Limited cutaneous systemic sclerosis

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.Full text  Abstract

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

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

2. Steen VD, Wigley F, Hummers LK, et al. Systemic sclerosis. In: Hochberg MC, Silman AJ, Smolen JS, et al., eds. Rheumatology volume two. 3rd ed. Edinburgh: Mosby; 2003:1455-513.

3. Barnett AJ, Miller M, Littlejohn GO. The diagnosis and classification of scleroderma (systemic sclerosis). Postgrad Med J. 1988 Feb;64(748):121-5.Full text  Abstract

4. Winterbauer RH. Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutaneous calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp. 1964 Jun;114:361-83. Abstract

5. Rodnan GP, Jablonska S, Medsger TA. Classification and nomenclature of progressive systemic sclerosis. Clin Rheum Dis. 1979;5:5-13.

6. 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 Jan-Feb;22(3 suppl 33):S5-13. Abstract

7. 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 May;23(5):581-90.Full text  Abstract

8. Englert H, Roberts-Thomson PJ, Byth K, et al. Familial scleroderma: nature, nurture or both? Intern Med J. 2008 Apr;38(4):235-42. Abstract

9. 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

10. 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.Full text  Abstract

11. Bernatsky S, Joseph L, Pineau CA, et al. Scleroderma prevalence: demographic variations in a population-based sample. Arthritis Rheum. 2009 Mar 15;61(3):400-4Full text  Abstract

12. 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

13. Hoffmann-Vold AM, Midtvedt Ø, Molberg Ø, et al. Prevalence of systemic sclerosis in south-east Norway. Rheumatology (Oxford). 2012 Sep;51(9):1600-5.Full text  Abstract

14. Mayes MD. Epidemiologic studies of environmental agents and systemic autoimmune diseases. Environ Health Perspect. 1999 Oct;107(suppl 5):743-8.Full text  Abstract

15. Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med. 2009 May 7;360(19):1989-2003. Abstract

16. Benfaremo D, Svegliati S, Paolini C, et al. Systemic sclerosis: from pathophysiology to novel therapeutic approaches. Biomedicines. 2022 Jan 12;10(1):163.Full text  Abstract

17. Cutolo M, Soldano S, Smith V. Pathophysiology of systemic sclerosis: current understanding and new insights. Expert Rev Clin Immunol. 2019 Jul;15(7):753-64. Abstract

18. Matucci-Cerinic M, Steen V, Nash P, et al. The complexity of managing systemic sclerosis: screening and diagnosis. Rheumatology (Oxford). 2009 Jun;48(suppl 3):iii8-13.Full text  Abstract

19. Perelas A, Silver RM, Arrossi AV, et al. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020 Mar;8(3):304-20. Abstract

20. Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020 May 14;55(5):1902026.Full text  Abstract

21. Hachulla E, Agard C, Allanore Y, et al. French recommendations for the management of systemic sclerosis. Orphanet J Rare Dis. 2021 Jul 26;16(suppl 2):322.Full text  Abstract

22. Cavazzana I, Vojinovic T, Airo' P, et al. Systemic sclerosis-specific antibodies: novel and classical biomarkers. Clin Rev Allergy Immunol. 2022 Jun 18 [Epub ahead of print].Full text  Abstract

23. Zakopoulos NA, Kotsis VT, Gialafos EJ, et al. Systemic sclerosis is not associated with clinical or ambulatory blood pressure. Clin Exp Rheumatol. 2003 Mar-Apr;21(2):199-204. Abstract

24. Garner R, Kumari R, Lanyon P, et al. Prevalence, risk factors and associations of primary Raynaud's phenomenon: systematic review and meta-analysis of observational studies. BMJ Open. 2015 Mar 16;5(3):e006389.Full text  Abstract

25. Minier T, Guiducci S, Bellando-Randone S; EUSTAR co-workers. Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis. 2014 Dec;73(12):2087-93. Abstract

26. 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

27. Boin F, Wigley FM. Understanding, assessing and treating Raynaud's phenomenon. Curr Opin Rheumatol. 2005 Nov;17(6):752-60. Abstract

28. Dimitroulas T, Giannakoulas G, Karvounis H, et al. Natriuretic peptides in systemic sclerosis-related pulmonary arterial hypertension. Semin Arthritis Rheum. 2010 Feb;39(4):278-84. Abstract

29. Pope JE, Bellamy N, Seibold JR, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum. 2001 Jun;44(6):1351-8.Full text  Abstract

30. van den Hoogen FH, Boerbooms AM, Swaak AJ, et al. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol. 1996 Apr;35(4):364-72.Full text  Abstract

31. 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

32. 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

33. Walker KM, Pope J; participating members of the Scleroderma Clinical Trials Consortium (SCTC), Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails - a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012 Aug;42(1):42-55. Abstract

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

35. 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

36. Huisstede BM, Hoogvliet P, Paulis WD, et al. Effectiveness of interventions for secondary Raynaud's phenomenon: a systematic review. Arch Phys Med Rehabil. 2011 Jul;92(7):1166-80.Full text  Abstract

37. Wigley FM, Seibold JR, Wise RA, et al. Intravenous iloprost treatment of Raynaud's phenomenon and ischemic ulcers secondary to systemic sclerosis. J Rheumatol. 1992 Sep;19(9):1407-14. Abstract

38. Wigley FM, Wise RA, Seibold JR, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med. 1994 Feb 1;120(3):199-206. Abstract

39. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am. 2003 May;29(2):391-408. Abstract

40. Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. 2003 May;29(2):315-33. Abstract

41. Steen VD, DeMarco P. Complications in the use of angiotensin receptor blockers in the treatment of scleroderma renal crisis. (American College of Rheumatology 2001 Annual Scientific Meeting abstracts; abstract 2064.) Arthritis Rheum. 2001 Feb;44(suppl 9):S397.Full text

42. Teixeira L, Mahr A, Berezne A, et al. Scleroderma renal crisis, still a life-threatening complication. Ann N Y Acad Sci. 2007 Jun;1108:249-58. Abstract

43. 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

44. ClinicalTrials.gov. Mycophenolate in limited cutaneous systemic sclerosis (MINIMISE-Pilot) (MINIMISE). NCT04927390. 6 July 2022 [internet publication].Full text

45. Engel G, Rockson SG. Treprostinil for the treatment of severe digital necrosis in systemic sclerosis. Vasc Med. 2005 Feb;10(1):29-32. Abstract

46. Chung L, Fiorentino D. A pilot trial of treprostinil for the treatment and prevention of digital ulcers in patients with systemic sclerosis. J Am Acad Dermatol. 2006 May;54(5):880-2. Abstract

47. Shah AA, Schiopu E, Hummers LK, et al. Open label study of escalating doses of oral treprostinil diethanolamine in patients with systemic sclerosis and digital ischemia: pharmacokinetics and correlation with digital perfusion. Arthritis Res Ther. 2013 Apr 18;15(2):R54.Full text  Abstract

48. Abraham DJ, Shiwen X, Black CM, et al. Tumor necrosis factor alpha suppresses the induction of connective tissue growth factor by transforming growth factor-beta in normal and scleroderma fibroblasts. J Biol Chem. 2000 May 19;275(20):15220-5.Full text  Abstract

49. ClinicalTrials.gov. Safety and tolerability study of AVID200 in patients with diffuse cutaneous systemic sclerosis. NCT03831438. 23 October 2020 [internet publication].Full text

50. Lafyatis R, Spiera R, Domsic R, et al. THU0329 safety, target engagement, and initial efficacy of AVID200, a first-in-class potent and isoform-selective inhibitor of TGF-beta 1 and 3, in patients with diffuse cutaneous systemic sclerosis (DCSSC): a phase 1 dose escalation study. Annals of the Rheumatic Diseases 2020;79:394-5.Full text

51. McGonagle D, Tan AL, Madden J, et al. Successful treatment of resistant scleroderma-associated interstitial lung disease with rituximab. Rheumatology (Oxford). 2008 Apr;47(4):552-3.Full text  Abstract

52. Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010 Feb;49(2):271-80.Full text  Abstract

53. de Figueiredo Caldas MMV, de Azevedo KPM, de França Nunes AC, et al. Is rituximab effective for systemic sclerosis? A systematic review and meta-analysis. Adv Rheumatol. 2021 Feb 27;61(1):15. Abstract

54. Papadakis V, Karakasis D, Sfikakis PP, et al. Is imatinib mesylate a promising drug in scleroderma due to extensive chronic graft-versus-host disease? Leuk Lymphoma. 2009 Mar;50(3):471-4. Abstract

55. Soria A, Cario-André M, Lepreux S, et al. The effect of imatinib (Glivec®) on scleroderma and normal dermal fibroblasts: a preclinical study. Dermatology. 2008;216(2):109-17. Abstract

56. Chung L, Fiorentino DF, Benbarak MJ, et al. Molecular framework for response to imatinib mesylate in systemic sclerosis. Arthritis Rheum. 2009 Feb;60(2):584-91.Full text  Abstract

57. Pope J, McBain D, Petrlich L, et al. Imatinib in active diffuse cutaneous systemic sclerosis: results of a six-month, randomized, double-blind, placebo-controlled, proof-of-concept pilot study at a single center. Arthritis Rheum. 2011 Nov;63(11):3547-51.Full text  Abstract

58. Spiera RF, Gordon JK, Mersten JN, et al. Imatinib mesylate (Gleevec) in the treatment of diffuse cutaneous systemic sclerosis: results of a 1-year, phase IIa, single-arm, open-label clinical trial. Ann Rheum Dis. 2011 Jun;70(6):1003-9.Full text  Abstract

59. 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

60. American College of Rheumatology. 2022 American College of Rheumatology (ACR) guideline for vaccinations in patients with rheumatic and musculoskeletal diseases. Feb 2023 [internet publication].Full text

61. Vaidya PN, Basyal B, Finnigan NA. Scleroderma and renal crisis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan.Full text  Abstract

62. Desai CS, Lee DC, Shah SJ. Systemic sclerosis and the heart: current diagnosis and management. Curr Opin Rheumatol. 2011 Nov;23(6):545-54.Full text  Abstract

63. Parks JL, Taylor MH, Parks LP, et al. Systemic sclerosis and the heart. Rheum Dis Clin North Am. 2014 Feb;40(1):87-102. Abstract

64. Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010 Oct;69(10):1809-15.Full text  Abstract

65. 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

66. Khanna D, Denton CP, Jahreis A, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet. 2016 Jun 25;387(10038):2630-40. Abstract

67. Hirani N, Brunner NW, Kapasi A, et al. Canadian Cardiovascular Society/Canadian Thoracic Society position statement on pulmonary hypertension. Can J Cardiol. 2020 Jul;36(7):977-92.Full text  Abstract

68. Jinnin M, Ihn H, Yamane K, et al. Clinical features of patients with systemic sclerosis accompanied by rheumatoid arthritis. Clin Exp Rheumatol. 2003 Jan-Feb;21(1):91-4. Abstract

69. Horimoto AM, da Costa IP. Overlap between systemic sclerosis and rheumatoid arthritis: a distinct clinical entity? [in English, Portuguese]. Rev Bras Reumatol Engl Ed. 2016 Jul-Aug;56(4):287-98.Full text  Abstract

70. Canadian Scleroderma Research Group; Muangchan C, Baron M, Pope J. The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis. A systematic review. J Rheumatol. 2013 Sep;40(9):1545-56.Full text  Abstract

71. Jung M, Bonner A, Hudson M, et al. Myopathy is a poor prognostic feature in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG) cohort. Scand J Rheumatol. 2014;43(3):217-20. Abstract

72. Zhou M, Jiang L, Nie L, et al. Myopathy is a risk factor for poor prognosis of patients with systemic sclerosis: a retrospective cohort study. Medicine (Baltimore). 2020 Aug 14;99(33):e21734.Full text  Abstract

73. Pope JE. Scleroderma overlap syndromes. Curr Opin Rheumatol. 2002 Nov;14(6):704-10. Abstract