Treatment options in systemic sclerosis

Bibliography

• Allanore Y, Kahan A. Treatment of systemic sclerosis. Joint Bone Spine 2006;73:363-8
   PubMed Web of Science ®Google Scholar
• Kowal-Bielecka O, Landewé 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;68:620-8
   PubMed Web of Science ®Google Scholar
• Allanore Y, Borderie D, Lemarechal H, et al. Acute and sustained effects of dihydropyridine-type calcium channel antagonists on oxidative stress in systemic sclerosis. Am J Med 2004;116:595-600
   PubMed Web of Science ®Google Scholar
• Thompson AE, Shea B, Welch V, et al. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum 2001;44:1841-7
   PubMed Web of Science ®Google Scholar
• Rademaker M, Cooke ED, Almond NE, 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;298:561-4
   PubMed Web of Science ®Google Scholar
• Scorza R, Caronni M, Mascagni B, et al. Effects of long-term cyclic iloprost therapy in systemic sclerosis with Raynaud's phenomenon. A randomized, controlled study. Clin Exp Rheumatol 2001;19:503-8
   PubMed Web of Science ®Google Scholar
• Stratton R, Shiwen X, Martini G, et al. Iloprost suppresses connective tissue growth factor production in fibroblasts and in the skin of scleroderma patients. J Clin Invest 2000;108:241-50
   Web of Science ®Google Scholar
• Bettoni L, Geri A, Airo P, et al. Systemic sclerosis therapy with iloprost: a prospective observational study of 30 patients treated for a median of 3 years. Clin Rheumatol 2002;21:244-50
   PubMed Web of Science ®Google Scholar
• Biasi D, Carletto A, Caramaschi P, et al. Iloprost as cyclic five-day infusions in the treatment of scleroderma. An open pilot study in 20 patients treated for one year. Rev Rhum Engl Ed 1998;65:745-50
   PubMedGoogle Scholar
• Ceru S, Pancera P, Sansone S, et al. Effects of five-day versus one-day infusion of iloprost on the peripheral microcirculation in patients with systemic sclerosis. Clin Exp Rheumatol 1997;15:381-5
   PubMed Web of Science ®Google Scholar
• Belch JJ, Capell HA, Cooke ED, et al. Oral iloprost as a treatment for Raynaud's syndrome: a double blind multicentre placebo controlled study. Ann Rheum Dis 1995;54:197-200
   PubMed Web of Science ®Google Scholar
• Kyle MV, Belcher G, Hazleman BL. Placebo controlled study showing therapeutic benefit of iloprost in the treatment of Raynaud's phenomenon. J Rheumatol 1992;19:1403-6
   PubMed Web of Science ®Google Scholar
• Lau CS, Belch JJ, Madhok R, et al. A randomised, double-blind study of cicaprost, an oral prostacyclin analogue, in the treatment of Raynaud's phenomenon secondary to systemic sclerosis. Clin Exp Rheumatol 1993;11:35-40
   PubMed Web of Science ®Google Scholar
• McHugh NJ, Csuka M, Watson H, et al. Infusion of iloprost, a prostacyclin analogue, for treatment of Raynaud's phenomenon in systemic sclerosis. Ann Rheum Dis 1988;47:43-7
   PubMed Web of Science ®Google Scholar
• Wigley FM, Wise RA, Seibold JR, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med 1994;120:199-206
   PubMed Web of Science ®Google Scholar
• Yardumian DA, Isenberg DA, Rustin M, et al. Successful treatment of Raynaud's syndrome with iloprost, a chemically stable prostacyclin analogue. Br J Rheumatol 1988;27:220-6
   PubMedGoogle Scholar
• Pope J, Fenlon D, Thompson A, et al. Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev 1998(2):CD000953
   Google Scholar
• Kawald A, Burmester GR, Huscher D, et al. Low versus high-dose iloprost therapy over 21 days in patients with secondary Raynaud's phenomenon and systemic sclerosis: a randomized, open, single-center study. J Rheumatol 2008;35:1830-7
   PubMed Web of Science ®Google Scholar
• Wigley FM, Korn JH, Csuka ME, et al. Oral iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis: a multicenter, placebo-controlled, double-blind study. Arthritis Rheum 1998;41:670-7
   PubMed Web of Science ®Google Scholar
• Black CM, Halkier SA, Rensen L, et al. Oral iloprost in Raynaud's phenomenon secondary to systemic sclerosis: a multicentre, placebocontrolled, dose-comparison study. Br J Rheumatol 1998;37:952-60
   PubMedGoogle Scholar
• Seibold JR, Wigley FM, Schiopu E, et al. Digital ischemic ulcers in scleroderma treated with oral treprostinil diethanolamine: a randomized, double-blind, placebo-controlled, multicenter study. Arthritis Rheum 2010;63(Suppl):S968-9
   Google Scholar
• Chung L, Fiorentino D. A pilot trial of treprostinil for the treatment and prevention of digital ulcers in patientswith systemic sclerosis. J Am Acad Dermatol 2006;54:880-2
   PubMed Web of Science ®Google Scholar
• Hettema ME, Zhang D, Bootsma H, Kallenberg CGM. Bosentan therapy for patients with severe Raynaud's phenomenon in systemic sclerosis. Ann Rheum Dis 2007;66:1398-9
   PubMed Web of Science ®Google Scholar
• Ramos-Casals M, Brito-Zeron P, Nardi N, et al. Successful treatment of severe Raynaud's phenomenon with bosentan in four patients with systemic sclerosis. Rheumatology 2004;43:1454-6
   PubMed Web of Science ®Google Scholar
• Hettema ME, Zhang D, Bootsma H, Kallenberg CGM. Successful treatment of patients with severe secondary Raynaud's phenomenon with the endothelin receptor antagonist bosentan. Rheumatology 2006;45:iii45-i4
   PubMed Web of Science ®Google Scholar
• Garcia de la Pena-Lefebvre P, Rodriguez Rubio S, Valero Exposito M, et al. Long-term experience of bosentan for treating ulcers and healed ulcers in systemic sclerosis patients. Rheumatology 2008;4:464-6
   PubMed Web of Science ®Google Scholar
• Seibold JR, M Matucci-Cerinic M, Denton CP, et al. Bosentan reduces the number of new digital ulcers in patients with systemic sclerosis. Ann Rheum Dis 2006;65(Suppl II):90
   Google Scholar
• Korn JH, Mayes M, Matucci Cerinic M, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004;50:3985-93
   PubMed Web of Science ®Google Scholar
• Matucci-Cerinic M, Denton CP, Furst DE, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 2011;70:32-8
   PubMed Web of Science ®Google Scholar
• Launay D, Diot E, Pasquier E, et al. Bosentan for treatment of active digital ulcers in patients with systemic sclerosis. Presse Med 2006;35:587-92
   PubMed Web of Science ®Google Scholar
• Humbert M, Cabane J. Successful treatment of systemic sclerosis digital ulcers and pulmonary arterial hypertension with endothelin receptor antagonist bosentan. Rheumatology 2003;42:191-3
   PubMed Web of Science ®Google Scholar
• Matucci-Cerinic M, Seibold Jr. Digital ulcers and outcomes assessment in scleroderma. Rheumatology (Oxford) 2008;47: (Suppl 5):v46-7
   Web of Science ®Google Scholar
• De LaVega J, Derk CT. Phosphodiesterase-5 inhibitors for the treatment of Raynaud's: a novel indication. Expert Opin Investig Drugs 2009;18:23-9
   PubMed Web of Science ®Google Scholar
• Badesch DB, Abman SH, Simonneau G, et al. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest 2007;131:1917-28
   PubMed Web of Science ®Google Scholar
• Sharma R. Novel phosphodiesterase-5 inhibitors: current indications and future directions. Indian J Med Sci 2007;61:667-79
   PubMedGoogle Scholar
• Fries R, Shariat K, von Wilmowsky H, Böhm M. Sildenafil in the treatment of raynaud's phenomenon resistant to vasodilatory therapy. Circulation 2005;112:2980-5
   PubMed Web of Science ®Google Scholar
• Herrick A, Gabrielli A, Tamimi N, et al. Modified-release sildenafil reduces Raynaud's phenomenon attack frequency in limited cutaneous systemic sclerosis. Arthritis Rheum 2011;63:775-82
   PubMed Web of Science ®Google Scholar
• Brueckner CS, Becker MO, Kroencke T, et al. Effect of sildenafil on digital ulcers in systemic sclerosis: analysis from a single centre pilot study. Ann Rheum Dis 2010;69:1475-8
   PubMed Web of Science ®Google Scholar
• Kumar U, Gokhle SS, Sreenivas V, et al. Prospective, open-label, uncontrolled pilot study to study safety and efficacy of sildenafil in systemic sclerosis–related pulmonary artery hypertension and cutaneous vascular complications. Rheumatol Int 2013;33:1047-52
   PubMed Web of Science ®Google Scholar
• Moinzadeh P, Hunzelmann N, Kreig T. Combination therapy with an endothelin-1 receptor antagonist (bosentan) and a phosphodiesterase V inhibitor (sildenafil) for the management of severe digital ulcerations in systemic sclerosis. J Am Acad Dermatol 2011;65:e102-4
   Google Scholar
• Schiopu E, Hsu VM, Impens AJ, et al. Randomized placebo-controlled crossover trial of tadalafil in Raynaud's phenomenon secondary to systemic sclerosis. J Rheumatol 2009;36:2264-8
   PubMed Web of Science ®Google Scholar
• Shenoy PD, Kumar S, Jha LK, et al. Efficacy of tadalafil in secondary Raynaud's phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatology 2010;49:2420-8
   PubMed Web of Science ®Google Scholar
• Teh LS, Manning J, Moore T, et al. Sustained-release transdermal glyceryl trinitrate patches as a treatment for primary and secondary Raynaud's phenomenon. Br J Rheumatol 1995;34:636-41
   PubMedGoogle Scholar
• Galluccio F, Matucci Cerinic M. Two faces of the same coin: raynaud phenomenon and digital ulcers in systemic sclerosis. Autoimmun Rev 2011;10:241-3
   PubMed Web of Science ®Google Scholar
• Yoshimasu T, Ikeda T, Uede K, et al. Effects of sarpogrelate hydrochloride on skin ulcers and quality of life in patients with systemic sclerosis. J Dermatol 2012;39:536-40
   PubMed Web of Science ®Google Scholar
• Dziadzio M, Denton CP, Smith R, et al. Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial. Arthritis Rheum 1999;42:2646-55
   PubMed Web of Science ®Google Scholar
• Herrick AL. Management of raynaud's phenomenon and digital ischemia. Curr Rheumatol Rep 2013;15:303
   PubMed Web of Science ®Google Scholar
• Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 2010;138:1383-94
   PubMed Web of Science ®Google Scholar
• Coghlan JG, Denton CP, Grünig E, et al. on behalf of the DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2013; Epub ahead of print
   PubMed Web of Science ®Google Scholar
• Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension Arthritis Rheum. 2013; doi: 10.1002/art.38172; Epub ahead of print
   Google Scholar
• Hachulla E, Denton CP. Early intervention in pulmonary arterial hypertension associated with systemic sclerosis: an essential component of disease management. Eur Respir Rev 2010;19:314-20
   PubMedGoogle Scholar
• McGoon MD, Krichman A, Farber HW, et al. Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc 2008;83:923-31
   PubMed Web of Science ®Google Scholar
• Galiè N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS); International Society of Heart and Lung Transplantation (ISHLT). Eur Respir J 2009;34:1219-63
   PubMed Web of Science ®Google Scholar
• Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001;358:1119-23
   PubMed Web of Science ®Google Scholar
• Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346:896-903
   PubMed Web of Science ®Google Scholar
• Denton CP, Pope JE, Peter HH, et al. Tracleer Use in PAH associated with Scleroderma and Connective Tissue Diseases (TRUST) Investigators. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis 2008;67:1222-8
   PubMed Web of Science ®Google Scholar
• Denton CP, Humbert M, Rubin L, Black CM. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 2006;65:1336-40
   PubMed Web of Science ®Google Scholar
• Launay D, Sitbon O, Le Pavec J, et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil. Rheumatology (Oxford) 2010;49:490-500
   PubMed Web of Science ®Google Scholar
• Kovacs G, Maier R, Aberer E, et al. Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure. Arthritis Rheum 2012;64:1257-62
   PubMed Web of Science ®Google Scholar
• ` GalieN, Olschewski H, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008;117:3010-19
   PubMed Web of Science ®Google Scholar
• Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148-57
   PubMed Web of Science ®Google Scholar
• Liu C, Chen J. Endothelin receptor antagonists for pulmonary arterial hypertension. Cochrane Database Syst Rev 2006;3:CD004434
   Google Scholar
• Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119:2894-903
   PubMed Web of Science ®Google Scholar
• Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000;21:425-34
   Web of Science ®Google Scholar
• Tapson VF, Gomberg-Maitland M, McLaughlin VV, et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest 2006;129:683-8
   PubMed Web of Science ®Google Scholar
• Sitbon O, Manes A, Jais X, et al. Rapid switch from intravenous epoprostenol to intravenous treprostinil in patients with pulmonary arterial hypertension. J Cardiovasc Pharmacol 2007;49:1-5
   PubMed Web of Science ®Google Scholar
• Oudiz RJ, Farber HW. Dosing considerations in the use of intravenous prostanoids in pulmonary arterial hypertension: an experience-based review. Am Heart J 2009;157:625-35
   PubMed Web of Science ®Google Scholar
• Sun YJ, Xiong CM, Shan GL, et al. Iloprost Therapy on Pulmonary Hypertension Study Group. Inhaled low-dose iloprost for pulmonary hypertension: a prospective, multicenter, open-label study. Clin Cardiol 2012;35:365-70
   PubMed Web of Science ®Google Scholar
• Olschewski H, Simonneau G, Galie N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002;347:322-9
   PubMed Web of Science ®Google Scholar
• Johnson SR, Brode SK, Mielniczuk LM, Granton JT. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med 2012;106:730e739
   Web of Science ®Google Scholar
• Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 1990;113:352-7
   PubMed Web of Science ®Google Scholar
• Steen VD, Medsger TA Jr. Long-term outcomes of scleroderma renal crisis. Ann Intern Med 2000;133:600-3
   PubMed Web of Science ®Google Scholar
• Chiba N, De Gara CJ, Wilkinson JM, Hunt RH. Speed of healing and symptom relief in grade II to IV gastroesophageal reflux disease: a meta-analysis. Gastroenterology 1997;112:1798-810
   PubMed Web of Science ®Google Scholar
• Sandqvist G, Akesson A, Eklund M. Evaluation of paraffin bath treatment in patients with systemic sclerosis. Disabil Rehabil 2004;26(16):981-7
   PubMed Web of Science ®Google Scholar
• Randone SB, Guiducci S, Cerinic MM. Musculoskeletal involvement in systemic sclerosis. Best Pract Res Clin Rheumatol 2008;22:339-50
   PubMed Web of Science ®Google Scholar
• Daoussis D, Antonopoulos I, Liossis SNC, et al. Treatment of systemic sclerosis-associated calcinosis: a case report of rituximab-induced regression of CREST-related calcinosis and review of the literature. Semin Arthritis Rheum 2012;41:822-9
   PubMed Web of Science ®Google Scholar
• Guillevin L, Cordier JF, Lhote F, et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum 1997;40:2187-98
   PubMed Web of Science ®Google Scholar
• Miniati I, Conforti ML, Guiducci S, Matucci Cerinic M. David against Goliath: may we target and defeat interstial lung disease systemic sclerosis. Clin Exp Rheumtol 2007;25:167-71
   Web of Science ®Google Scholar
• Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354:2655-66
   PubMed Web of Science ®Google Scholar
• 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 Resp Crit Care Med 2007;76:1026-34
   Web of Science ®Google Scholar
• 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. Arhtrit Rheum 2006;54:3962-70
   PubMed Web of Science ®Google Scholar
• Nadashkevich O, Davis P, Fritzler M, Kovalenko W. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheum 2006;25:205-12
   PubMed Web of Science ®Google Scholar
• Nannini C, West CP, Erwin PJ, Matteson EL. 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. Arhtrit Res Ther 2008;10:R124
   Web of Science ®Google Scholar
• Van den Hoogen FH, Boerbooms AM, Swaak AJ, et al. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 1996;35:364-72
   PubMedGoogle Scholar
• Pope JE, Bellamy N, Seibold JR, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 2001;44:1351-8
   PubMed Web of Science ®Google Scholar
• Walker KM, Pope J; participating members of the Scleroderma Clinical Trials Consortium (SCTC) and Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails - a consensus of systemic sclerosis experts. Semin Arthrit Rheum 2012;42:42-55
   PubMed Web of Science ®Google Scholar
• Vanthuyne M, Blockmans D, Westhovens R, et al. A pilot study of mycophenolate mofetil combined to intravenous methylprednisolone pulses and oral low-dose glucocorticoids in severe early systemic sclerosis. Clin Exp Rheumatol 2007;25:287-92
   PubMed Web of Science ®Google Scholar
• Derk CT, Grace E, Shenin M, et al. A prospective open-label study of mycophenolate mofetil for the treatment of diffuse systemic sclerosis. Rheumatology 2009;48:1595-9
   PubMed Web of Science ®Google Scholar
• Herrick AL, Lunt M, Whidby N, et al. Observational study of treatment outcome in early diffuse cutaneous systemic sclerosis. J Rheumatol 2010;37:116-24
   PubMed Web of Science ®Google Scholar
• Koutroumpas A, Ziogas A, Alexiou et al. Mycophenolate mofetil in systemic sclerosis-associated interstitial lung disease. Clin Rheumatol 2010;29:1167-8
   PubMed Web of Science ®Google Scholar
• Nihtyanova SI, Brough GM, Black CM, Denton CP. Mycophenolate mofetil in diffuse cutaneous systemic sclerosis-a retrospective analysis. Rheumatology (Oxford) 2007;46:442-5
   PubMed Web of Science ®Google Scholar
• Gerbino AJ, Goss CH, Molitor JA. Effect of mycophenolate mofetil on pulmonary function in Scleroderma-associated interstitial lung disease. Chest 2008;133:455-60
   PubMed Web of Science ®Google Scholar
• Liossis SNC, Bounas A, Anonopoulos AP. Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease. Rheumatology 2006;45:1005-8
   PubMed Web of Science ®Google Scholar
• Zamora AC, Wolters PJ, Collard HR, et al. Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Resp Med 2008;102:150-5
   PubMed Web of Science ®Google Scholar
• Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, et al. Effect of mycophenolate sodium in scleroderma-related interstitial lung disease. Clin Rheumatol 2011;30:1393-8
   PubMed Web of Science ®Google Scholar
• 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;25:613-16
   PubMed Web of Science ®Google Scholar
• Bérezné 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. Rheumatology 2008;35(6):1064-72
   Web of Science ®Google Scholar
• Griffiths B, Miles S, Moss H, et al. Systemic sclerosis and interstitial lung disease: a pilot study using pulse intravenous methylprednisolone and cyclophosphamide to assess the effect on high resolution computed tomography scan and lung function. J Rheumatol 2002;29:2371-8
   PubMed Web of Science ®Google Scholar
• Yiannopoulos G, Pastromas V, Antonopoulos I, et al. Combination of intravenous pulses of cyclophosphamide and methylprednisolone in patients with systemic sclerosis and interstitial lung disease. Rheumatol Int 2007;27:357-61
   PubMed Web of Science ®Google Scholar
• Teixeira L, Mouthon L, Mahr A, et al. for the Groupe Français de Recherche sur la Sclérodermie. Scleroderma renal crisis: presentation, outcome and risk factors based on retrospective multicenter study of 50 patients. Ann Rheum Dis 2006;54(Suppl):S 743
   Google Scholar
• DeMarco PJ, Weisman MH, Seibold JR, et al. Predictors and outcomes of scleroderma renal crisis: the high dose versus low-dose of penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum 2002;46:2983-9
   PubMed Web of Science ®Google Scholar
• Helfrich DJ, Banner B, Steen VD, Medsger TA Jr. Normotensive renal failure in systemic sclerosis. Arthritis Rheum 1989;32:1128-34
   PubMed Web of Science ®Google Scholar
• Bosello S, De Santis M, Lama G, et al. B cell depletion in diffuse progressive systemic sclerosis: safety, skin score modification and IL-6 modulation in an up to thirty-six months follow-up open-label trial. Arthritis Res Ther 2010;12:R54
   PubMed Web of Science ®Google Scholar
• Smith V, Van Praet JT, Vandooren B, et al. Rituximab in diffuse cutaneous systemic sclerosis: an open label clinical and histopatological study. Ann Rheum Dis 2010;69:193-7
   PubMed Web of Science ®Google Scholar
• Layaftis R, Kissin E, York M, et al. B cell depletion with rituximab in patients with diffuse cutaneous systemic sclerosis. Arthritis Rheum 2009;60:578-83
   PubMed Web of Science ®Google Scholar
• Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology 2010;49:271-80
   PubMed Web of Science ®Google Scholar
• Daoussis D, Liossis SN, Tsmandas AC, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheum 2012;30:17-22
   PubMed Web of Science ®Google Scholar
• 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 2012; Epub ahead of print
   Web of Science ®Google Scholar
• Shima Y, Kuwahara Y, Murota H, et al. The skin of patients with systemic sclerosis softened during the treatment with anti-IL-6 receptor antibody tocilizumab. Rheumatology 2010;49:2408-12
   PubMed Web of Science ®Google Scholar
• Mauviel A, Daireaux M, Rédini F, et al. Tumor necrosis factor inhibits collagen and fibronectin synthesis in human dermal fibroblasts. FEBS Lett 1988;236:47-52
   PubMed Web of Science ®Google Scholar
• Mauviel A, Heino J, Kähäri VM, et al. Comparative effects of interleukin-1 and tumor necrosis factor-alpha on collagen production and corresponding procollagen mRNA levels in human dermal fibroblasts. J Invest Dermatol 1991;96:243-9
   PubMed Web of Science ®Google Scholar
• Piguet PF, Collart MA, Grau GE, et al. Requirement of tumour necrosis factor for development of silica-induced pulmonary fibrosis. Nature 1990;344:245-7
   PubMed Web of Science ®Google Scholar
• Piguet PF, Vesin C. Treatment by human recombinant soluble TNF receptor of pulmonary fibrosis induced by bleomycin or silica in mice. Eur Respir J 1994;7:515-18
   PubMed Web of Science ®Google Scholar
• Lam GK, Hummers LK, Woods A, Wigley FM. Efficacy and safety of etanercept in the treatment of scleroderma-associated joint disease. J Rheumatol 2007;34:1636-7
   PubMed Web of Science ®Google Scholar
• Denton CP, Engelhart M, Tvede N, et al. An open label pilot study of infliximab in diffuse cutaneous systemic sclerosis. Ann Rheum Dis 2009;68:1433-9
   PubMed Web of Science ®Google Scholar
• Ellman MH, MacDonald PA, Hayes FA. Etanercept as treatment for diffuse scleroderma. A pilot study [abstract]. Arthritis Rheum 2000;43(Suppl):392
   Google Scholar
• Bosello S, De Santis M, Tolusso B, et al. Tumor necrosis factor-alfa inhibitor therapy in erosive polyarthritis secondary to systemic sclerosis. Ann Intern Med 2005;143:918-20
   PubMed Web of Science ®Google Scholar
• Distler JH, Jordan S, Airo P, et al. Is there a role for TNFα antagonists in the treatment of SSc? EUSTAR expert consensus development using the Delphi technique. Clin Exp Rheumatol 2011;29:40-5
   PubMed Web of Science ®Google Scholar
• Denton CP, Merkel PA, Furst DE, et al. Recombinant Human anti-transforming growth factor β1 antibody therapy in systemic sclerosis a multicenter, randomized, placebo-controlled phase I/II trial of Cat-192. Arthritis Rheum 2007;56:323-33
   PubMed Web of Science ®Google Scholar
• 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;70:1003-9
   PubMed Web of Science ®Google Scholar
• Khanna D, Saggar R, Mayes MD, 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;63:3540-6
   PubMed Web of Science ®Google Scholar
• 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;63:3547-51
   PubMed Web of Science ®Google Scholar
• Levy Y, Amital H, Langevitz P, et al. Intravenous immunoglobulin modulates cutaneous involvement and reduces skin fibrosis in systemic sclerosis: an open-label study. Arthritis Rheum 2004;50:1005-7
   PubMed Web of Science ®Google Scholar
• Amital H, Rewald E, Levy Y, et al. Fibrosis regression induced by intravenous gammaglobulin treatment. Ann Rheum Dis 2003;6:175-7
   Web of Science ®Google Scholar
• Nacci F, Righi A, Conforti ML, et al. Intravenous immunoglobulins improve the function and ameliorate joint involvement in systemic sclerosis: a pilot study. Ann Rheum Dis 2007;6:977-9
   Web of Science ®Google Scholar
• Levy JB, Pusey CD. Nephrotoxicity of intravenous immunoglobulin. QJM 2000;93:751-5
   PubMed Web of Science ®Google Scholar
• Binks M, Passweg JR, Furst D, et al. Phase I/II trial of autologous stem cell transplantation in systemic sclerosis: procedure related mortality and impact on skin disease. Ann Rheum Dis 2001;60:577-84
   PubMed Web of Science ®Google Scholar
• Saccardi R, Tyndall A, Coghlan G, et al. Consensus statement concerning cardiotoxicity occurring during haematopoietic stem cell transplantation in the treatment of autoimmune diseases, with special reference to systemic sclerosis and multiple sclerosis. Bone Marrow Transplant 2004;34:877-88
   PubMedGoogle Scholar
• Farge D, Passweg J, van Laar JM, et al. Autologous stem cell transplantation in the treatment of systemic sclerosis: report from the EBMT/EULAR registry. Ann Rheum Dis 2004;63:974-81
   PubMed Web of Science ®Google Scholar
• Bournia VKK, Vlachoyiannopoulos PG, Selmi C, et al. Recent advances in the treatment of systemic sclerosis. Clinic Rev Allerg Immunol 2009;36:176-200
   PubMed Web of Science ®Google Scholar
• Naraghi K, van Laar JM. Update on stem cell transplantation for systemic sclerosis: recent trial results. Curr Rheumatol Rep 2013;15:326
   PubMed Web of Science ®Google Scholar
• Henes JC, Schmalzing M, Vogel W, et al. Optimization of autologous stem cell transplantation for systemic sclerosis – a single-center long term experience in 26 patients with severe organ manifestations. J Rheumatol 2012;39:269-75
   PubMed Web of Science ®Google Scholar
• Vonk MC, Marjanovic Z, van den Hoogen FH, et al. Longterm follow-up results after autologous haematopoietic stem cell transplantation for severe systemic sclerosis. Ann Rheum Dis 2008;67:98-104
   PubMed Web of Science ®Google Scholar
• Burt RK, Shah SJ, Dill K, 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 2011;378:498-506
   PubMed Web of Science ®Google Scholar
• Van Laar JM, Farge D, Sont JK, et al. The ASTIS trial: autologous stem cell transplantation versus IV pulse cyclophosphamide in poor prognosis systemic sclerosis, first results. EULAR, Annual Congress of the European League Against Rheumatism. Ann Rheum Dis 2012;71(Suppl 3):151
   Web of Science ®Google Scholar
• Farge D, van Laar JM, Sont JK, et al. Autologous hematopoietic stem cell transplantation versus intravenous pulse therapy cyclophosphamide for severe or rapidly progressive systemic sclerosis, the ASTIS Trial. Blood (ASH Ann Meet Abstr) 2012;120:964
   Google Scholar
• Clinical Trials.gov: scleroderma: Cyclophosphamide or Transplantation (SCOT). Available from: http://www.clinicaltrials.gov/ct2/show/NCT00114530?term=SCOT+trial&rank=6 [Accessed January 2013]
   Google Scholar
• Saggar R, Khanna D, Furst DE, et al. Systemic sclerosis and bilateral lung transplantation: a single centre experience. Eur Respir J 2010;36:893-900
   PubMed Web of Science ®Google Scholar
• Knobler RM, French LE, Kim Y, et al. Systemic sclerosis study, A randomized, double blind, placebo controlled trial of photophoresis in systemic sclerosis. J Am Acad Dermatol 2006;54:793-9
   PubMed Web of Science ®Google Scholar
• Enomoto DN, Mekkes JR, Bossuyt PM, et al. Treatment of patients with Systemic sclerosis with extracorporeal photochemotherapy (photophoresis). J Am Acad Dermatol 1999;41:9122-59
   Web of Science ®Google Scholar
• Schachna L, Medsger TA Jr, Dauber JH, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006;54:3954-61
   PubMed Web of Science ®Google Scholar
• Martens E, Lange P, Pohl T, et al. Heart transplantation in a 36-year-old experiencing terminal heart failure caused by systemic sclerosis. Transplantation 2012;94:e13-15
   Web of Science ®Google Scholar
• Quartier P, Bonnet D, Fournet JC, et al. Severe cardiac involvement in children with systemic sclerosis and myositis. J Rheumatol 2002;29:1767-73
   PubMed Web of Science ®Google Scholar
• Gibney EM, Parikh CR, Jani A, et al. Kidney transplantation for systemic sclerosis improves survival and may modulate disease activity. Am J Transplant 2004;4:2027-31
   PubMed Web of Science ®Google Scholar
• Woodhall PB, McCoy RC, Gunnells JC, Seigler HF. Apparent recurrence of progressive systemic sclerosis in a renal allograft. JAMA 1976;236:1032-4
   PubMed Web of Science ®Google Scholar
• Caplin NJ, Dikman S, Winston J, et al. Recurrence of scleroderma in a renal allograft from an identical twinsister. Am J Kidney Dis 1999;33:e7
   Google Scholar
• Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 1990;113:352-7
   PubMed Web of Science ®Google Scholar