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 Sep-Oct;10(5):485-8. 摘要

Wigley FM. Clinical practice: Raynaud's phenomenon. N Eng J Med. 2002 Sep 26;347(13):1001-8. 摘要

Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006 Jun 22;354(25):2655-66.全文  摘要

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 Mar-Apr;28(2 Suppl 58):S42-6.全文  摘要

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 Mar;31(2 Suppl 76):157-65. 摘要

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 Sep;75(3):369-76. 摘要

Johnson SR, Brode SK, Mielniczuk LM, et al. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012 May;106(5):730-9. 摘要

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 Nov;72(11):1747-55.全文  摘要

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 Jan;42(1):60-3. 摘要

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 Aug;54(8):1454-8. 摘要

4. LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets, and pathogenesis. J Rheumatol. 1988 Feb;15(2):202-5. 摘要

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 Feb;37(4):223-35. 摘要

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 Jan;58(1):15-25. 摘要

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 Oct;52(10):3305-14.全文  摘要

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 Jul;44(7):1654-9. 摘要

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 Jun;44(6):1359-62.全文  摘要

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 Jan;53(1):74-80. 摘要

12. Nietert PJ, Silver RM. Systemic sclerosis: environmental and occupational risk factors. Curr Opin Rheumatol. 2000 Nov;12(6):520-6. 摘要

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 Jan 11;11:9.全文  摘要

15. Kallenberg CG. Early detection of connective tissue disease in patients with Raynaud's phenomenon. Rheum Dis Clin North Am. 1990 Feb;16(1):11-30. 摘要

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. 1990 Feb;16(1):11-30. 摘要

17. Leroy EC, Medsger TA. Raynaud's phenomenon: a proposal for classification. Clin Exp Rheumatol. 1992 Sep-Oct;10(5):485-8. 摘要

18. Wigley FM. Clinical practice: Raynaud's phenomenon. N Eng J Med. 2002 Sep 26;347(13):1001-8. 摘要

19. Turner R, Lipshutz W, Miller W, et al. Esophageal dysfunction in collagen vascular disease. Am J Med Sci. 1973 Mar;265(3):191-9. 摘要

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 Aug;28(4):281-6. 摘要

21. Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007 Aug;100(8):485-94.全文  摘要

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 Dec;42(12):2660-5.全文  摘要

23. Deswal A, Follansbee WP. Cardiac involvement in scleroderma. Rheum Dis Clin North Am. 1996 Nov;22(4):841-60. 摘要

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 Jun;50(6):1909-17. 摘要

25. Thompson AE, Shea B, Welch V, et al. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum. 2001;44(8):1841-7.全文  摘要

26. Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76(8):1327-39.全文  摘要

27. Roustit M, Blaise S, Allanore Y, et al. Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud's phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis. 2013;72:1696-9. 摘要

28. 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 Jun;75(6):1009-15.全文  摘要

29. 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 Jan;70(1):32-8.全文  摘要

30. Pope J, Fenlon D, Thompson A, et al. Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev. 2000 Apr 27;1998(2):CD000953.全文  摘要

31. Hughes M, Allanore Y, El Aoufy K, et al. A practical approach to the management of digital ulcers in patients with systemic sclerosis: a narrative review. JAMA Dermatol. 2021 Jul 1;157(7):851-8.全文  摘要

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 Jan-Feb;35(1):35-8. 摘要

33. Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006 Jun 22;354(25):2655-66.全文  摘要

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 Feb;36(2):323-9. 摘要

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 Jun 25;311(24):2490-8.全文  摘要

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 Mar-Apr;28(2 Suppl 58):S42-6.全文  摘要

37. European Medicines Agency. CMDh confirms recommendations on restricting use of domperidone-containing medicines. April 2014 [internet publication].全文

38. European Medicines Agency. European Medicines Agency recommends changes to the use of metoclopramide. July 2013 [internet publication].全文

39. Tzouvelekis A, Bonella F, Spagnolo P. Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag. 2015 Mar 3;11:359-70.全文  摘要

40. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 2019 Jun 27;380(26):2518-28.全文  摘要

41. Maher TM, Mayes MD, Kreuter M, et al. Effect of Nintedanib on lung function in patients with systemic sclerosis-associated interstitial lung disease: further analyses of a randomized, double-blind, placebo-controlled trial. Arthritis Rheumatol. 2021 Apr;73(4):671-6.全文  摘要

42. National Institute for Health and Care Excellence. Scleroderma: oral mycophenolate. July 2014 [internet publication]全文

43. Fernández-Codina A, Walker KM, Pope JE, et al. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheumatol. 2018 Nov;70(11):1820-8.全文  摘要

44. Omair MA, Alahmadi A, Johnson SR. Safety and effectiveness of mycophenolate in systemic sclerosis. A systematic review. PLoS One. 2015 May 1;10(5):e0124205.全文  摘要

45. Tashkin DP, Roth MD, Clements PJ, et al; Sclerodema Lung Study II Investigators. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016 Sep;4(9):708-19. 摘要

46. Barnes H, Holland AE, Westall GP, et al. Cyclophosphamide for connective tissue disease-associated interstitial lung disease. Cochrane Database Syst Rev. 2018 Jan 3;1(1):CD010908.全文  摘要

47. 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 Dec;54(12):3962-70.全文  摘要

48. 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 Dec;54(12):3954-61.全文  摘要

49. 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 Mar Apr;23(2):178-83. 摘要

50. 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 Nov;60(11):763-71. 摘要

51. 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 Sep;41(9):1613-9. 摘要

52. 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 Mar;31(2 Suppl 76):157-65. 摘要

53. 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 Sep;75(3):369-76. 摘要

54. Helfrich DJ, Banner B, Steen VD, et al. Normotensive renal failure in systemic sclerosis. Arthritis Rheum. 1989 Sep;32(9):1128-34. 摘要

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

56. 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 Jan;37(1):105-15. 摘要

57. 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 Jun 15;183(12):1723-9.全文  摘要

58. 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 Jun;67(6):808-14. 摘要

59. Grünig E. Treatment of pulmonary arterial hypertension in connective tissue disease. Drugs. 2012 May 28;72(8):1039-56. 摘要

60. Klinger JR, Elliott CG, Levine DJ, et al. Therapy for Pulmonary Arterial Hypertension in Adults: Update of the CHEST Guideline and Expert Panel Report. Chest. 2019 Mar;155(3):565-86.全文  摘要

61. Herrick A. Raynaud's phenomenon (secondary). Clinical Evid. 2008 Sep 26;2008:1125.全文  摘要

62. Roustit M, Blaise S, Allanore Y, et al. Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud's phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis. 2013 Oct;72(10):1696-9.全文  摘要

63. O'Donohue WJ. Home oxygen therapy. Clin Chest Med. 1997 Sep;18(3):535-45. 摘要

64. Johnson SR, Brode SK, Mielniczuk LM, et al. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012 May;106(5):730-9. 摘要

65. Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020 Oct;8(10):963-74.全文  摘要

66. ClinicalTrials.gov. KD025 in subjects with diffuse cutaneous systemic sclerosis. ClinicalTrials.gov Identifier: NCT03919799. Jan 2022 [internet publication].全文

67. ClinicalTrials.gov. Dedicated drug-drug interaction (DDI) study in healthy volunteers. ClinicalTrials.gov Identifier: NCT04939467. Oct 2021 [internet publication].全文

68. Daoussis D, Melissaropoulos K, Sakellaropoulos G, et al. A multicenter, open-label, comparative study of B-cell depletion therapy with Rituximab for systemic sclerosis-associated interstitial lung disease. Semin Arthritis Rheum. 2017 Apr;46(5):625-31.全文  摘要

69. Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003 Aug;48(8):2246-55. 摘要

70. 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 Sep;33(9):1915-6. 摘要

71. Kaide CG, Khandelwal S. Hyperbaric oxygen: applications in infectious disease. Emerg Med Clin North Am. 2008 May;26(2):571-95. 摘要

72. 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 Jul;37(7):485-94. 摘要