therapy for malnutrition and related gastro-intestinal disorders in systemic sclerosis: recommendations of a North American expert panel. Clin Exp Rheumatol. 2010;28(suppl 58):S42-S46.

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Iudici M, van der Goes MC, Valentini G, et al. Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review. Clin Exp Rheumatol. 2013;31(suppl 76):157-165.

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Medsger TA, Masi AT, Rodnan GP, et al. Survival with systemic sclerosis: a life-table analysis of clinical and demographic factors in 309 patients. Ann Intern Med. 1971;75:369-376.

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Johnson SR, Brode SK, Mielniczuk LM, et al. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012;106:730-739.

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1.  van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013;72:1747-1755.

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2.  Hoffmann-Vold AM, Gunnarsson R, Garen T, et al. Performance of the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for systemic sclerosis (SSc) in large, well-defined cohorts of SSc and mixed connective tissue disease. J Rheumatol. 2015;42:60-63.

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3.  Jordan S, Maurer B, Toniolo M, et al. Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice. Rheumatology (Oxford). 2015;54:1454-1458.

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4.  LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets, and pathogenesis. J Rheumatol. 1988;15:202-205.

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5.  Mayes MD, Reveille JD. Epidemiology, demographics, and genetics. In: Clements P, Furst D, ed. Systemic sclerosis, 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2004:89-93.

6.  Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008;37:223-235.

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7.  Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States: part I. Arthritis Rheum. 2008;58:15-25.

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8.  Zhou X, Tan FK, Xiong M, et al. Monozygotic twins clinically discordant for scleroderma show concordance for fibroblast gene expression profiles. Arthritis Rheum. 2005;52:3305-3314.

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9.  Kuwana M, Feghali CA, Medsger TA Jr, et al. Autoreactive T cells to topoisomerase I in monozygotic twins disconcordant for systemic sclerosis. Arthritis Rheum. 2001;44:1654-1659.

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10.  Arnett FC, Cho M, Chatterjee S, et al. Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis Rheum. 2001;44:1359-1362.

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11.  Tan FK, Stivers DN, Arnett FC, et al. HLA haplotypes and microsatellite polymorphisms in and around the major histocompatibility complex region in a Native American population with a high prevalence of scleroderma (systemic sclerosis). Tissue Antigens. 1999;53:74-80.

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12.  Nietert PJ, Silver RM. Systemic sclerosis: environmental and occupational risk factors. Curr Opin Rheumatol. 2000;12:520-526.

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13.  Harris ED Jr, Budd RC, Firestein GS, et al. Kelley's textbook of rheumatology. 7th Ed. Elsevier Saunders; 2005.

14.  Assassi S, Radstake TR, Mayes MD, et al. Genetics of scleroderma: implications for personalized medicine? BMC Med. 2013;11:9.

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15.  Kallenberg CG. Early detection of connective tissue disease in patients with Raynaud's phenomenon. Rheum Dis Clin North Am. 1990;16:11-30.

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16.  Kallenberg CG Wouda AA, Hoet MH, et al. Development of connective tissue disease in patients 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;47:634-641.

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17.  Leroy EC, Medsger TA. Raynaud's phenomenon: a proposal for classification. Clin Exp Rheumatol 1992;10:485-488.

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18.  Wigley FM. Clinical practice: Raynaud's phenomenon. N Eng J Med. 2002;347:1001-1008.

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19.  Turner R, Lipshutz W, Miller W, et al. Esophageal dysfunction in collagen vascular disease. Am J Med Sci. 1973;265:191-199.

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20.  Akesson A, Wollheim FA. Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. Br J Rheumatol. 1989;28:281-286.

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21.  Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100:485-494.

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22.  Bryan C, Knight C, Black CM, et al. Prediction of five-year survival following presentation with scleroderma: development of a simple model using three disease factors at first visit. Arthritis Rheum. 1999;42:2660-2665.

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23.  Deswal A, Follansbee WP. Cardiac involvement in scleroderma. Rheum Dis Clin North Am. 1996;22:841-860.

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24.  Clements PJ, Goldin JG, Kleerup EC, et al. Regional differences in bronchoalveolar lavage and thoracic high resolution computed tomography results in dyspneic patients with systemic sclerosis. Arthritis Rheum. 2004;50:1909-1917.

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25.  Thompson AE, Shea B, Welch V, et al. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum. 2001;44:1841-1847.

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26.  Herrick A. Raynaud's phenomenon (secondary). Clinical Evid. 2008;pii:1125. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2907943/ (last accessed 10 August 2016).

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27.  Brueckner CS, Becker MO, Kroencke T, et al. Effect of sildenafil on digital ulcers in systemic sclerosis: analysis from a single centre pilot study. Ann Rheum Dis. 2010;69:1475-1478.

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28.  Shenoy PD, Kumar S, Jha LK, et al. Efficacy of tadalafil in secondary Raynaud's phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatology (Oxford). 2010;49:2420-2428.

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29.  Hachulla E, Hatron PY, Carpentier P, et al. Efficacy of sildenafil on ischaemic digital ulcer healing in systemic sclerosis: the placebo-controlled SEDUCE study. Ann Rheum Dis. 2016;75:1009-1015.

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30.  Matucci-Cerinic M, Denton CP, Furst DE, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011;70:32-38.

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31.  Pope J, Fenlon D, Thompson A, et al. Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev. 2000;(2):CD000953.

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32.  Valentini G, Paone C, La Montagna G, et al. Low-dose intravenous cyclophosphamide in systemic sclerosis: an open prospective efficacy study in patients with early diffuse disease. Scand J Rheumatol. 2006;35:35-38.

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33.  Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655-2666.

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34.  Johnson SR, Feldman BM, Pope JE, et al. Shifting our thinking about uncommon disease trials: the case of methotrexate in scleroderma. J Rheumatol. 2009;36:323-329.

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35.  van Laar JM, Farge D, Sont JK, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA. 2014;311:2490-2498.

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36.  Baron M, Bernier P, Côté LF, et al. Screening and therapy for malnutrition and related gastro-intestinal disorders in systemic sclerosis: recommendations of a North American expert panel. Clin Exp Rheumatol. 2010;28(suppl 58):S42-S46.

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37.  European Medicines Agency. CMDh confirms recommendations on restricting use of domperidone-containing medicines. April 2014. http://www.ema.europa.eu (last accessed 10 August 2016).

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38.  European Medicines Agency. European Medicines Agency recommends changes to the use of metoclopramide. July 2013. http://www.ema.europa.eu (last accessed 10 August 2016).

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39.  Hoyles RK, Ellis RW, Wellsbury J, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 2006;54:3962-3970.

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40.  National Institute for Health and Care Excellence. Scleroderma: oral mycophenolate. July 2014. http://www.nice.org.uk (last accessed 10 August 2016).

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41.  Schachna L, Medsger TA Jr, Dauber JH, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2006;54:3954-3961.

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42.  Shitrit D, Amital A, Peled N, et al. Lung transplantation in patients with scleroderma: case series, review of the literature, and criteria for transplantation. Clin Transplant. 2009;23:178-183.

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43.  Beckett VL, Donadio JV Jr, Brennan LA Jr, et al. Use of captopril as early therapy for renal scleroderma: a prospective study. Mayo Clin Proc. 1985;60:763-771.

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44.  Steen VD, Medsger TA Jr. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41:1613-1619.

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45.  Iudici M, van der Goes MC, Valentini G, et al. Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review. Clin Exp Rheumatol. 2013;31(suppl 76):157-165.

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46.  Medsger TA, Masi AT, Rodnan GP, et al. Survival with systemic sclerosis: a life-table analysis of clinical and demographic factors in 309 patients. Ann Intern Med. 1971;75:369-376.

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47.  Helfrich DJ, Banner B, Steen VD, et al. Normotensive renal failure in systemic sclerosis. Arthritis Rheum. 1989;32:1128-1134.

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48.  Tamborrini G, Distler O. Update in pulmonary hypertension associated with connective tissue diseases: a systematic literature review. Dtsche Med Wochenschr. 2008;133(suppl 6):S199-S202. [in German]

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49.  Kowal-Bielecka O, Avouac J, Pittrow D, et al. Echocardiography as an outcome measure in scleroderma-related pulmonary arterial hypertension: a systematic literature analysis by the EPOSS group. J Rheumatol. 2010;37:105-115.

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50.  Jing ZC, Yu ZX, Shen JY, et al, Efficacy and safety of Vardenafil in the treatment of pulmonary arterial hypertension (EVALUATION) Study Group. Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med. 2011;183:1723-1729.

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51.  Avouac J, Wipff J, Kahan A, et al. Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials. Ann Rheum Dis. 2008;67:808-814.

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52.  Grünig E. Treatment of pulmonary arterial hypertension in connective tissue disease. Drugs. 2012;72:1039-1056.

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53.  Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014;146:449-475.

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54.  O'Donohue WJ. Home oxygen therapy. Clin Chest Med. 1997;18:535-545.

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55.  Johnson SR, Brode SK, Mielniczuk LM, et al. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012;106:730-739.

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56.  Tzouvelekis A, Bonella F, Spagnolo P. Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag. 2015;11:359-370.

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57.  Beretta L, Scorza R, Del Papa N, et al. Bosentan improves functional class, pulmonary artery systolic pressure, and DLCO in scleroderma patients with pulmonary hypertension: a possible synergy with iloprost. J Rheumatol. 2006;33:1915-1916.

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58.  Kaide CG, Khandelwal S. Hyperbaric oxygen: applications in infectious disease. Emerg Med Clin North Am. 2008;26:571-595.

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59.  Proudman SM, Stevens WM, Sahhar J, et al. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Intern Med J. 2007;37:485-494.

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