Reference for Chapter 56: Systemic sclerosis (systemic scleroderma)

1 Varga J. Systemic sclerosis: an update. BullHosp JT Dis 2008;66(3):198–202.
2 Sticherling M. Systemic sclerosis – focus on dermatological aspects. Part 2: diagnostics, therapy. J Deut Dermatol Gesell 2012;10(11):783–91.
3 Sticherling M. Systemic sclerosis–dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J Deut Dermatol Gesell 2012;10(10):705–18; quiz 16.
4 LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988;15(2):202–5. Pubmed link
5 LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001;28(7):1573–6. Pubmed link
6 Bennett RM. Scleroderma overlap syndromes. Rheum Dis Clin North Am 1990;16(1):185–98. Pubmed link
7 Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP. Clinical and serological hallmarks of systemic sclerosis overlap syndromes. J Rheumatol 2011;38(11):2406–9. Cross Ref link Pubmed link
8 Poormoghim H, Lucas M, Fertig N, Medsger TA, Jr. Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty‐eight patients. Arthritis Rheum 2000;43(2):444–51. Cross Ref link Pubmed link
9 Diab S, Dostrovsky N, Hudson M, et al. Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects. J Rheumatol 2014;41(11):2179–85. Cross Ref link Pubmed link
10 Simeon‐Aznar CP, Tolosa‐Vilella C, Gabarro‐Julia L, et al. Systemic sclerosis sine scleroderma and limited cutaneous systemic sclerosis: similarities and differences. Clin Exp Rheumatol 2014;32(6 Suppl. 86):S33–40. Pubmed link
11 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. Arthritis Rheum 2013;65(11):2737–47. Cross Ref link Pubmed link
12 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;23(5):581–90. Cross Ref link Pubmed link
13 Hoffmann‐Vold AM, Gunnarsson R, Garen T, Midtvedt O, Molberg O. 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(1):60–3. Cross Ref link Pubmed link
14 Walker JG, Fritzler MJ. Update on autoantibodies in systemic sclerosis. Curr Opin Rheumatol 2007;19(6):580–91. Cross Ref link Pubmed link
15 Bunn CC, Black CM. Systemic sclerosis: an autoantibody mosaic. Clin Exp Immunol 1999;117(2):207–8. Cross Ref link Pubmed link
16 Walker UA, Tyndall A, Czirjak L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research Group database. Ann Rheum Dis 2007;66(6):754–63. Cross Ref link Pubmed link
17 Steen VD, Powell DL, Medsger TA, Jr. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum 1988;31(2):196–203. Cross Ref link Pubmed link
18 Nihtyanova SI, Denton CP. Autoantibodies as predictive tools in systemic sclerosis. Nature Rev Rheumatol 2010;6(2):112–16. Cross Ref link
19 Milano A, Pendergrass SA, Sargent JL, et al. Molecular subsets in the gene expression signatures of scleroderma skin. PLOS One 2008;3(7):e2696. Cross Ref link Pubmed link
20 Sargent JL, Milano A, Bhattacharyya S, et al. A TGFbeta‐responsive gene signature is associated with a subset of diffuse scleroderma with increased disease severity. J Invest Dermatol 2010;130(3):694–705. Cross Ref link Pubmed link
21 Hinchcliff M, Huang CC, Wood TA, et al. Molecular signatures in skin associated with clinical improvement during mycophenolate treatment in systemic sclerosis. J Invest Dermatol 2013;133(8):1979–89. Cross Ref link Pubmed link
22 Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 2008;37(4):223–35. Cross Ref link Pubmed link
23 Steen VD, Medsger TA, Jr. Epidemiology and natural history of systemic sclerosis. Rheum Dis Clin North Am 1990;16(1):1–10. Pubmed link
24 Silman AJ, Newman J. Epidemiology of systemic sclerosis. Curr Opin Rheumatol 1996;8(6):585–9. Cross Ref link Pubmed link
25 Walker UA, Tyndall A, Czirjak L, et al. Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database. Ann Rheum Dis 2009;68(6):856–62. Cross Ref link Pubmed link
26 Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol 2012;24(2):165–70. Cross Ref link Pubmed link
27 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;48(8):2246–55. Cross Ref link Pubmed link
28 Reveille JD. Ethnicity and race and systemic sclerosis: how it affects susceptibility, severity, antibody genetics, and clinical manifestations. Curr Rheumatol Rep 2003;5(2):160–7. Cross Ref link Pubmed link
29 Harley JB, Neas BR. Oklahoma Choctaw and systemic sclerosis: the founder effect and genetic susceptibility. Arthritis Rheum 1998;41(10):1725–8. Cross Ref link Pubmed link
30 Silman AJ, Howard Y, Hicklin AJ, Black C. Geographical clustering of scleroderma in south and west London. Br J Rheumatol 1990;29(2):93–6. Pubmed link
31 Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res 2010;62(2):213–18. Cross Ref link
32 Silman AJ. Epidemiology of scleroderma. Ann Rheum Dis 1991;50(Suppl. 4):846–53. Cross Ref link Pubmed link
33 Medsger TA, Jr. Epidemiology of systemic sclerosis. Clin Dermatol 1994;12(2):207–16. Cross Ref link Pubmed link
34 Alba MA, Velasco C, Simeon CP, et al. Early‐ versus late‐onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients. Medicine 2014;93(2):73–81. Cross Ref link Pubmed link
35 Ohta A, Nagai M, Nishina M, Tomimitsu H, Kohsaka H. Age at onset and gender distribution of systemic lupus erythematosus, polymyositis/dermatomyositis, and systemic sclerosis in Japan. Mod Rheumatol 2013;23(4):759–64. Cross Ref link Pubmed link
36 Strickland G, Pauling J, Cavill C, Shaddick G, McHugh N. Mortality in systemic sclerosis – a single centre study from the UK. Clin Rheumatol 2013;32(10):1533–9. Cross Ref link Pubmed link
37 Hugle T, Schuetz P, Daikeler T, et al. Late‐onset systemic sclerosis – a systematic survey of the EULAR scleroderma trials and research group database. Rheumatology 2011;50(1):161–5. Cross Ref link Pubmed link
38 Zulian F, Cuffaro G, Sperotto F. Scleroderma in children: an update. Curr Opin Rheumatol 2013;25(5):643–50. Cross Ref link Pubmed link
39 Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003;29(2):239–54. Cross Ref link Pubmed link
40 Oliver JE, Silman AJ. Why are women predisposed to autoimmune rheumatic diseases? Arthritis Res Ther 2009;11(5):252. Cross Ref link Pubmed link
41 Soldano S, Montagna P, Villaggio B, et al. Endothelin and sex hormones modulate the fibronectin synthesis by cultured human skin scleroderma fibroblasts. Ann Rheum Dis 2009;68(4):599–602. Cross Ref link Pubmed link
42 Soldano S, Montagna P, Brizzolara R, et al. Effects of estrogens on extracellular matrix synthesis in cultures of human normal and scleroderma skin fibroblasts. Ann NY Acad Sci 2010;1193:25–9. Cross Ref link Pubmed link
43 Hussein H, Lee P, Chau C, Johnson SR. The effect of male sex on survival in systemic sclerosis. J Rheumatol 2014;41(11):2193–200. Cross Ref link Pubmed link
44 Nashid M, Khanna PP, Furst DE, et al. Gender and ethnicity differences in patients with diffuse systemic sclerosis – analysis from three large randomized clinical trials. Rheumatology 2011;50(2):335–42. Cross Ref link Pubmed link
45 Meyer OC, Fertig N, Lucas M, Somogyi N, Medsger TA, Jr. Disease subsets, antinuclear antibody profile, and clinical features in 127 French and 247 US adult patients with systemic sclerosis. J Rheumatol 2007;34(1):104–9. Pubmed link
46 McNearney TA, Hunnicutt SE, Fischbach M, et al. Perceived functioning has ethnic‐specific associations in systemic sclerosis: another dimension of personalized medicine. J Rheumatol 2009;36(12):2724–32. Cross Ref link Pubmed link
47 Gelber AC, Manno RL, Shah AA, 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 2013;92(4):191–205. Cross Ref link Pubmed link
48 Reveille JD, Fischbach M, McNearney T, et al. Systemic sclerosis in 3 US ethnic groups: a comparison of clinical, sociodemographic, serologic, and immunogenetic determinants. Semin Arthritis Rheum 2001;30(5):332–46. Cross Ref link Pubmed link
49 Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 2005;35(1):35–42. Cross Ref link Pubmed link
50 Krzyszczak ME, Li Y, Ross SJ, et al. Gender and ethnicity differences in the prevalence of scleroderma‐related autoantibodies. Clin Rheumatol 2011;30(10):1333–9. Cross Ref link Pubmed link
51 Laing TJ, Gillespie BW, Toth MB, et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum 1997;40(4):734–42. Cross Ref link Pubmed link
52 Low AH, Johnson SR, Lee P. Ethnic influence on disease manifestations and autoantibodies in Chinese‐descent patients with systemic sclerosis. J Rheumatol 2009;36(4):787–93. Cross Ref link Pubmed link
53 Arnett FC, Gourh P, Shete S, et al. Major histocompatibility complex (MHC) class II alleles, haplotypes and epitopes which confer susceptibility or protection in systemic sclerosis: analyses in 1300 Caucasian, African‐American and Hispanic cases and 1000 controls. Ann Rheum Dis 2010;69(5):822–7. Cross Ref link Pubmed link
54 Kuwana M, Kaburaki J, Arnett FC, Howard RF, Medsger TA, Jr, Wright TM. Influence of ethnic background on clinical and serologic features in patients with systemic sclerosis and anti‐DNA topoisomerase I antibody. Arthritis Rheum 1999;42(3):465–74. Cross Ref link Pubmed link
55 Tan FK, Arnett FC, Reveille JD, et al. Autoantibodies to fibrillin 1 in systemic sclerosis: ethnic differences in antigen recognition and lack of correlation with specific clinical features or HLA alleles. Arthritis Rheum 2000;43(11):2464–71. Cross Ref link Pubmed link
56 Avouac J, Airo P, Dieude P, et al. Associated autoimmune diseases in systemic sclerosis define a subset of patients with milder disease: results from 2 large cohorts of European Caucasian patients. J Rheumatol 2010;37(3):608–14. Cross Ref link Pubmed link
57 Caramaschi P, Biasi D, Volpe A, Carletto A, Cecchetto M, Bambara LM. Coexistence of systemic sclerosis with other autoimmune diseases. Rheumatol Int 2007;27(4):407–10. Cross Ref link Pubmed link
58 Hudson M, Rojas‐Villarraga A, Coral‐Alvarado P, et al. Polyautoimmunity and familial autoimmunity in systemic sclerosis. J Autoimmun 2008;31(2):156–9. Cross Ref link Pubmed link
59 Cappelli S, Bellando Randone S, Martinovic D, et al. ‘To be or not to be,’ ten years after: evidence for mixed connective tissue disease as a distinct entity. Semin Arthritis Rheum 2012;41(4):589–98. Cross Ref link Pubmed link
60 Tani C, Carli L, Vagnani S, et al. The diagnosis and classification of mixed connective tissue disease. J Autoimmun 2014;48/49:46–9. Cross Ref link
61 Hajas A, Szodoray P, Nakken B, et al. Clinical course, prognosis, and causes of death in mixed connective tissue disease. J Rheumatol 2013;40(7):1134–42. Cross Ref link Pubmed link
62 Moinzadeh P, Aberer E, Ahmadi‐Simab K, et al. Disease progression in systemic sclerosis‐overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Ann Rheum Dis 2015;74(4):730–7. Cross Ref link Pubmed link
63 Szodoray P, Hajas A, Kardos L, et al. Distinct phenotypes in mixed connective tissue disease: subgroups and survival. Lupus 2012;21(13):1412–22. Cross Ref link Pubmed link
64 Wooten M. Systemic sclerosis and malignancy: a review of the literature. South Med J 2008;101(1):59–62. Cross Ref link Pubmed link
65 Onishi A, Sugiyama D, Kumagai S, Morinobu A. Cancer incidence in systemic sclerosis: meta‐analysis of population‐based cohort studies. Arthritis Rheum 2013;65(7):1913–21. Cross Ref link Pubmed link
66 Zhang JQ, Wan YN, Peng WJ, et al. The risk of cancer development in systemic sclerosis: a meta‐analysis. Cancer Epidemiol 2013;37(5):523–7. Cross Ref link Pubmed link
67 Bonifazi M, Tramacere I, Pomponio G, et al. Systemic sclerosis (scleroderma) and cancer risk: systematic review and meta‐analysis of observational studies. Rheumatology 2013;52(1):143–54. Cross Ref link Pubmed link
68 Derk CT, Rasheed M, Artlett CM, Jimenez SA. A cohort study of cancer incidence in systemic sclerosis. J Rheumatol 2006;33(6):1113–16. Pubmed link
69 Moinzadeh P, Fonseca C, Hellmich M, et al. Association of anti‐RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 2014;16(1):R53. Cross Ref link Pubmed link
70 Olesen AB, Svaerke C, Farkas DK, Sorensen HT. Systemic sclerosis and the risk of cancer: a nationwide population‐based cohort study. Br J Dermatol 2010;163(4):800–6. Cross Ref link Pubmed link
71 Higuchi M, Horiuchi T, Ishibashi N, Yoshizawa S, Niho Y, Nagasawa K. Anticentromere antibody as a risk factor for cancer in patients with systemic sclerosis. Clin Rheumatol 2000;19(2):123–6. Cross Ref link Pubmed link
72 Siau K, Laversuch CJ, Creamer P, O'Rourke KP. Malignancy in scleroderma patients from south west England: a population‐based cohort study. Rheumatol Int 2011;31(5):641–5. Cross Ref link Pubmed link
73 Derk CT, Sakkas LI, Rasheed M, Artlett C, Jimenez SA. Autoantibodies in patients with systemic sclerosis and cancer: a case–control study. J Rheumatol 2003;30(9):1994–6. Pubmed link
74 Airo P, Ceribelli A, Cavazzana I, Taraborelli M, Zingarelli S, Franceschini F. Malignancies in Italian patients with systemic sclerosis positive for anti‐RNA polymerase III antibodies. J Rheumatol 2011;38(7):1329–34. Cross Ref link Pubmed link
75 Shah AA, Rosen A, Hummers L, Wigley F, Casciola‐Rosen L. Close temporal relationship between onset of cancer and scleroderma in patients with RNA polymerase I/III antibodies. Arthritis Rheum 2010;62(9):2787–95. Cross Ref link Pubmed link
76 Prescott RJ, Freemont AJ, Jones CJ, Hoyland J, Fielding P. Sequential dermal microvascular and perivascular changes in the development of scleroderma. J Pathol 1992;166(3):255–63. Cross Ref link Pubmed link
77 Fleischmajer R, Perlish JS, Shaw KV, Pirozzi DJ. Skin capillary changes in early systemic scleroderma. Electron microscopy and ‘in vitro’ autoradiography with tritiated thymidine. Arch Dermatol 1976;112(11):1553–7. Cross Ref link Pubmed link
78 Claman HN, Giorno RC, Seibold JR. Endothelial and fibroblastic activation in scleroderma. The myth of the ‘uninvolved skin’. Arthritis Rheum 1991;34(12):1495–501. Cross Ref link Pubmed link
79 Kraling BM, Maul GG, Jimenez SA. Mononuclear cellular infiltrates in clinically involved skin from patients with systemic sclerosis of recent onset predominantly consist of monocytes/macrophages. Pathobiology 1995;63(1):48–56. Cross Ref link Pubmed link
80 Fleischmajer R, Perlish JS, Reeves JR. Cellular infiltrates in scleroderma skin. Arthritis Rheum 1977;20(4):975–84. Cross Ref link Pubmed link
81 Fleischmajer R, Perlish JS, West WP. Ultrastructure of cutaneous cellular infiltrates in scleroderma. Arch Dermatol 1977;113(12):1661–6. Cross Ref link Pubmed link
82 Denton CP, Ong VH. Targeted therapies for systemic sclerosis. Nature Rev Rheumatol 2013;9(8):451–64. Cross Ref link
83 Akimoto S, Ishikawa O, Igarashi Y, Kurosawa M, Miyachi Y. Dermal mast cells in scleroderma: their skin density, tryptase/chymase phenotypes and degranulation. Br J Dermatol 1998;138(3):399–406. Cross Ref link Pubmed link
84 Yukawa S, Yamaoka K, Sawamukai N, et al. Dermal mast cell density in fingers reflects severity of skin sclerosis in systemic sclerosis. Mod Rheumatol 2013;23(6):1151–7. Cross Ref link Pubmed link
85 Hugle T, Hogan V, White KE, van Laar JM. Mast cells are a source of transforming growth factor beta in systemic sclerosis. Arthritis Rheum 2011;63(3):795–9. Cross Ref link Pubmed link
86 Falanga V, Soter NA, Altman RD, Kerdel FA. Elevated plasma histamine levels in systemic sclerosis (scleroderma). Arch Dermatol 1990;126(3):336–8. Cross Ref link Pubmed link
87 Seibold JR, Giorno RC, Claman HN. Dermal mast cell degranulation in systemic sclerosis. Arthritis Rheum 1990;33(11):1702–9. Cross Ref link Pubmed link
88 Frech T, De Domenico I, Murtaugh MA, et al. Autophagy is a key feature in the pathogenesis of systemic sclerosis. Rheumatol Int 2014;34(3):435–9. Cross Ref link Pubmed link
89 Aden N, Shiwen X, Aden D, et al. Proteomic analysis of scleroderma lesional skin reveals activated wound healing phenotype of epidermal cell layer. Rheumatology 2008;47(12):1754–60. Cross Ref link Pubmed link
90 Aden N, Nuttall A, Shiwen X, et al. Epithelial cells promote fibroblast activation via IL‐1alpha in systemic sclerosis. J Invest Dermatol 2010;130(9):2191–200. Cross Ref link Pubmed link
91 Denton CP, Xu S, Black CM, Pearson JD. Scleroderma fibroblasts show increased responsiveness to endothelial cell‐derived IL‐1 and bFGF. J Invest Dermatol 1997;108(3):269–74. Cross Ref link Pubmed link
92 Kirk TZ, Mayes MD. IL‐1 rescues scleroderma myofibroblasts from serum‐starvation‐induced cell death. Biochem Biophys Res Commun 1999;255(1):129–32. Cross Ref link Pubmed link
93 Daoussis D, Liossis SN. B cells tell scleroderma fibroblasts to produce collagen. Arthritis Res Ther 2013;15(6):125. Cross Ref link Pubmed link
94 Lafyatis R, York M. Innate immunity and inflammation in systemic sclerosis. Curr Opin Rheumatol 2009;21(6):617–22. Cross Ref link Pubmed link
95 Artlett CM, Sassi‐Gaha S, Rieger JL, Boesteanu AC, Feghali‐Bostwick CA, Katsikis PD. The inflammasome activating caspase 1 mediates fibrosis and myofibroblast differentiation in systemic sclerosis. Arthritis Rheum 2011;63(11):3563–74. Cross Ref link Pubmed link
96 O'Reilly S. Innate immunity in systemic sclerosis pathogenesis. Clin Sci 2014;126(5):329–37. Cross Ref link Pubmed link
97 Fleischmajer R, Gay S, Meigel WN, Perlish JS. Collagen in the cellular and fibrotic stages of scleroderma. Arthritis Rheum 1978;21(4):418–28. Cross Ref link Pubmed link
98 Fleischmajer R, Gay S, Perlish JS, Cesarini JP. Immunoelectron microscopy of type III collagen in normal and scleroderma skin. J Invest Dermatol 1980;75(2):189–91. Cross Ref link Pubmed link
99 Manetti M, Milia AF, Guiducci S, Romano E, Matucci‐Cerinic M, Ibba‐Manneschi L. Progressive loss of lymphatic vessels in skin of patients with systemic sclerosis. J Rheumatol 2011;38(2):297–301. Cross Ref link Pubmed link
100 Akhmetshina A, Beer J, Zwerina K, et al. Decreased lymphatic vessel counts in patients with systemic sclerosis: association with fingertip ulcers. Arthritis Rheum 2010;62(5):1513–22. Cross Ref link Pubmed link
101 Rossi A, Sozio F, Sestini P, et al. Lymphatic and blood vessels in scleroderma skin, a morphometric analysis. Hum Pathol 2010;41(3):366–74. Cross Ref link Pubmed link
102 Masi AT, D'Angelo WA. Epidemiology of fatal systemic sclerosis (diffuse scleroderma). A 15‐year survey in Baltimore. Ann Intern Med 1967;66(5):870–83. Cross Ref link Pubmed link
103 D'Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty‐eight autopsy cases and fifty‐eight matched controls. Am J Med 1969;46(3):428–40. Cross Ref link Pubmed link
104 Fischer A, Swigris JJ, Groshong SD, et al. Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. Chest 2008;134(3):601–5. Cross Ref link Pubmed link
105 Al‐Sabbagh MR, Steen VD, Zee BC, et al. Pulmonary arterial histology and morphometry in systemic sclerosis: a case–control autopsy study. J Rheumatol 1989;16(8):1038–42. Pubmed link
106 Hassoun PM, Mouthon L, Barbera JA, et al. Inflammation, growth factors, and pulmonary vascular remodeling. J Am Coll Cardiol 2009;54(Suppl. 1):S10–19. Cross Ref link Pubmed link
107 Cool CD, Kennedy D, Voelkel NF, Tuder RM. Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection. Hum Pathol 1997;28(4):434–42. Cross Ref link Pubmed link
108 Voelkel NF, Tuder RM. Cellular and molecular mechanisms in the pathogenesis of severe pulmonary hypertension. Eur Respir J 1995;8(12):2129–38. Cross Ref link Pubmed link
109 Trostle DC, Bedetti CD, Steen VD, Al‐Sabbagh MR, Zee B, Medsger TA, Jr. Renal vascular histology and morphometry in systemic sclerosis. A case–control autopsy study. Arthritis Rheum 1988;31(3):393–400. Cross Ref link Pubmed link
110 Penn H, Quillinan N, Khan K, et al. Targeting the endothelin axis in scleroderma renal crisis: rationale and feasibility. Q J Med 2013;106(9):839–48. Cross Ref link
111 Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long‐term outcomes. Q J Med 2007;100(8):485–94. Cross Ref link
112 Batal I, Domsic RT, Shafer A, et al. Renal biopsy findings predicting outcome in scleroderma renal crisis. Hum Pathol 2009;40(3):332–40. Cross Ref link Pubmed link
113 Markiewicz M, Smith EA, Rubinchik S, Dong JY, Trojanowska M, LeRoy EC. The 72‐kilodalton IE‐1 protein of human cytomegalovirus (HCMV) is a potent inducer of connective tissue growth factor (CTGF) in human dermal fibroblasts. Clin Exp Rheumatol 2004;22(3 Suppl. 33):S31–4. Pubmed link
114 Moroncini G, Mori S, Tonnini C, Gabrielli A. Role of viral infections in the etiopathogenesis of systemic sclerosis. Clin Exp Rheumatol 2013;31(2 Suppl. 76):3–7. Pubmed link
115 Moinzadeh P, Khan K, Ong VH, Denton CP. Sustained improvement of diffuse systemic sclerosis following human cytomegalovirus infection offers insight into pathogenesis and therapy. Rheumatology 2012;51(12):2296–8. Cross Ref link Pubmed link
116 Frech TM, Khanna D, Maranian P, Frech EJ, Sawitzke AD, Murtaugh MA. Probiotics for the treatment of systemic sclerosis‐associated gastrointestinal bloating/distention. Clin Exp Rheumatol 2011;29(2 Suppl. 65):S22–5. Pubmed link
117 Arnett FC, Cho M, Chatterjee S, Aguilar MB, Reveille JD, Mayes MD. Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis Rheum 2001;44(6):1359–62. Cross Ref link Pubmed link
118 Frech T, Khanna D, Markewitz B, Mineau G, Pimentel R, Sawitzke A. Heritability of vasculopathy, autoimmune disease, and fibrosis in systemic sclerosis: a population‐based study. Arthritis Rheum 2010;62(7):2109–16. Pubmed link
119 Broen JC, Coenen MJ, Radstake TR. Genetics of systemic sclerosis: an update. Curr Rheumatol Rep 2012;14(1):11–21. Cross Ref link Pubmed link
120 Allanore Y, Saad M, Dieude P, Avouac J, Distler JH, Amouyel P, et al. Genome‐wide scan identifies TNIP1, PSORS1C1, and RHOB as novel risk loci for systemic sclerosis. PLoS genetics. 2011;7(7):e1002091. Cross Ref link Pubmed link
121 Radstake TR, Gorlova O, Rueda B, et al. Genome‐wide association study of systemic sclerosis identifies CD247 as a new susceptibility locus. Nature Genet 2010;42(5):426–9. Cross Ref link Pubmed link
122 Mayes MD, Bossini‐Castillo L, Gorlova O, et al. Immunochip analysis identifies multiple susceptibility loci for systemic sclerosis. Am J Hum Genet 2014;94(1):47–61. Cross Ref link Pubmed link
123 Dieude P, Boileau C, Guedj M, et al. Independent replication establishes the CD247 gene as a genetic systemic sclerosis susceptibility factor. Ann Rheum Dis 2011;70(9):1695–6. Cross Ref link Pubmed link
124 Bossini‐Castillo L, Martin JE, Broen J, et al. Confirmation of TNIP1 but not RHOB and PSORS1C1 as systemic sclerosis risk factors in a large independent replication study. Ann Rheum Dis 2013;72(4):602–7. Cross Ref link Pubmed link
125 Jin J, Chou C, Lima M, Zhou D, Zhou X. Systemic sclerosis is a complex disease associated mainly with immune regulatory and inflammatory genes. Open Rheumatol J 2014;8:29–42. Cross Ref link Pubmed link
126 Martin JE, Assassi S, Diaz‐Gallo LM, et al. A systemic sclerosis and systemic lupus erythematosus pan‐meta‐GWAS reveals new shared susceptibility loci. Hum Mol Genet 2013;22(19):4021–9. Cross Ref link Pubmed link
127 Bossini‐Castillo L, Martin JE, Broen J, et al. A GWAS follow‐up study reveals the association of the IL12RB2 gene with systemic sclerosis in Caucasian populations. Hum Mol Genet 2012;21(4):926–33. Cross Ref link Pubmed link
128 Martin JE, Broen JC, Carmona FD, et al. Identification of CSK as a systemic sclerosis genetic risk factor through Genome Wide Association Study follow‐up. Hum Mol Genet 2012;21(12):2825–35. Cross Ref link Pubmed link
129 Carmona FD, Cenit MC, Diaz‐Gallo LM, et al. New insight on the Xq28 association with systemic sclerosis. Ann Rheum Dis 2013;72(12):2032–8. Cross Ref link Pubmed link
130 Dieude P, Bouaziz M, Guedj M, et al. Evidence of the contribution of the X chromosome to systemic sclerosis susceptibility: association with the functional IRAK1 196Phe/532Ser haplotype. Arthritis Rheum 2011;63(12):3979–87. Cross Ref link Pubmed link
131 Ciechomska M, van Laar JM, O'Reilly S. Emerging role of epigenetics in systemic sclerosis pathogenesis. Genes Immun 2014;15(7):433–9. Cross Ref link Pubmed link
132 Altorok N, Almeshal N, Wang Y, Kahaleh B. Epigenetics, the holy grail in the pathogenesis of systemic sclerosis. Rheumatology 2014; 10.1093/rheumatology/keu155. Pubmed link
133 Broen JC, Radstake TR, Rossato M. The role of genetics and epigenetics in the pathogenesis of systemic sclerosis. Nature Rev Rheumatol 2014;10(11):671–81. Cross Ref link
134 Barbieri I, Cannizzaro E, Dawson MA. Bromodomains as therapeutic targets in cancer. Brief Funct Genomics 2013;12(3):219–30. Cross Ref link Pubmed link
135 Tarakhovsky A. Logic of the inflammation‐associated transcriptional response. Adv Immunol 2013;119:107–33. Cross Ref link Pubmed link
136 Klein K, Gay S. Epigenetics in rheumatoid arthritis. Curr Opin Rheumatol 2015;27(1):76–82. Cross Ref link Pubmed link
137 Meng S, Zhang L, Tang Y, et al. BET inhibitor JQ1 blocks inflammation and bone destruction. J Dent Res 2014;93(7):657–62. Cross Ref link Pubmed link
138 Mora GF. Systemic sclerosis: environmental factors. J Rheumatol 2009;36(11):2383–96. Cross Ref link Pubmed link
139 Sluis‐Cremer GK, Hessel PA, Nizdo EH, Churchill AR, Zeiss EA. Silica, silicosis, and progressive systemic sclerosis. Br J Ind Med 1985;42(12):838–43. Pubmed link
140 Dospinescu P, Jones GT, Basu N. Environmental risk factors in systemic sclerosis. Curr Opin Rheumatol 2013;25(2):179–83. Cross Ref link Pubmed link
141 Marie I, Gehanno JF, Bubenheim M, et al. Prospective study to evaluate the association between systemic sclerosis and occupational exposure and review of the literature. Autoimmun Rev 2014;13(2):151–6. Cross Ref link Pubmed link
142 Psarras A, Gkougkourelas I, Tselios K, Sarantopoulos A, Boura P. Systemic sclerosis and silicone breast implant: a case report and review of the literature. Case Rep Rheumatol 2014;2014:809629. Pubmed link
143 Pavlov‐Dolijanovic S, Damjanov NS, Vujasinovic Stupar NZ, Radunovic GL, Stojanovic RM, Babic D. Late appearance and exacerbation of primary Raynaud's phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3,035 patients. Rheumatol Int 2013;33(4):921–6. Cross Ref link Pubmed link
144 Steen V, Denton CP, Pope JE, Matucci‐Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology 2009;48(Suppl. 3):iii19–24. Cross Ref link Pubmed link
145 Moinzadeh P, Nihtyanova SI, Howell K, Ong VH, Denton CP. Impact of hallmark autoantibody reactivity on early diagnosis in scleroderma. Clin Rev Allergy Immunol 2012;43(3):249–55. Cross Ref link Pubmed link
146 Ferri C, Valentini G, Cozzi F, et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine 2002;81(2):139–53. Cross Ref link Pubmed link
147 Hunzelmann N, Genth E, Krieg T, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 2008;47(8):1185–92. Cross Ref link Pubmed link
148 Frech TM, Baron M. Understanding itch in systemic sclerosis in order to improve patient quality of life. Clin Exp Rheumatol 2013;31(2 Suppl. 76):81–8. Pubmed link
149 El‐Baalbaki G, Razykov I, Hudson M, et al. Association of pruritus with quality of life and disability in systemic sclerosis. Arthritis Care Res 2010;62(10):1489–95. Cross Ref link
150 Steen VD, Medsger TA, Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43(11):2437–44. Cross Ref link Pubmed link
151 Thombs BD, Hudson M, Bassel M, Taillefer SS, Baron M, Canadian Scleroderma Research Group. Sociodemographic, disease, and symptom correlates of fatigue in systemic sclerosis: evidence from a sample of 659 Canadian Scleroderma Research Group Registry patients. Arthritis Rheum 2009;61(7):966–73. Cross Ref link Pubmed link
152 Assassi S, Leyva AL, Mayes MD, et al. Predictors of fatigue severity in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort. PLOS One 2011;6(10):e26061. Cross Ref link Pubmed link
153 Avouac J, Fransen J, Walker UA, et al. Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. Ann Rheum Dis 2011;70(3):476–81. Cross Ref link Pubmed link
154 Koenig M, Joyal F, Fritzler MJ, et al. Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud's phenomenon to systemic sclerosis: a twenty‐year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 2008;58(12):3902–12. Cross Ref link Pubmed link
155 Minier T, Guiducci S, Bellando‐Randone S, et al. 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;73(12):2087–93. Cross Ref link Pubmed link
156 Mazzotti NG, Bredemeier M, Brenol CV, Xavier RM, Cestari TF. Assessment of nailfold capillaroscopy in systemic sclerosis by different optical magnification methods. Clin Exp Dermatol 2014;39(2):135–41. Cross Ref link Pubmed link
157 Clements P, Lachenbruch P, Siebold J, et al. Inter and intraobserver variability of total skin thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol 1995;22(7):1281–5. Pubmed link
158 Clements PJ, Lachenbruch PA, Ng SC, Simmons M, Sterz M, Furst DE. Skin score. A semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis. Arthritis Rheum 1990;33(8):1256–63. Cross Ref link Pubmed link
159 Furst DE, Clements PJ, Steen VD, et al. The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis. J Rheumatol 1998;25(1):84–8. Pubmed link
160 Valentini G, D'Angelo S, Della Rossa A, Bencivelli W, Bombardieri S. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. IV. Assessment of skin thickening by modified Rodnan skin score. Ann Rheum Dis 2003;62(9):904–5. Cross Ref link Pubmed link
161 Kissin EY, Schiller AM, Gelbard RB, et al. Durometry for the assessment of skin disease in systemic sclerosis. Arthritis Rheum 2006;55(4):603–9. Cross Ref link Pubmed link
162 Seyger MM, van den Hoogen FH, de Boo T, de Jong EM. Reliability of two methods to assess morphea: skin scoring and the use of a durometer. J Am Acad Dermatol 1997;37(5 Pt 1):793–6. Cross Ref link Pubmed link
163 Ch'ng SS, Roddy J, Keen HI. A systematic review of ultrasonography as an outcome measure of skin involvement in systemic sclerosis. Int J Rheum Dis 2013;16(3):264–72. Cross Ref link Pubmed link
164 Moore TL, Lunt M, McManus B, Anderson ME, Herrick AL. Seventeen‐point dermal ultrasound scoring system – a reliable measure of skin thickness in patients with systemic sclerosis. Rheumatology 2003;42(12):1559–63. Cross Ref link Pubmed link
165 Abignano G, Aydin SZ, Castillo‐Gallego C, et al. Virtual skin biopsy by optical coherence tomography: the first quantitative imaging biomarker for scleroderma. Ann Rheum Dis 2013;72(11):1845–51. Cross Ref link Pubmed link
166 Razykov I, Levis B, Hudson M, Baron M, Thombs BD, Canadian Scleroderma Research Group. Prevalence and clinical correlates of pruritus in patients with systemic sclerosis: an updated analysis of 959 patients. Rheumatology 2013;52(11):2056–61. Cross Ref link Pubmed link
167 Greydanus MP, Camilleri M. Abnormal postcibal antral and small bowel motility due to neuropathy or myopathy in systemic sclerosis. Gastroenterology 1989;96(1):110–15. Pubmed link
168 Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 2007;66(7):940–4. Cross Ref link Pubmed link
169 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;69(10):1809–15. Cross Ref link Pubmed link
170 Coghlan JG, Denton CP, Grunig E, et al. Evidence‐based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014;73(7):1340–9. Cross Ref link Pubmed link
171 Denton CP, Lapadula G, Mouthon L, Muller‐Ladner U. Renal complications and scleroderma renal crisis. Rheumatology 2009;48(Suppl. 3):iii32–5. Cross Ref link Pubmed link
172 Cuomo G, Zappia M, Iudici M, Abignano G, Rotondo A, Valentini G. The origin of tendon friction rubs in patients with systemic sclerosis: a sonographic explanation. Arthritis Rheum 2012;64(4):1291–3. Cross Ref link Pubmed link
173 Stoenoiu MS, Houssiau FA, Lecouvet FE. Tendon friction rubs in systemic sclerosis: a possible explanation – an ultrasound and magnetic resonance imaging study. Rheumatology 2013;52(3):529–33. Cross Ref link Pubmed link
174 Dore 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 2013;65(8):1385–9. Cross Ref link
175 Steen VD, Medsger TA, Jr. The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis. Arthritis Rheum 1997;40(6):1146–51. Cross Ref link Pubmed link
176 Ranque B, Authier FJ, Berezne A, Guillevin L, Mouthon L. Systemic sclerosis‐associated myopathy. Ann NY Acad Sci 2007;1108:268–82. Cross Ref link Pubmed link
177 Jung M, Bonner A, Hudson M, Baron M, Pope JE, Canadian Scleroderma Research Group. 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. Cross Ref link Pubmed link
178 Pavlov‐Dolijanovic S, Damjanov NS, Stojanovic RM, Vujasinovic Stupar NZ, Stanisavljevic DM. Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: a follow‐up study of 3,029 patients with primary Raynaud's phenomenon. Rheumatol Int 2012;32(10):3039–45. Cross Ref link Pubmed link
179 Pavlov‐Dolijanovic SR, Damjanov NS, Vujasinovic Stupar NZ, Baltic S, Babic DD. The value of pattern capillary changes and antibodies to predict the development of systemic sclerosis in patients with primary Raynaud's phenomenon. Rheumatol Int 2013;33(12):2967‐73. Cross Ref link Pubmed link
180 Prete M, Fatone MC, Favoino E, Perosa F. Raynaud's phenomenon: from molecular pathogenesis to therapy. Autoimmun Rev 2014;13(6):655–67. Cross Ref link Pubmed link
181 Soma Y, Tamaki T, Kikuchi K, et al. Coexistence of morphea and systemic sclerosis. Dermatology 1993;186(2):103–5. Cross Ref link Pubmed link
182 Mittal G, Maddison P, Williams W. Systemic sclerosis, morphoea and breast cancer. Rheumatology 2006;45(1):119–20. Cross Ref link Pubmed link
183 Kim A, Marinkovich N, Vasquez R, Jacobe HT. Clinical features of patients with morphea and the pansclerotic subtype: a cross‐sectional study from the morphea in adults and children cohort. J Rheumatol 2014;41(1):106–12. Cross Ref link Pubmed link
184 Pinal‐Fernandez I, Selva‐O' Callaghan A, Grau JM. Diagnosis and classification of eosinophilic fasciitis. Autoimmun Rev 2014;13(4–5):379–82. Cross Ref link Pubmed link
185 Miller JJ, 3rd. The fasciitis‐morphea complex in children. Am J Dis Child 1992;146(6):733–6. Pubmed link
186 Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 1988;17(4):221–31. Cross Ref link Pubmed link
187 Azevedo VF, Serafini SZ, Werner B, Muller CS, Franchini CF, Morais RL. Stiff skin syndrome versus scleroderma: a report of two cases. Clin Rheumatol 2009;28(9):1107–11. Cross Ref link Pubmed link
188 Loeys BL, Gerber EE, Riegert‐Johnson D, et al. Mutations in fibrillin‐1 cause congenital scleroderma: stiff skin syndrome. Sci Transl Med 2010;2(23):23ra0.
189 Assassi S, Del Junco D, Sutter K, et al. Clinical and genetic factors predictive of mortality in early systemic sclerosis. Arthritis Rheum 2009;61(10):1403–11. Cross Ref link Pubmed link
190 Rubio‐Rivas M, Royo C, Simeon CP, Corbella X, Fonollosa V. Mortality and survival in systemic sclerosis: systematic review and meta‐analysis. Semin Arthritis Rheum 2014;44(2):208–19. Cross Ref link Pubmed link
191 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. Q J Med 2010;103(2):109–15. Cross Ref link
192 Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long‐term survival in systemic sclerosis. Arthritis Rheumatol 2014;66(6):1625–35. Cross Ref link Pubmed link
193 Shand L, Lunt M, Nihtyanova S, et al. Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model. Arthritis Rheum 2007;56(7):2422–31. Cross Ref link Pubmed link
194 Saketkoo LA, Mittoo S, Huscher D, et al. Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials. Thorax 2014;69(5):428–36. Cross Ref link Pubmed link
195 Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Rrespir Crit Care Med 2008;177(11):1248–54. Cross Ref link
196 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 2012;51(6):1017–26. Cross Ref link Pubmed link
197 Nikpour M, Baron M. Mortality in systemic sclerosis: lessons learned from population‐based and observational cohort studies. Curr Opin Rheumatol 2014;26(2):131–7. Cross Ref link Pubmed link
198 Chung L, Farber HW, Benza R, et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the Reveal Registry. Chest 2014;146(6):1494–504. Cross Ref link Pubmed link
199 Chung L, Domsic RT, Lingala B, 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 2014;66(3):489–95. Cross Ref link
200 Tashkin DP, Elashoff R, Clements PJ, et al. Effects of 1‐year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 2007;176(10):1026–34. Cross Ref link Pubmed link
201 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(12):3962–70. Cross Ref link Pubmed link
202 Valerio CJ, Handler CE, Kabunga P, Smith CJ, Denton CP, Coghlan JG. Clinical experience with bosentan and sitaxentan in connective tissue disease‐associated pulmonary arterial hypertension. Rheumatology 2010;49(11):2147–53. Cross Ref link Pubmed link
203 Williams MH, Das C, Handler CE, et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006;92(7):926–32. Cross Ref link Pubmed link
204 Schneeberger D, Tyndall A, Kay J, et al. Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database. Rheumatology 2013;52(3):560–7. Cross Ref link Pubmed link
205 Furst DE, Clements PJ, Harris R, Ross M, Levy J, Paulus HE. Measurement of clinical change in progressive systemic sclerosis: a 1 year double‐blind placebo‐controlled trial of N‐acetylcysteine. Ann Rheum Dis 1979;38(4):356–61. Cross Ref link Pubmed link
206 Clements P, Lachenbruch P, Furst D, Paulus H. The course of skin involvement in systemic sclerosis over three years in a trial of chlorambucil versus placebo. Arthritis Rheum 1993;36(11):1575–9. Cross Ref link Pubmed link
207 Black CM, Silman AJ, Herrick AI, et al. Interferon‐alpha does not improve outcome at one year in patients with diffuse cutaneous scleroderma: results of a randomized, double‐blind, placebo‐controlled trial. Arthritis Rheum 1999;42(2):299–305. Cross Ref link Pubmed link
208 Mayes MD, O'Donnell D, Rothfield NF, Csuka ME. Minocycline is not effective in systemic sclerosis: results of an open‐label multicenter trial. Arthritis Rheum 2004;50(2):553–7. Cross Ref link Pubmed link
209 Khanna D, Clements PJ, Furst DE, et al. Recombinant human relaxin in the treatment of systemic sclerosis with diffuse cutaneous involvement: a randomized, double‐blind, placebo‐controlled trial. Arthritis Rheum 2009;60(4):1102–11. Cross Ref link Pubmed link
210 Knobler RM, French LE, Kim Y, et al. A randomized, double‐blind, placebo‐controlled trial of photopheresis in systemic sclerosis. J Am Acad Dermatol 2006;54(5):793–9. Cross Ref link Pubmed link
211 Gambichler T, Terras S, Kreuter A. Treatment regimens, protocols, dosage, and indications for UVA1 phototherapy: facts and controversies. Clin Dermatol 2013;31(4):438–54. Cross Ref link Pubmed link
212 Durand F, Staumont D, Bonnevalle A, Hachulla E, Hatron PY, Thomas P. Ultraviolet A1 phototherapy for treatment of acrosclerosis in systemic sclerosis: controlled study with half‐side comparison analysis. Photodermatol Photoimmunol Photomed 2007;23(6):215–21. Cross Ref link Pubmed link
213 Furst DE, Clements PJ. D‐penicillamine is not an effective treatment in systemic sclerosis. Scand J Rheumatol 2001;30(4):189–91. Cross Ref link Pubmed link
214 Medsger TA, Jr, Lucas M, Wildy KS, Baker C. D‐penicillamine in systemic sclerosis? Yes! Scand J Rheumatol 2001;30(4):192–4. Cross Ref link Pubmed link
215 Clements PJ, Seibold JR, Furst DE, et al. High‐dose versus low‐dose D‐penicillamine in early diffuse systemic sclerosis trial: lessons learned. Semin Arthritis Rheum 2004;33(4):249–63. Cross Ref link Pubmed link
216 Iwamoto N, Distler JH, Distler O. Tyrosine kinase inhibitors in the treatment of systemic sclerosis: from animal models to clinical trials. Curr Rheumatol Rep 2011;13(1):21–7. Cross Ref link Pubmed link
217 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;70(6):1003–9. Cross Ref link Pubmed link
218 Gordon J, Udeh U, Doobay K, et al. Imatinib mesylate (Gleevec) in the treatment of diffuse cutaneous systemic sclerosis: results of a 24‐month open label, extension phase, single‐centre trial. Clin Exp Rheumatol 2014;32(6 Suppl. 86):S189–93.
219 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;63(11):3547–51. Cross Ref link Pubmed link
220 Bournia VK, Evangelou K, Sfikakis PP. Therapeutic inhibition of tyrosine kinases in systemic sclerosis: a review of published experience on the first 108 patients treated with imatinib. Semin Arthritis Rheum 2013;42(4):377–90. Cross Ref link Pubmed link
221 Prey S, Ezzedine K, Doussau A, et al. Imatinib mesylate in scleroderma‐associated diffuse skin fibrosis: a phase II multicentre randomized double‐blinded controlled trial. Br J Dermatol 2012;167(5):1138–44. Cross Ref link Pubmed link
222 Fraticelli P, Gabrielli B, Pomponio G, et al. Low‐dose oral imatinib in the treatment of systemic sclerosis interstitial lung disease unresponsive to cyclophosphamide: a phase II pilot study. Arthritis Res Ther 2014;16(4):R144. Cross Ref link Pubmed link
223 Takehara K, Ihn H, Sato S. A randomized, double‐blind, placebo‐controlled trial: intravenous immunoglobulin treatment in patients with diffuse cutaneous systemic sclerosis. Clin Exp Rheumatol 2013;31(2 Suppl. 76):151–6. Pubmed link
224 Pope JE, Bellamy N, Seibold JR, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 2001;44(6):1351–8. Cross Ref link Pubmed link
225 Van den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB. 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;35(4):364–72. Cross Ref link Pubmed link
226 Nihtyanova SI, Brough GM, Black CM, Denton CP. Mycophenolate mofetil in diffuse cutaneous systemic sclerosis – a retrospective analysis. Rheumatology 2007;46(3):442–5. Cross Ref link Pubmed link
227 Mendoza FA, Nagle SJ, Lee JB, Jimenez SA. A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol 2012;39(6):1241–7. Cross Ref link Pubmed link
228 Derk CT, Grace E, Shenin M, Naik M, Schulz S, Xiong W. A prospective open‐label study of mycophenolate mofetil for the treatment of diffuse systemic sclerosis. Rheumatology 2009;48(12):1595–9. Cross Ref link Pubmed link
229 Panopoulos ST, Bournia VK, Trakada G, Giavri I, Kostopoulos C, Sfikakis PP. Mycophenolate versus cyclophosphamide for progressive interstitial lung disease associated with systemic sclerosis: a 2‐year case control study. Lung 2013;191(5):483–9. Cross Ref link Pubmed link
230 Le EN, Wigley FM, Shah AA, Boin F, Hummers LK. Long‐term experience of mycophenolate mofetil for treatment of diffuse cutaneous systemic sclerosis. Ann Rheum Dis 2011;70(6):1104–7. Cross Ref link Pubmed link
231 Tzouvelekis A, Galanopoulos N, Bouros E, et al. Effect and safety of mycophenolate mofetil or sodium in systemic sclerosis‐associated interstitial lung disease: a meta‐analysis. Pulm Med 2012;2012:143637. Cross Ref link Pubmed link
232 Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354(25):2655–66. Cross Ref link Pubmed link
233 Nadashkevich O, Davis P, Fritzler M, Kovalenko W. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 2006;25(2):205–12. Cross Ref link Pubmed link
234 Paone C, Chiarolanza I, Cuomo G, et al. Twelve‐month azathioprine as maintenance therapy in early diffuse systemic sclerosis patients treated for 1‐year with low dose cyclophosphamide pulse therapy. Clin Exp Rheumatol 2007;25(4):613–16. Pubmed link
235 Berezne A, Ranque B, Valeyre D, et al. Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open‐label study. J Rheumatol 2008;35(6):1064–72. Pubmed link
236 Iudici M, Cuomo G, Vettori S, et al. Low‐dose pulse cyclophosphamide in interstitial lung disease associated with systemic sclerosis (SSc‐ILD): efficacy of maintenance immunosuppression in responders and non‐responders. Semin Arthritis Rheum 2015;44(4):437–44. Cross Ref link Pubmed link
237 Poormoghim H, Rezaei N, Sheidaie Z, et al. Systemic sclerosis: comparison of efficacy of oral cyclophosphamide and azathioprine on skin score and pulmonary involvement‐a retrospective study. Rheumatol Int 2014;34(12):1691–9. Cross Ref link Pubmed link
238 Walker KM, Pope J, participating members of the Scleroderma Clinical Trials Consortium (SCTC), Canadian Scleroderma Research Group. Treatment of systemic sclerosis complications: what to use when first‐line treatment fails – a consensus of systemic sclerosis experts. Semin Arthritis Rheum 2012;42(1):42–55. Cross Ref link Pubmed link
239 Kowal‐Bielecka O, Landewe R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009;68(5):620–8. Cross Ref link Pubmed link
240 Frech TM, Shanmugam VK, Shah AA, et al. Treatment of early diffuse systemic sclerosis skin disease. Clin Exp Rheumatol 2013;31(2 Suppl. 76):166–71. Pubmed link
241 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(24):2490–8. Cross Ref link Pubmed link
242 Van Laar JM, McSweeney PA. High‐dose immunosuppressive therapy and autologous progenitor cell transplantation for systemic sclerosis. Best Pract Res Clin Haematol 2004;17(2):233–45. Cross Ref link Pubmed link
243 Van Laar JM, Sullivan K. Stem cell transplantation in systemic sclerosis. Curr Opin Rheumatol 2013;25(6):719–25. Cross Ref link Pubmed link
244 Yoshizaki A, Yanaba K, Yoshizaki A, et al. Treatment with rapamycin prevents fibrosis in tight‐skin and bleomycin‐induced mouse models of systemic sclerosis. Arthritis Rheum 2010;62(8):2476–87. Cross Ref link Pubmed link
245 Su TI, Khanna D, Furst DE, et al. Rapamycin versus methotrexate in early diffuse systemic sclerosis: results from a randomized, single‐blind pilot study. Arthritis Rheum 2009;60(12):3821–30. Cross Ref link Pubmed link
246 Elhai M, Meunier M, Matucci‐Cerinic M, et al. Outcomes of patients with systemic sclerosis‐associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study. Ann Rheum Dis 2013;72(7):1217–20. Cross Ref link Pubmed link
247 De Paoli FV, Nielsen BD, Rasmussen F, Deleuran B, Sondergaard K. Abatacept induces clinical improvement in patients with severe systemic sclerosis. Scand J Rheumatol 2014;43(4):342–5. Cross Ref link Pubmed link
248 Jordan S, Distler JH, Maurer B, et al. Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 2015;74(6):1188–94. Cross Ref link Pubmed link
249 Saito E, Sato S, Nogi S, et al. A case of rheumatoid arthritis and limited systemic sclerosis overlap successfully treated with tocilizumab for arthritis and concomitant generalized lymphadenopathy and primary biliary cirrhosis. Case Rep Rheumatol 2014;2014:386328. Pubmed link
250 Shima Y, Hosen N, Hirano T, et al. Expansion of range of joint motion following treatment of systemic sclerosis with tocilizumab. Mod Rheumatol 2015;25(1):134–7. Cross Ref link Pubmed link
251 Quillinan NP, McIntosh D, Vernes J, Haq S, Denton CP. Treatment of diffuse systemic sclerosis with hyperimmune caprine serum (AIMSPRO): a phase II double‐blind placebo‐controlled trial. Ann Rheum Dis 2014;73(1):56–61. Cross Ref link Pubmed link
252 Clements PJ, Tan M, McLaughlin VV, et al. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis‐associated PAH. Ann Rheum Dis 2012;71(2):249–52. Cross Ref link Pubmed link
253 Murdaca G, Spano F, Puppo F. Current therapies for the treatment of systemic sclerosis‐related pulmonary arterial hypertension: efficacy and safety. Expert Opin Drug Safety 2014;13(3):295–305. Cross Ref link
254 George PM, Oliver E, Dorfmuller P, et al. Evidence for the involvement of type I interferon in pulmonary arterial hypertension. Circ Res 2014;114(4):677–88. Cross Ref link Pubmed link
255 Steen VD. Kidney involvement in systemic sclerosis. Presse Med 2014;43(10 Pt 2):e305–14. Cross Ref link Pubmed link
256 Mouthon L, Mehrenberger M, Teixeira L, et al. Endothelin‐1 expression in scleroderma renal crisis. Hum Pathol 2011;42(1):95–102. Cross Ref link Pubmed link
257 Murdaca G, Spano F, Contatore M, Guastalla A, Puppo F. Potential use of TNF‐alpha inhibitors in systemic sclerosis. Immunotherapy 2014;6(3):283–9. Cross Ref link Pubmed link
258 Foocharoen C, Tyndall A, Hachulla E, 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;14(1):R37. Cross Ref link Pubmed link
259 Reiter N, El‐Shabrawi L, Leinweber B, Berghold A, Aberer E. Calcinosis cutis: part II. Treatment options. J Am Acad Dermatol 2011;65(1):15–22; quiz 3–4. Cross Ref link Pubmed link
260 Walker KM, Pope J, Scleroderma Clinical Trials Consortium (SCTC), Canadian Scleroderma Research Group. Expert agreement on EULAR/EUSTAR recommendations for the management of systemic sclerosis. J Rheumatol 2011;38(7):1326–8. Cross Ref link Pubmed link

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