Systemic sclerosis: An update in 2016

Autoimmunity Reviews

Volumn 15, Issue 5, 2016, Pages 417-426

  Desbois, Anne Claire ^a,b,c   Cacoub, Patrice ^a,b,d  

^a SORBONNE UNIVERSITÉ   (France)

^b Inflammation Immunopathology Biotherapy Department (DHU i2B)   (France)

^c Sorbonne Université   (France)

^d PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

Author keywords

Autoimmune disease; Systemic sclerosis; Treatment

Indexed keywords

BIOLOGICAL MARKER;

AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION; FINGER ULCER; FORCED VITAL CAPACITY; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; INTERSTITIAL LUNG DISEASE; MORBIDITY; MORTALITY; PREDICTIVE VALUE; PROGNOSIS; PULMONARY HYPERTENSION; RAYNAUD PHENOMENON; REVIEW; SCLERODERMA; SKIN DEFECT; SYSTEMIC SCLEROSIS; COMPLICATION; LUNG DISEASE; SCLERODERMA, SYSTEMIC;

BIOMARKERS; HUMANS; LUNG DISEASES; PROGNOSIS; SCLERODERMA, SYSTEMIC;

EID: 84971282268     PISSN: 15689972     EISSN: 18730183     Source Type: Journal    
DOI: 10.1016/j.autrev.2016.01.007     Document Type: Review

References (108)

1. Almeida C., Almeida I., Vasconcelos C. Quality of life in systemic sclerosis. Autoimmun Rev 2015, 14:1087-1096. 10.1016/j.autrev.2015.07.012.
2. Elhai M., Avouac J., Walker U.A., Matucci-Cerinic M., Riemekasten G., Airo P., et al. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 2014, 10.1136/annrheumdis-2014-206386.
3. Bruni C., Raja J., Denton C.P., Matucci-Cerinic M. The clinical relevance of sexual dysfunction in systemic sclerosis. Autoimmun Rev 2015, 14:1111-1115. 10.1016/j.autrev.2015.07.016.
4. Minier T., Guiducci S., Bellando-Randone S., Bruni C., Lepri G., Czirjak L., 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, 73:2087-2093. 10.1136/annrheumdis-2013-203716.
5. Clements P.J., Lachenbruch P.A., Seibold J.R., Zee B., Steen V.D., Brennan P., et al. Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 1993, 20:1892-1896.
6. Walker U.A., Tyndall A., Czirjak L., Denton C., Farge-Bancel D., Kowal-Bielecka O., 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, 66:754-763. 10.1136/ard.2006.062901.
7. Mihai C., Landewe R., van der Heijde D., Walker U.A., Constantin P.I., Gherghe A.M., et al. Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis 2015, 10.1136/annrheumdis-2014-205897.
8. Galiè N., Humbert M., Vachiery J.-L., Gibbs S., Lang I., Torbicki A., et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015, 46:903-975. 10.1183/13993003.01032-2015.
9. Mukerjee D., St George D., Coleiro B., Knight C., Denton C.P., Davar J., et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003, 62:1088-1093.
10. Hachulla E., de Groote P., Gressin V., Sibilia J., Diot E., Carpentier P., et al. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 2009, 60:1831-1839. 10.1002/art.24525.
11. Hassoun M. P. Lung involvement in systemic sclerosis. Presse Med 2011, 40:e25-e39. 10.1016/j.lpm.2010.08.006.
12. Coghlan J.G., Denton C.P., Grunig E., Bonderman D., Distler O., Khanna D., et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014, 73:1340-1349. 10.1136/annrheumdis-2013-203301.
13. Nihtyanova S.I., Schreiber B.E., Ong V.H., Rosenberg D., Moinzadeh P., Coghlan J.G., et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol Hoboken NJ 2014, 66:1625-1635. 10.1002/art.38390.
14. Fischer A., Swigris J.J., Groshong S.D., Cool C.D., Sahin H., Lynch D.A., et al. Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. CHEST J 2008, 134:601-605.
15. Papagoras C., Achenbach K., Tsifetaki N., Tsiouris S., Fotopoulos A., Drosos A.A. Heart involvement in systemic sclerosis: a combined echocardiographic and scintigraphic study. Clin Rheumatol 2014, 33:1105-1111. 10.1007/s10067-014-2666-3.
16. Marie I., Leroi A.-M., Menard J.-F., Levesque H., Quillard M., Ducrotte P. Fecal calprotectin in systemic sclerosis and review of the literature. Autoimmun Rev 2015, 14:547-554. 10.1016/j.autrev.2015.01.018.
17. De Groote P., Gressin V., Hachulla E., Carpentier P., Guillevin L., Kahan A., et al. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis 2008, 67:31-36.
18. Avouac J., Walker U.A., Hachulla E., Riemekasten G., Cuomo G., Carreira P.E., et al. Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 2014, 10.1136/annrheumdis-2014-205295.
19. Steen V.D. Scleroderma renal crisis. Rheum Dis Clin North Am 2003, 29:315-333.
20. Mouthon L., Bussone G., Berezné A., Noël L.-H., Guillevin L. Scleroderma renal crisis. J Rheumatol 2014, 41:1040-1048. 10.3899/jrheum.131210.
21. Amaral T.N., Peres F.A., Lapa A.T., Marques-Neto J.F., Appenzeller S. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum 2013, 43:335-347. 10.1016/j.semarthrit.2013.05.002.
22. Diab S., Dostrovsky N., Hudson M., Tatibouet S., Fritzler M.J., Baron M., et al. Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects. J Rheumatol 2014, 41:2179-2185.
23. Marangoni R.G., Rocha L.F., Del Rio A.P.T., Yoshinari N.H., Marques-Neto J.F., Sampaio-Barros P.D. Systemic sclerosis sine scleroderma: distinct features in a large Brazilian cohort. Rheumatology 2013, 52:1520-1524. 10.1093/rheumatology/ket163.
24. Bhansing K.J., Lammens M., Knaapen H.K., van Riel P.L., van Engelen B.G., Vonk M.C. Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology. Arthritis Res Ther 2014, 16:R111.
25. Guillen-Del Castillo A., Pilar Simeón-Aznar C., Fonollosa-Pla V., Alonso-Vila S., Reverte-Vinaixa M.M., Muñoz X., et al. Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody. Semin Arthritis Rheum 2014, 44:331-337. 10.1016/j.semarthrit.2014.07.002.
26. Marie I. Systemic sclerosis and occupational exposure: towards an extension of legal recognition as occupational disorder in 2014?. Rev Médecine Interne Fondée Par Société Natl Francaise Médecine Interne 2014, 35:631-635. 10.1016/j.revmed.2014.04.002.
27. Martin J.-E., Broen J.C., Carmona F.D., Teruel M., Simeon C.P., Vonk M.C., et al. Identification of CSK as a systemic sclerosis genetic risk factor through genome wide association study follow-up. Hum Mol Genet 2012, 21:2825-2835. 10.1093/hmg/dds099.
28. Radstake T.R.D.J., Gorlova O., Rueda B., Martin J.-E., Alizadeh B.Z., Palomino-Morales R., et al. Genome-wide association study of systemic sclerosis identifies CD247 as a new susceptibility locus. Nat Genet 2010, 42:426-429. 10.1038/ng.565.
29. Rueda B., Gourh P., Broen J., Agarwal S.K., Simeon C., Ortego-Centeno N., et al. BANK1 functional variants are associated with susceptibility to diffuse systemic sclerosis in Caucasians. Ann Rheum Dis 2010, 69:700-705. 10.1136/ard.2009.118174.
30. Dieudé P., Guedj M., Wipff J., Ruiz B., Hachulla E., Diot E., et al. STAT4 is a genetic risk factor for systemic sclerosis having additive effects with IRF5 on disease susceptibility and related pulmonary fibrosis. Arthritis Rheum 2009, 60:2472-2479. 10.1002/art.24688.
31. Arnett F.C., Gourh P., Shete S., Ahn C.W., Honey R.E., Agarwal S.K., 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, 69:822-827. 10.1136/ard.2009.111906.
32. Gilchrist F.C., Bunn C., Foley P.J., Lympany P.A., Black C.M., Welsh K.I., et al. Class II HLA associations with autoantibodies in scleroderma: a highly significant role for HLA-DP. Genes Immun 2001, 2:76-81. 10.1038/sj.gene.6363734.
33. Distler O., Del Rosso A., Giacomelli R., Cipriani P., Conforti M.L., Guiducci S., et al. Angiogenic and angiostatic factors in systemic sclerosis: increased levels of vascular endothelial growth factor are a feature of the earliest disease stages and are associated with the absence of fingertip ulcers. Arthritis Res 2002, 4:R11.
34. Maurer B., Distler A., Suliman Y.A., Gay R.E., Michel B.A., Gay S., et al. Vascular endothelial growth factor aggravates fibrosis and vasculopathy in experimental models of systemic sclerosis. Ann Rheum Dis 2014, 73:1880-1887. 10.1136/annrheumdis-2013-203535.
35. Manetti M., Guiducci S., Romano E., Bellando-Randone S., Lepri G., Bruni C., et al. Increased plasma levels of the VEGF165b splice variant are associated with the severity of nailfold capillary loss in systemic sclerosis. Ann Rheum Dis 2013, 72:1425-1427. 10.1136/annrheumdis-2012-203183.
36. Shirai Y., Okazaki Y., Inoue Y., Tamura Y., Yasuoka H., Takeuchi T., et al. Elevated levels of pentraxin 3 in systemic sclerosis: associations with vascular manifestations and defective vasculogenesis: PTX3 and SSc vasculopathy. Arthritis Rheumatol 2015, 67:498-507. 10.1002/art.38953.
37. Dumoitier N., Lofek S., Mouthon L. Pathophysiology of systemic sclerosis: state of the art in 2014. Presse Méd Paris France 1983 2014, 43:e267-e278. 10.1016/j.lpm.2014.08.001.
38. Kawakami T., Ihn H., Xu W., Smith E., LeRoy C., Trojanowska M. Increased expression of TGF-beta receptors by scleroderma fibroblasts: evidence for contribution of autocrine TGF-beta signaling to scleroderma phenotype. J Invest Dermatol 1998, 110:47-51. 10.1046/j.1523-1747.1998.00073.x.
39. Parel Y., Aurrand-Lions M., Scheja A., Dayer J.-M., Roosnek E., Chizzolini C. Presence of CD4+CD8+ double-positive T cells with very high interleukin-4 production potential in lesional skin of patients with systemic sclerosis. Arthritis Rheum 2007, 56:3459-3467. 10.1002/art.22927.
40. Luzina I.G., Atamas S.P., Wise R., Wigley F.M., Choi J., Xiao H.Q., et al. Occurrence of an activated, profibrotic pattern of gene expression in lung CD8+ T cells from scleroderma patients. Arthritis Rheum 2003, 48:2262-2274. 10.1002/art.11080.
41. Sato S., Fujimoto M., Hasegawa M., Takehara K. Altered blood B lymphocyte homeostasis in systemic sclerosis: expanded naive B cells and diminished but activated memory B cells. Arthritis Rheum 2004, 50:1918-1927. 10.1002/art.20274.
42. Becker M.O., Kill A., Kutsche M., Guenther J., Rose A., Tabeling C., et al. Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis. Am J Respir Crit Care Med 2014, 190:808-817. 10.1164/rccm.201403-0442OC.
43. Sato S., Hasegawa M., Takehara K. Serum levels of interleukin-6 and interleukin-10 correlate with total skin thickness score in patients with systemic sclerosis. J Dermatol Sci 2001, 27:140-146.
44. Chizzolini C. T cells, B cells, and polarized immune response in the pathogenesis of fibrosis and systemic sclerosis. Curr Opin Rheumatol 2008, 20:707-712. 10.1097/BOR.0b013e32830c45ae.
45. Van Bon L., Affandi A.J., Broen J., Christmann R.B., Marijnissen R.J., Stawski L., et al. Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med 2014, 370:433-443. 10.1056/NEJMoa1114576.
46. Mehra S., Walker J., Patterson K., Fritzler M.J. Autoantibodies in systemic sclerosis. Autoimmun Rev 2013, 12:340-354. 10.1016/j.autrev.2012.05.011.
47. Van den Hoogen F., Khanna D., Fransen J., Johnson S.R., Baron M., Tyndall A., et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis 2013, 72:1747-1755. 10.1136/annrheumdis-2013-204424.
48. Sobanski V., Dauchet L., Lefèvre G., Lambert M., Morell-Dubois S., Sy T., et al. Prevalence of anti-RNA polymerase III antibodies in systemic sclerosis: new data from a French cohort and a systematic review and meta-analysis: prevalence of anti-RNAP III in SSc. Arthritis Rheumatol 2014, 66:407-417. 10.1002/art.38219.
49. Hesselstrand R., Scheja A., Wuttge D. Scleroderma renal crisis in a Swedish systemic sclerosis cohort: survival, renal outcome, and RNA polymerase III antibodies as a risk factor. Scand J Rheumatol 2012, 41:39-43. 10.3109/03009742.2011.610032.
50. Nikpour M., Hissaria P., Byron J., Sahhar J., Micallef M., Paspaliaris W., et al. Prevalence, correlates and clinical usefulness of antibodies to RNA polymerase III in systemic sclerosis: a cross-sectional analysis of data from an Australian cohort. Arthritis Res Ther 2011, 13:R211.
51. Moinzadeh P., Fonseca C., Hellmich M., Shah A.A., Chighizola C., Denton C.P., et al. Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 2014, 16:R53.
52. Graf S.W., Hakendorf P., Lester S., Patterson K., Walker J.G., SMITH M., et al. South Australian scleroderma register: autoantibodies as predictive biomarkers of phenotype and outcome. Int J Rheum Dis 2012, 15:102-109.
53. Mitri G.M., Lucas M., Fertig N., Steen V.D., Medsger T.A. A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement. Arthritis Rheum 2003, 48:203-209. 10.1002/art.10760.
54. Ceribelli A., Cavazzana I., Franceschini F., Airò P., Tincani A., Cattaneo R., et al. Anti-Th/To are common antinucleolar autoantibodies in Italian patients with scleroderma. J Rheumatol 2010, 37:2071-2075. 10.3899/jrheum.100316.
55. Sharif R., Fritzler M.J., Mayes M.D., Gonzalez E.B., McNearney T.A., Draeger H., et al. Anti-fibrillarin antibody in African American patients with systemic sclerosis: immunogenetics, clinical features, and survival analysis. J Rheumatol 2011, 38:1622-1630. 10.3899/jrheum.110071.
56. Aggarwal R., Lucas M., Fertig N., Oddis C.V., Medsger T.A. Anti-U3 RNP autoantibodies in systemic sclerosis. Arthritis Rheum 2009, 60:1112-1118. 10.1002/art.24409.
57. LeRoy E.C., Medsger T.A. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001, 28:1573-1576.
58. Koenig M., Joyal F., Fritzler M.J., Roussin A., Abrahamowicz M., Boire G., 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, 58:3902-3912. 10.1002/art.24038.
59. Valentini G., Cuomo G., Abignano G., Petrillo A., Vettori S., Capasso A., et al. Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features. Rheumatol Oxf Engl 2011, 50:317-323. 10.1093/rheumatology/keq176.
60. Clements P.J., Tan M., McLaughlin V.V., Oudiz R.J., Tapson V.F., Channick R.N., 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, 71:249-252. 10.1136/annrheumdis-2011-200265.
61. Chung L., Liu J., Parsons L., Hassoun P.M., McGoon M., Badesch D.B., et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 2010, 138:1383-1394. 10.1378/chest.10-0260.
62. Chung L., Farber H.W., Benza R., Miller D.P., Parsons L., Hassoun P.M., et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the reveal registry. CHEST J 2014, 146:1494-1504.
63. Winstone T.A., Assayag D., Wilcox P.G., Dunne J.V., Hague C.J., Leipsic J., et al. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest 2014, 146:422-436. 10.1378/chest.13-2626.
64. Iudici M., Moroncini G., Cipriani P., Giacomelli R., Gabrielli A., Valentini G. Where are we going in the management of interstitial lung disease in patients with systemic sclerosis?. Autoimmun Rev 2015, 14:575-578. 10.1016/j.autrev.2015.02.002.
65. Kowal-Bielecka O., Landewe R., Avouac J., Chwiesko S., Miniati I., Czirjak L., 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, 68:620-628. 10.1136/ard.2008.096677.
66. Thompson A.E., Shea B., Welch V., Fenlon D., Pope J.E. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum 2001, 44:1841-1847. 10.1002/1529-0131(200108)44:8<1841::AID-ART322>3.0.CO;2-8.
67. Pope J., Fenlon D., Thompson A., Shea B., Furst D., Wells G., et al. Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev 2000, 10.1002/14651858.CD000953.
68. Scorza R., Caronni M., Mascagni B., Berruti V., Bazzi S., Micallef E., et al. Effects of long-term cyclic iloprost therapy in systemic sclerosis with Raynaud's phenomenon. A randomized, controlled study. Clin Exp Rheumatol 2001, 19:503-508.
69. Rademaker M., Cooke E.D., Almond N.E., Beacham J.A., Smith R.E., Mant T.G., et al. Comparison of intravenous infusions of iloprost and oral nifedipine in treatment of Raynaud's phenomenon in patients with systemic sclerosis: a double blind randomised study. BMJ 1989, 298:561-564.
70. Roustit M., Blaise S., Allanore Y., Carpentier P.H., Caglayan E., Cracowski J.-L. Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud's phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis 2013, 72:1696-1699. 10.1136/annrheumdis-2012-202836.
71. Hughes M., Ong V.H., Anderson M.E., Hall F., Moinzadeh P., Griffiths B., et al. Consensus best practice pathway of the UK Systemic Sclerosis Study Group: digital vasculopathy in systemic sclerosis. Rheumatology 2015, 10.1093/rheumatology/kev201.
72. Wigley F.M., Seibold J.R., Wise R.A., McCloskey D.A., Dole W.P. Intravenous iloprost treatment of Raynaud's phenomenon and ischemic ulcers secondary to systemic sclerosis. J Rheumatol 1992, 19:1407-1414.
73. Korn J.H., Mayes M., Matucci Cerinic M., Rainisio M., Pope J., Hachulla E., et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004, 50:3985-3993. 10.1002/art.20676.
74. Matucci-Cerinic M., Denton C.P., Furst D.E., Mayes M.D., Hsu V.M., Carpentier P., et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 2011, 70:32-38. 10.1136/ard.2010.130658.
75. Tingey T., Shu J., Smuczek J., Pope J. Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res 2013, 65:1460-1471. 10.1002/acr.22018.
76. Hachulla E., Hatron P.-Y., Carpentier P., Agard C., Chatelus E., Jego P., et al. Efficacy of sildenafil on ischaemic digital ulcer healing in systemic sclerosis: the placebo-controlled SEDUCE study. Ann Rheum Dis 2015, 10.1136/annrheumdis-2014-207001.
77. Fernandes das Neves M., Oliveira S., MC A., J.D.A. Treatment of systemic sclerosis with tocilizumab. Rheumatology 2015, 54:371-372. 10.1093/rheumatology/keu435.
78. Steen V.D., Costantino J.P., Shapiro A.P., Medsger T.A. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 1990, 113:352-357.
79. Steen V.D. Kidney involvement in systemic sclerosis. Presse Med 2014, 43:e305-e314. 10.1016/j.lpm.2014.02.031.
80. Hudson M., Baron M., Tatibouet S., Furst D.E., Khanna D. Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis-results from the International Scleroderma Renal Crisis Survey. Semin Arthritis Rheum 2014, 43:666-672. 10.1016/j.semarthrit.2013.09.008.
81. Sanges S., Launay D., Rhee R.L., Sitbon O., Hachulla É., Mouthon L., et al. A prospective study of the 6 min walk test as a surrogate marker for haemodynamics in two independent cohorts of treatment-naïve systemic sclerosis-associated pulmonary arterial hypertension. Ann Rheum Dis 2015, 2015-207336. (annrheumdis). 10.1136/annrheumdis-2015-207336.
82. Girgis R.E., Mathai S.C., Krishnan J.A., Wigley F.M., Hassoun P.M. Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant 2005, 24:1626-1631.
83. Pulido T., Adzerikho I., Channick R.N., Delcroix M., Galiè N., Ghofrani H.-A., et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013, 369:809-818. 10.1056/NEJMoa1213917.
84. Badesch D.B., Tapson V.F., McGoon M.D., Brundage B.H., Rubin L.J., Wigley F.M., et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000, 132:425-434.
85. Galiè N., Corris P.A., Frost A., Girgis R.E., Granton J., Jing Z.C., et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 2013, 62:D60-D72. 10.1016/j.jacc.2013.10.031.
86. Tashkin D.P., Elashoff R., Clements P.J., Goldin J., Roth M.D., Furst D.E., et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006, 354:2655-2666.
87. Hoyles R.K., Ellis R.W., Wellsbury J., Lees B., Newlands P., Goh N.S.L., 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, 54:3962-3970. 10.1002/art.22204.
88. Nannini C., West C.P., Erwin P.J., Matteson E.L. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 2008, 10:R124.
89. Yilmaz N., Can M., Kocakaya D., Karakurt S., Yavuz S. Two-year experience with mycophenolate mofetil in patients with scleroderma lung disease: a case series. Int J Rheum Dis 2014, 17:923-928. 10.1111/1756-185X.12399.
90. Koutroumpas A., Ziogas A., Alexiou I., Barouta G., Sakkas L.I. Mycophenolate mofetil in systemic sclerosis-associated interstitial lung disease. Clin Rheumatol 2010, 29:1167-1168. 10.1007/s10067-010-1498-z.
91. Keir G.J., Maher T.M., Ming D., Abdullah R., de Lauretis A., Wickremasinghe M., et al. Rituximab in severe, treatment-refractory interstitial lung disease. Respirol Carlton Vic 2014, 19:353-359. 10.1111/resp.12214.
92. Daoussis D., Liossis S.-N.C., Tsamandas A.C., Kalogeropoulou C., Paliogianni F., Sirinian C., et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol 2012, 30:S17-S22.
93. Giuggioli D., Lumetti F., Colaci M., Fallahi P., Antonelli A., Ferri C. Rituximab in the treatment of patients with systemic sclerosis. Our experience and review of the literature. Autoimmun Rev 2015, 14:1072-1078. 10.1016/j.autrev.2015.07.008.
94. Jordan S., Maurer B., Toniolo M., Michel B., Distler O. Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice. Rheumatology 2015, 54:1454-1458. 10.1093/rheumatology/keu530.
95. Khanna D., Saggar R., Mayes M.D., Abtin F., Clements P.J., Maranian P., et al. A one-year, phase I/IIa, open-label pilot trial of imatinib mesylate in the treatment of systemic sclerosis-associated active interstitial lung disease. Arthritis Rheum 2011, 63:3540-3546. 10.1002/art.30548.
96. Fraticelli P., Gabrielli B., Pomponio G., Valentini G., Bosello S., Riboldi P., 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:R144.
97. Le E.N., Wigley F.M., Shah A.A., Boin F., Hummers L.K. Long-term experience of mycophenolate mofetil for treatment of diffuse cutaneous systemic sclerosis. Ann Rheum Dis 2011, 70:1104-1107. 10.1136/ard.2010.142000.
98. Mendoza F.A., Nagle S.J., Lee J.B., Jimenez S.A. A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol 2012, 39:1241-1247. 10.3899/jrheum.111229.
99. Poelman C.L., Hummers L.K., Wigley F.M., Anderson C., Boin F., Shah A.A. Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis. J Rheumatol 2015, 42:236-242. 10.3899/jrheum.140833.
100. 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, 31:151-156.
101. Bosello S.L., De Luca G., Rucco M., Berardi G., Falcione M., Danza F.M., et al. Long-term efficacy of B cell depletion therapy on lung and skin involvement in diffuse systemic sclerosis. Semin Arthritis Rheum 2015, 44:428-436. 10.1016/j.semarthrit.2014.09.002.
102. Huang J., Beyer C., Palumbo-Zerr K., Zhang Y., Ramming A., Distler A., et al. Nintedanib inhibits fibroblast activation and ameliorates fibrosis in preclinical models of systemic sclerosis. Ann Rheum Dis 2015, 10.1136/annrheumdis-2014-207109.
103. Gordon J.K., Martyanov V., Magro C., Wildman H.F., Wood T.A., Huang W.-T., et al. Nilotinib (Tasigna™) in the treatment of early diffuse systemic sclerosis: an open-label, pilot clinical trial. Arthritis Res Ther 2015, 17. 10.1186/s13075-015-0721-3.
104. Rice L.M., Padilla C.M., McLaughlin S.R., Mathes A., Ziemek J., Goummih S., et al. Fresolimumab treatment decreases biomarkers and improves clinical symptoms in systemic sclerosis patients. J Clin Invest 2015, 125:2795-2807. 10.1172/JCI77958.
105. Burt R.K., Shah S.J., Dill K., Grant T., Gheorghiade M., Schroeder J., et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet Lond Engl 2011, 378:498-506. 10.1016/S0140-6736(11)60982-3.
106. Van Laar J.M., Farge D., Sont J.K., Naraghi K., Marjanovic Z., Larghero J., et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 2014, 311:2490-2498. 10.1001/jama.2014.6368.
107. Mierau R., Moinzadeh P., Riemekasten G., Melchers I., Meurer M., Reichenberger F., et al. Frequency of disease-associated and other nuclear autoantibodies in patients of the German Network for Systemic Scleroderma: correlation with characteristic clinical features. Arthritis Res Ther 2011, 13:R172.
108. LeRoy E.C., Black C., Fleischmajer R., Jablonska S., Krieg T., Medsger T.A., Rowell N., Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. Journal Of Rheumatology 1988, 15(2):202-205.