Scleroderma Questions & Answers

1. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013 Nov. 65(11):2737-47. [QxMD MEDLINE Link].

2. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A. 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. [QxMD MEDLINE Link].

3. RODNAN GP, BENEDEK TG. An historical account of the study of progressive systemic sclerosis (diffuse scleroderma). Ann Intern Med. 1962 Aug. 57:305-19. [QxMD MEDLINE Link].

4. David M. A case of scleroderma mentioned by Hippocrates in his aphorisms. Korot. 1981. 8(1-2):61-3. [QxMD MEDLINE Link].

5. Rodnan G, Benedeck T. History of scleroderma. Ann Intern Med. 1978. 89:725.

6. Goetz RH. Pathology of progressive systemic sclerosis (generalized scleroderma) with special reference to changes in the viscera. Clin Proc S Afr. 1945. 4:337-342.

7. Katsumoto TR, Whitfield ML, Connolly MK. The pathogenesis of systemic sclerosis. Annu Rev Pathol. 2011. 6:509-37. [QxMD MEDLINE Link].

8. Varga J, Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007 Mar. 117(3):557-67. [QxMD MEDLINE Link].

9. Matucci-Cerinic M, Kahaleh B, Wigley FM. Review: evidence that systemic sclerosis is a vascular disease. Arthritis Rheum. 2013 Aug. 65(8):1953-62. [QxMD MEDLINE Link].

10. Jimenez SA. Role of Endothelial to Mesenchymal Transition in the Pathogenesis of the Vascular Alterations in Systemic Sclerosis. ISRN Rheumatol. 2013. 2013:835948. [QxMD MEDLINE Link].

11. Varga J, Whitfield ML. Transforming growth factor-beta in systemic sclerosis (scleroderma). Front Biosci (Schol Ed). 2009. 1:226-35. [QxMD MEDLINE Link].

12. Gilbane AJ, Denton CP, Holmes AM. Scleroderma pathogenesis: a pivotal role for fibroblasts as effector cells. Arthritis Res Ther. 2013. 15(3):215. [QxMD MEDLINE Link].

13. Gu YS, Kong J, Cheema GS, Keen CL, Wick G, Gershwin ME. The immunobiology of systemic sclerosis. Semin Arthritis Rheum. 2008 Oct. 38(2):132-60. [QxMD MEDLINE Link].

14. Chizzolini C, Brembilla NC, Montanari E, Truchetet ME. Fibrosis and immune dysregulation in systemic sclerosis. Autoimmun Rev. 2011 Mar. 10(5):276-81. [QxMD MEDLINE Link].

15. Barsotti S, Stagnaro C, d'Ascanio A, Della Rossa A. One year in review 2016: systemic sclerosis. Clin Exp Rheumatol. 2016 Sep-Oct. 34 Suppl 100 (5):3-13. [QxMD MEDLINE Link]. [Full Text].

16. Dospinescu P, Jones GT, Basu N. Environmental risk factors in systemic sclerosis. Curr Opin Rheumatol. 2013 Mar. 25(2):179-83. [QxMD MEDLINE Link].

17. Nietert PJ, Sutherland SE, Silver RM, Pandey JP, Knapp RG, Hoel DG. Is occupational organic solvent exposure a risk factor for scleroderma?. Arthritis Rheum. 1998 Jun. 41(6):1111-8. [QxMD MEDLINE Link].

18. Marie I, Gehanno JF, Bubenheim M, Duval-Modeste AB, Joly P, Dominique S. Prospective study to evaluate the association between systemic sclerosis and occupational exposure and review of the literature. Autoimmun Rev. 2014 Feb. 13(2):151-6. [QxMD MEDLINE Link].

19. Rubio-Rivas M, Moreno R, Corbella X. Occupational and environmental scleroderma. Systematic review and meta-analysis. Clin Rheumatol. 2017 Mar. 36 (3):569-582. [QxMD MEDLINE Link].

20. Barragán-Martínez C, Speck-Hernández CA, Montoya-Ortiz G, Mantilla RD, Anaya JM, Rojas-Villarraga A. Organic solvents as risk factor for autoimmune diseases: a systematic review and meta-analysis. PLoS One. 2012. 7(12):e51506. [QxMD MEDLINE Link]. [Full Text].

21. Barbosa NS, Wetter DA, Wieland CN, Shenoy NK, Markovic SN, Thanarajasingam U. Scleroderma Induced by Pembrolizumab: A Case Series. Mayo Clin Proc. 2017 Jul. 92 (7):1158-1163. [QxMD MEDLINE Link].

22. Moroncini G, Mori S, Tonnini C, Gabrielli A. Role of viral infections in the etiopathogenesis of systemic sclerosis. Clin Exp Rheumatol. 2013 Mar-Apr. 31(2 Suppl 76):3-7. [QxMD MEDLINE Link].

23. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003 May. 29(2):239-54. [QxMD MEDLINE Link].

24. Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008 Feb. 37(4):223-35. [QxMD MEDLINE Link].

25. Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol. 2012 Mar. 24(2):165-70. [QxMD MEDLINE Link].

26. Ranque B, Mouthon L. Geoepidemiology of systemic sclerosis. Autoimmun Rev. 2010 Mar. 9(5):A311-8. [QxMD MEDLINE Link].

27. Calderon LM, Pope JE. Scleroderma epidemiology update. Curr Opin Rheumatol. 2021 Mar 1. 33 (2):122-127. [QxMD MEDLINE Link].

28. Silver RM, Bogatkevich G, Tourkina E, Nietert PJ, Hoffman S. Racial differences between blacks and whites with systemic sclerosis. Curr Opin Rheumatol. 2012 Nov. 24(6):642-8. [QxMD MEDLINE Link].

29. Reveille JD. Ethnicity and race and systemic sclerosis: how it affects susceptibility, severity, antibody genetics, and clinical manifestations. Curr Rheumatol Rep. 2003 Apr. 5(2):160-7. [QxMD MEDLINE Link].

30. Gelber AC, Manno RL, Shah AA, Woods A, Le EN, Boin F, et al. Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins Scleroderma Center and review of the literature. Medicine (Baltimore). 2013 Jul. 92(4):191-205. [QxMD MEDLINE Link].

31. Laing TJ, Gillespie BW, Toth MB, Mayes MD, Gallavan RH Jr, Burns CJ. Racial differences in scleroderma among women in Michigan. Arthritis Rheum. 1997 Apr. 40(4):734-42. [QxMD MEDLINE Link].

32. Arnett FC, Howard RF, Tan F, Moulds JM, Bias WB, Durban E, et al. Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA haplotype. Arthritis Rheum. 1996 Aug. 39(8):1362-70. [QxMD MEDLINE Link].

33. De Angelis R, et al; Systemic sclerosis PRogression INvestiGation group (SPRING). Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology. J Rheumatol. 2022 Feb. 49 (2):176-185. [QxMD MEDLINE Link].

34. Frerix M, Kröger K, Szalay G, Müller-Ladner U, Tarner IH. Is osteonecrosis of the lunate bone an underestimated feature of systemic sclerosis? A case series of nine patients and review of literature. Semin Arthritis Rheum. 2015 Aug 4. [QxMD MEDLINE Link].

35. Heřmánková B, Špiritović M, Šmucrová H, Oreská S, Štorkánová H, Komarc M, et al. Female Sexual Dysfunction and Pelvic Floor Muscle Function Associated with Systemic Sclerosis: A Cross-Sectional Study. Int J Environ Res Public Health. 2022 Jan 5. 19 (1):[QxMD MEDLINE Link]. [Full Text].

36. Barnett AJ, Miller MH, Littlejohn GO. A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol. 1988 Feb. 15(2):276-83. [QxMD MEDLINE Link].

37. Mayes MD, Lacey JV Jr, 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 Aug. 48(8):2246-55. [QxMD MEDLINE Link].

38. Elhai M, Meune C, Avouac J, Kahan A, Allanore Y. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2012 Jun. 51(6):1017-26. [QxMD MEDLINE Link].

39. Nikpour M, Baron M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014 Mar. 26(2):131-7. [QxMD MEDLINE Link].

40. Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CP. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010 Feb. 103(2):109-15. [QxMD MEDLINE Link].

41. Cottrell TR, Wise RA, Wigley FM, Boin F. The degree of skin involvement identifies distinct lung disease outcomes and survival in systemic sclerosis. Ann Rheum Dis. 2014 Jun 1. 73(6):1060-6. [QxMD MEDLINE Link].

42. Winstone TA, Assayag D, Wilcox PG, Dunne JV, Hague CJ, Leipsic J. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: A systematic review. Chest. 2014 Feb 27. [QxMD MEDLINE Link].

43. Chung L, Domsic RT, Lingala B, Alkassab F, Bolster M, Csuka ME, et al. Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res (Hoboken). 2014 Mar. 66(3):489-95. [QxMD MEDLINE Link].

44. Woodworth TG, Suliman YA, Li W, Furst DE, Clements P. Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov. 12 (11):678-691. [QxMD MEDLINE Link].

45. Jancin B. Be vigilant for scleroderma renal crisis. Rheumatology News. Available at https://www.mdedge.com/rheumatology/article/223925/lupus-connective-tissue-diseases/be-vigilant-scleroderma-renal-crisis. June 15, 2020; Accessed: February 3 2022.

46. Krieg T, Takehara K. Skin disease: a cardinal feature of systemic sclerosis. Rheumatology (Oxford). 2009 Jun. 48 Suppl 3:iii14-8. [QxMD MEDLINE Link].

47. Czirják L, Foeldvari I, Müller-Ladner U. Skin involvement in systemic sclerosis. Rheumatology (Oxford). 2008 Oct. 47 Suppl 5:v44-5. [QxMD MEDLINE Link].

48. Abignano G, Del Galdo F. Quantitating skin fibrosis: innovative strategies and their clinical implications. Curr Rheumatol Rep. 2014 Mar. 16(3):404. [QxMD MEDLINE Link].

49. Kahaleh MB. Raynaud phenomenon and the vascular disease in scleroderma. Curr Opin Rheumatol. 2004 Nov. 16(6):718-22. [QxMD MEDLINE Link].

50. Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol. 2012 Aug. 8(8):469-79. [QxMD MEDLINE Link].

51. Prete M, Fatone MC, Favoino E, Perosa F. Raynaud's phenomenon: from molecular pathogenesis to therapy. Autoimmun Rev. 2014 Jun. 13(6):655-67. [QxMD MEDLINE Link].

52. Ennis H, Vail A, Wragg E, Taylor A, Moore T, Murray A. A prospective study of systemic sclerosis-related digital ulcers: prevalence, location, and functional impact. Scand J Rheumatol. 2013. 42(6):483-6. [QxMD MEDLINE Link].

53. Guiducci S, Giacomelli R, Cerinic MM. Vascular complications of scleroderma. Autoimmun Rev. 2007 Sep. 6(8):520-3. [QxMD MEDLINE Link].

54. Hettema ME, Bootsma H, Kallenberg CG. Macrovascular disease and atherosclerosis in SSc. Rheumatology (Oxford). 2008 May. 47(5):578-83. [QxMD MEDLINE Link].

55. Chu SY, Chen YJ, Liu CJ, Tseng WC, Lin MW, Hwang CY. Increased risk of acute myocardial infarction in systemic sclerosis: a nationwide population-based study. Am J Med. 2013 Nov. 126(11):982-8. [QxMD MEDLINE Link].

56. Gyger G, Baron M. Gastrointestinal manifestations of scleroderma: recent progress in evaluation, pathogenesis, and management. Curr Rheumatol Rep. 2012 Feb. 14(1):22-9. [QxMD MEDLINE Link].

57. Christmann RB, Wells AU, Capelozzi VL, Silver RM. Gastroesophageal reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic, and treatment evidence. Semin Arthritis Rheum. 2010 Dec. 40(3):241-9. [QxMD MEDLINE Link].

58. Butt S, Emmanuel A. Systemic sclerosis and the gut. Expert Rev Gastroenterol Hepatol. 2013 May. 7(4):331-9. [QxMD MEDLINE Link].

59. Di Ciaula A, Covelli M, Berardino M, Wang DQ, Lapadula G, Palasciano G, et al. Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma. BMC Gastroenterol. 2008 Feb 27. 8:7. [QxMD MEDLINE Link]. [Full Text].

60. Hung EW, Mayes MD, Sharif R, Assassi S, Machicao VI, Hosing C. Gastric antral vascular ectasia and its clinical correlates in patients with early diffuse systemic sclerosis in the SCOT trial. J Rheumatol. 2013 Apr. 40(4):455-60. [QxMD MEDLINE Link].

61. Ghrénassia E, Avouac J, Khanna D, Derk CT, Distler O, Suliman YA, et al. Prevalence, correlates and outcomes of gastric antral vascular ectasia in systemic sclerosis: a EUSTAR case-control study. J Rheumatol. 2014 Jan. 41(1):99-105. [QxMD MEDLINE Link].

62. Steen VD. The lung in systemic sclerosis. J Clin Rheumatol. 2005 Feb. 11(1):40-6. [QxMD MEDLINE Link].

63. Wells AU, Margaritopoulos GA, Antoniou KM, Denton C. Interstitial lung disease in systemic sclerosis. Semin Respir Crit Care Med. 2014 Apr. 35(2):213-21. [QxMD MEDLINE Link].

64. Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. Chest. 2013 Oct. 144(4):1346-56. [QxMD MEDLINE Link].

65. Clements PJ, Allanore Y, Khanna D, Singh M, Furst DE. Arthritis in systemic sclerosis: systematic review of the literature and suggestions for the performance of future clinical trials in systemic sclerosis arthritis. Semin Arthritis Rheum. 2012 Jun. 41(6):801-14. [QxMD MEDLINE Link].

66. Avouac J, Clements PJ, Khanna D, Furst DE, Allanore Y. Articular involvement in systemic sclerosis. Rheumatology (Oxford). 2012 Aug. 51(8):1347-56. [QxMD MEDLINE Link].

67. Randone SB, Guiducci S, Cerinic MM. Musculoskeletal involvement in systemic sclerosis. Best Pract Res Clin Rheumatol. 2008 Apr. 22(2):339-50. [QxMD MEDLINE Link].

68. Stoenoiu MS, Houssiau FA, Lecouvet FE. Tendon friction rubs in systemic sclerosis: a possible explanation--an ultrasound and magnetic resonance imaging study. Rheumatology (Oxford). 2013 Mar. 52(3):529-33. [QxMD MEDLINE Link].

69. Doré A, Lucas M, Ivanco D, Medsger TA Jr, Domsic RT. Significance of palpable tendon friction rubs in early diffuse cutaneous systemic sclerosis. Arthritis Care Res (Hoboken). 2013 Aug. 65(8):1385-9. [QxMD MEDLINE Link]. [Full Text].

70. Steen V. The heart in systemic sclerosis. Curr Rheumatol Rep. 2004 Apr. 6(2):137-40. [QxMD MEDLINE Link].

71. Desai CS, Lee DC, Shah SJ. Systemic sclerosis and the heart: current diagnosis and management. Curr Opin Rheumatol. 2011 Nov. 23(6):545-54. [QxMD MEDLINE Link].

72. Meune C, Vignaux O, Kahan A, Allanore Y. Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis. 2010 Jan. 103(1):46-52. [QxMD MEDLINE Link].

73. Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. Rheumatology (Oxford). 2006 Oct. 45 Suppl 4:iv14-7. [QxMD MEDLINE Link].

74. Vacca A, Meune C, Gordon J, Chung L, Proudman S, Assassi S. Cardiac arrhythmias and conduction defects in systemic sclerosis. Rheumatology (Oxford). 2013 Nov 15. [QxMD MEDLINE Link].

75. Chu SY, Chen YJ, Liu CJ, Tseng WC, Lin MW, Hwang CY. Increased risk of acute myocardial infarction in systemic sclerosis: a nationwide population-based study. Am J Med. 2013 Nov. 126(11):982-8. [QxMD MEDLINE Link].

76. Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. 2003 May. 29(2):315-33. [QxMD MEDLINE Link].

77. Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007 Aug. 100(8):485-94. [QxMD MEDLINE Link].

78. Steen VD, Syzd A, Johnson JP, Greenberg A, Medsger TA Jr. Kidney disease other than renal crisis in patients with diffuse scleroderma. J Rheumatol. 2005 Apr. 32(4):649-55. [QxMD MEDLINE Link].

79. 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. [QxMD MEDLINE Link].

80. Lally EV, Jimenez SA. Impotence in progressively systemic sclerosis. Ann Intern Med. 1981 Aug. 95(2):150-3. [QxMD MEDLINE Link].

81. Foocharoen C, Tyndall A, Hachulla E, Rosato E, Allanore Y, Farge-Bancel D, et al. Erectile dysfunction is frequent in systemic sclerosis and associated with severe disease: a study of the EULAR Scleroderma Trial and Research group. Arthritis Res Ther. 2012 Feb 20. 14(1):R37. [QxMD MEDLINE Link]. [Full Text].

82. Minervini R, Morelli G, Minervini A, Pampaloni S, Tognetti A, Fiorentini L, et al. Bladder involvement in systemic sclerosis: urodynamic and histological evaluation in 23 patients. Eur Urol. 1998. 34(1):47-52. [QxMD MEDLINE Link].

83. Bhadauria S, Moser DK, Clements PJ, Singh RR, Lachenbruch PA, Pitkin RM. Genital tract abnormalities and female sexual function impairment in systemic sclerosis. Am J Obstet Gynecol. 1995 Feb. 172(2 Pt 1):580-7. [QxMD MEDLINE Link].

84. Kobak S, Oksel F, Aksu K, Kabasakal Y. The frequency of sicca symptoms and Sjögren's syndrome in patients with systemic sclerosis. Int J Rheum Dis. 2013 Feb. 16(1):88-92. [QxMD MEDLINE Link].

85. Albilia JB, Lam DK, Blanas N, Clokie CM, Sándor GK. Small mouths ... Big problems? A review of scleroderma and its oral health implications. J Can Dent Assoc. 2007 Nov. 73(9):831-6. [QxMD MEDLINE Link].

86. Derk CT, Rasheed M, Spiegel JR, Jimenez SA. Increased incidence of carcinoma of the tongue in patients with systemic sclerosis. J Rheumatol. 2005 Apr. 32(4):637-41. [QxMD MEDLINE Link].

87. Busquets J, Lee Y, Santamarina L, Federman JL, Abel A, Del Galdo F. Acute retinal artery occlusion in systemic sclerosis: a rare manifestation of systemic sclerosis fibroproliferative vasculopathy. Semin Arthritis Rheum. 2013 Oct. 43(2):204-8. [QxMD MEDLINE Link].

88. Antonelli A, Fallahi P, Ferrari SM, Mancusi C, Giuggioli D, Colaci M, et al. Incidence of thyroid disorders in systemic sclerosis: results from a longitudinal follow-up. J Clin Endocrinol Metab. 2013 Jul. 98(7):E1198-202. [QxMD MEDLINE Link].

89. Marasini B, Ferrari PA, Solaro N, Selmi C. Thyroid dysfunction in women with systemic sclerosis. Ann N Y Acad Sci. 2007 Jun. 1108:305-11. [QxMD MEDLINE Link].

90. Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum. 2013 Dec. 43(3):335-47. [QxMD MEDLINE Link].

91. Thombs BD, Taillefer SS, Hudson M, Baron M. Depression in patients with systemic sclerosis: a systematic review of the evidence. Arthritis Rheum. 2007 Aug 15. 57(6):1089-97. [QxMD MEDLINE Link].

92. Malcarne VL, Fox RS, Mills SD, Gholizadeh S. Psychosocial aspects of systemic sclerosis. Curr Opin Rheumatol. 2013 Nov. 25(6):707-13. [QxMD MEDLINE Link].

93. Nguyen C, Ranque B, Baubet T, Bérezné A, Mestre-Stanislas C, Rannou F, et al. Clinical, functional and health-related quality of life correlates of clinically significant symptoms of anxiety and depression in patients with systemic sclerosis: a cross-sectional survey. PLoS One. 2014. 9(2):e90484. [QxMD MEDLINE Link]. [Full Text].

94. Frech TM, Smith G, Reily M, Chamberlain J, Murtaugh MA, Penrod J. Peripheral neuropathy: a complication of systemic sclerosis. Clin Rheumatol. 2013 Jun. 32(6):885-8. [QxMD MEDLINE Link].

95. Hsu V, Varga J, Schlesinger N. Calcinosis in scleroderma made crystal clear. Curr Opin Rheumatol. 2019 Nov. 31 (6):589-594. [QxMD MEDLINE Link].

96. Tyndall A, Fistarol S. The differential diagnosis of systemic sclerosis. Curr Opin Rheumatol. 2013 Nov. 25(6):692-9. [QxMD MEDLINE Link].

97. Yaqub A, Chung L, Rieger KE, Fiorentino DF. Localized cutaneous fibrosing disorders. Rheum Dis Clin North Am. 2013 May. 39(2):347-64. [QxMD MEDLINE Link].

98. Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. Clin Rev Allergy Immunol. 2017 Dec. 53 (3):306-336. [QxMD MEDLINE Link].

99. Canady J, Karrer S, Fleck M, Bosserhoff AK. Fibrosing connective tissue disorders of the skin: molecular similarities and distinctions. J Dermatol Sci. 2013 Jun. 70(3):151-8. [QxMD MEDLINE Link].

100. Zuccaro G Jr. Esophagoscopy and endoscopic esophageal ultrasound in the assessment of esophageal function. Semin Thorac Cardiovasc Surg. 2001 Jul. 13(3):226-33. [QxMD MEDLINE Link].

101. Chapin R, Hant FN. Imaging of scleroderma. Rheum Dis Clin North Am. 2013 Aug. 39(3):515-46. [QxMD MEDLINE Link].

102. Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, et al. Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med. 2008 Jan 1. 177(1):91-8. [QxMD MEDLINE Link]. [Full Text].

103. Kowal-Bielecka O, Kowal K, Highland KB, Silver RM. Bronchoalveolar lavage fluid in scleroderma interstitial lung disease: technical aspects and clinical correlations: review of the literature. Semin Arthritis Rheum. 2010 Aug. 40(1):73-88. [QxMD MEDLINE Link].

104. van Bon L, Affandi AJ, Broen J, Christmann RB, Marijnissen RJ, Stawski L, et al. Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med. 2014 Jan 30. 370(5):433-43. [QxMD MEDLINE Link]. [Full Text].

105. Allanore Y, Meune C. N-terminal pro brain natriuretic peptide: the new cornerstone of cardiovascular assessment in systemic sclerosis. Clin Exp Rheumatol. 2009 May-Jun. 27(3 Suppl 54):59-63. [QxMD MEDLINE Link].

106. Chighizola C, Meroni PL, Schreiber BE, Coghlan JG, Denton CP, Ong VH. Role of N-terminal pro-brain natriuretic peptide in detecting clinically significant cardiac involvement in systemic sclerosis patients. Clin Exp Rheumatol. 2012 Mar-Apr. 30(2 Suppl 71):S81-5. [QxMD MEDLINE Link].

107. Thakkar V, Stevens W, Prior D, Youssef P, Liew D, Gabbay E, et al. The inclusion of N-terminal pro-brain natriuretic peptide in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension: a cohort study. Arthritis Res Ther. 2013. 15(6):R193. [QxMD MEDLINE Link]. [Full Text].

108. Hughes M, Lilleker JB, Herrick AL, Chinoy H. Cardiac troponin testing in idiopathic inflammatory myopathies and systemic sclerosis-spectrum disorders: biomarkers to distinguish between primary cardiac involvement and low-grade skeletal muscle disease activity. Ann Rheum Dis. 2015 May. 74 (5):795-8. [QxMD MEDLINE Link]. [Full Text].

109. Hsu VM, Moreyra AE, Wilson AC, Shinnar M, Shindler DM, Wilson JE, et al. Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right-heart catheterization. J Rheumatol. 2008 Mar. 35(3):458-65. [QxMD MEDLINE Link].

110. Avouac J, Huscher D, Furst DE, Opitz CF, Distler O, Allanore Y. Expert consensus for performing right heart catheterisation for suspected pulmonary arterial hypertension in systemic sclerosis: a Delphi consensus study with cluster analysis. Ann Rheum Dis. 2014 Jan. 73(1):191-7. [QxMD MEDLINE Link].

111. Freire V, Bazeli R, Elhai M, Campagna R, Pessis É, Avouac J. Hand and wrist involvement in systemic sclerosis: US features. Radiology. 2013 Dec. 269(3):824-30. [QxMD MEDLINE Link].

112. Boutry N, Hachulla E, Zanetti-Musielak C, Morel M, Demondion X, Cotten A. Imaging features of musculoskeletal involvement in systemic sclerosis. Eur Radiol. 2007 May. 17(5):1172-80. [QxMD MEDLINE Link].

113. Cutolo M, Sulli A, Smith V. How to perform and interpret capillaroscopy. Best Pract Res Clin Rheumatol. 2013 Apr. 27(2):237-48. [QxMD MEDLINE Link].

114. Sulli A, Ruaro B, Alessandri E, Pizzorni C, Cimmino MA, Zampogna G. Correlations between nailfold microangiopathy severity, finger dermal thickness and fingertip blood perfusion in systemic sclerosis patients. Ann Rheum Dis. 2014 Jan. 73(1):247-51. [QxMD MEDLINE Link].

115. Herrick AL, Cutolo M. Clinical implications from capillaroscopic analysis in patients with Raynaud's phenomenon and systemic sclerosis. Arthritis Rheum. 2010 Sep. 62(9):2595-604. [QxMD MEDLINE Link].

116. Smith V, Riccieri V, Pizzorni C, Decuman S, Deschepper E, Bonroy C. Nailfold capillaroscopy for prediction of novel future severe organ involvement in systemic sclerosis. J Rheumatol. 2013 Dec. 40(12):2023-8. [QxMD MEDLINE Link].

117. Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum. 2005 Aug. 35(1):35-42. [QxMD MEDLINE Link].

118. Mehra S, Walker J, Patterson K, Fritzler MJ. Autoantibodies in systemic sclerosis. Autoimmun Rev. 2013 Jan. 12(3):340-54. [QxMD MEDLINE Link].

119. Perera A, Fertig N, Lucas M, Rodriguez-Reyna TS, Hu P, Steen VD. Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti-topoisomerase I antibody. Arthritis Rheum. 2007 Aug. 56(8):2740-6. [QxMD MEDLINE Link].

120. D'Aoust J, Hudson M, Tatibouet S, Wick J, Mahler M, Baron M, et al. Clinical and Serologic Correlates of Anti-PM/Scl Antibodies in Systemic Sclerosis: A Multicenter Study of 763 Patients. Arthritis Rheumatol. 2014 Jun. 66(6):1608-15. [QxMD MEDLINE Link].

121. Pignone A, Matucci-Cerinic M, Lombardi A, Fedi R, Fargnoli R, De Dominicis R. High resolution computed tomography in systemic sclerosis. Real diagnostic utilities in the assessment of pulmonary involvement and comparison with other modalities of lung investigation. Clin Rheumatol. 1992 Dec. 11(4):465-72. [QxMD MEDLINE Link].

122. Khanna D, Tseng CH, Farmani N, Steen V, Furst DE, Clements PJ. Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum. 2011 Oct. 63(10):3078-85. [QxMD MEDLINE Link].

123. Hudson M, Assayag D, Caron M, Fox BD, Hirsch A, Steele R. Comparison of different measures of diffusing capacity for carbon monoxide (DLCO) in systemic sclerosis. Clin Rheumatol. 2013 Oct. 32(10):1467-74. [QxMD MEDLINE Link].

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Abhishek Nandan, MD Associate Professor of Medicine, Associate Program Director for Rheumatology Fellowship, Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Virginia Commonwealth University School of Medicine; Staff Physician, Richmond VA Medical Center, Central Virginia Veterans Health Care System

Abhishek Nandan, MD is a member of the following medical societies: American College of Rheumatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Lewis Katz School of Medicine at Temple University

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

John Varga, MD Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University

John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical and Translational Research, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon) Professor and Co-Director, Jefferson Institute of Molecular Medicine, Director, Connective Tissue Diseases Section, Director, The Scleroderma Center, Sidney Kimmel Medical College of Thomas Jefferson University

Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon) is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Society for Biochemistry and Molecular Biology, Royal College of Physicians, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Fabian A Mendoza-Ballesteros, MD, FACR Assistant Professor of Medicine, Division of Rheumatology, Jefferson Institute of Molecular Medicine; Associate Director of The Scleroderma Center, Director of Scleroderma Program, Sidney Kimmel Medical College of Thomas Jefferson University

Fabian A Mendoza-Ballesteros, MD, FACR is a member of the following medical societies: American College of Rheumatology

Disclosure: Nothing to disclose.

Patrick M Cronin, DO, FACR Clinical Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pennsylvania Health System, Pennsylvania Hospital

Patrick M Cronin, DO, FACR is a member of the following medical societies: American College of Rheumatology, American Osteopathic Association, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Andrew S Koenig, DO Consulting Staff, Division of Rheumatology, Rancoccas Hospital

Andrew S Koenig, DO is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Osteopathic Association

Disclosure: Nothing to disclose.

Marie Spevak O'Brien, DO Assistant Clinical Professor of Medicine, Arthritis and Rheumatology, Lehigh Valley Physician Group

Marie Spevak O'Brien, DO is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association, American Osteopathic Association, International Society for Clinical Densitometry, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Acknowledgments

The assistance of Kenneth Brown in the preparation of this manuscript is gratefully acknowledged.

Sergio A Jimenez, MD, was recipient of NIH/NIAMS grant #RO-1 AR19616.

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