Systemic sclerosis (scleroderma)

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.Full text  Abstract

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.Full text  Abstract

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.Full text  Abstract

American College of Rheumatology​. 2023 American College of Rheumatology (ACR) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease. Aug 2023 [internet publication].Full text

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. Abstract

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. Abstract

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.Full text  Abstract

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. Abstract

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. Abstract

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

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. Abstract

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. Abstract

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.Full text  Abstract

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. Abstract

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.Full text  Abstract

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. Abstract

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

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.Full text  Abstract

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. Abstract

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. Abstract

17. American College of Rheumatology. 2023 American College of Rheumatology (ACR) guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic disease​. Aug 2023 [internet publication].Full text

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

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

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

21. 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. Abstract

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

23. 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.Full text  Abstract

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

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.Full text  Abstract

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.Full text  Abstract

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 Oct;72(10):1696-9.Full text  Abstract

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.Full text  Abstract

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.Full text  Abstract

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.Full text  Abstract

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.Full text  Abstract

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. Abstract

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.Full text  Abstract

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. Abstract

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.Full text  Abstract

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.Full text  Abstract

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

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

39. Rusu A, Munteanu AC, Arbănași EM, et al. Overview of side-effects of antibacterial fluoroquinolones: new drugs versus old drugs, a step forward in the safety profile? Pharmaceutics. 2023 Mar 1;15(3):804.Full text  Abstract

40. American College of Rheumatology​. 2023 American College of Rheumatology (ACR) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease. Aug 2023 [internet publication].Full text

41. Raghu G, Montesi SB, Silver RM, et al. Treatment of systemic sclerosis-associated interstitial lung disease: evidence-based recommendations. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2024 Jan 15;209(2):137-52.Full text  Abstract

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

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.Full text  Abstract

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.Full text  Abstract

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. Abstract

46. 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. Abstract

47. 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. Abstract

48. 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.Full text  Abstract

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

50. 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.Full text  Abstract

51. 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.Full text  Abstract

52. 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.Full text  Abstract

53. 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. Abstract

54. 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. Abstract

55. 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. Abstract

56. 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. Abstract

57. 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. Abstract

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

59. 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.Full text  Abstract

60. 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. Abstract

61. 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.Full text  Abstract

62. 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. Abstract

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

64. 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.Full text  Abstract

65. Herrick A. Raynaud's phenomenon (secondary). Clinical Evid. 2008 Sep 26;2008:1125.Full text  Abstract

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

67. 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. Abstract

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

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

70. 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. Abstract

71. 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. Abstract

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

73. 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. Abstract