Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Rheumatic Disease Clinics of North America

Volumn 41, Issue 3, 2015, Pages 489-506

  Valenzuela, Antonia ^a   Nandagopal, Saranya ^a   Steen, Virginia D ^b   Chung, Lorinda ^a  

^a STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b GEORGETOWN UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Diagnosis; Monitoring; Pulmonary arterial hypertension; Systemic sclerosis

Indexed keywords

AMBRISENTAN; AMINO TERMINAL PRO BRAIN NATRIURETIC PEPTIDE; ANTINUCLEAR ANTIBODY; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; CENTROMERE ANTIBODY; ENDOGLIN; ENDOTHELIAL CELL ANTIBODY; ENDOTHELIN 1; ENDOTHELIN RECEPTOR ANTAGONIST; GUANYLATE CYCLASE ACTIVATOR; ILOPROST; INTERLEUKIN 1BETA; MACITENTAN; PHOSPHODIESTERASE V INHIBITOR; PLACEBO; PROSTACYCLIN; PROSTANOID; RIBONUCLEOPROTEIN ANTIBODY; RIOCIGUAT; SILDENAFIL; TREPROSTINIL; TUMOR NECROSIS FACTOR ALPHA; VASCULAR CELL ADHESION MOLECULE 1; VASCULOTROPIN; VON WILLEBRAND FACTOR; BIOLOGICAL MARKER;

CLINICAL FEATURE; CONTINUOUS INFUSION; DRUG EFFECT; DRUG EFFICACY; DRUG SAFETY; HEART CATHETERIZATION; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HIGH RISK POPULATION; HUMAN; INTERSTITIAL LUNG DISEASE; LUNG FUNCTION TEST; LUNG TRANSPLANTATION; MORTALITY; PATIENT MONITORING; PHYSICAL EXAMINATION; PREDICTIVE VALUE; PREVALENCE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; REVIEW; RISK FACTOR; SCREENING TEST; SENSITIVITY AND SPECIFICITY; SURVIVAL RATE; SYSTEMIC SCLEROSIS; TRANSTHORACIC ECHOCARDIOGRAPHY; COMPLICATION; EXERCISE TEST; HYPERTENSION, PULMONARY; LUNG DISEASES, INTERSTITIAL; PHYSIOLOGIC MONITORING;

BIOMARKERS; CARDIAC CATHETERIZATION; EXERCISE TEST; HUMANS; HYPERTENSION, PULMONARY; LUNG DISEASES, INTERSTITIAL; MONITORING, PHYSIOLOGIC; RISK FACTORS; SCLERODERMA, SYSTEMIC;

EID: 84937979210     PISSN: 0889857X     EISSN: 15583163     Source Type: Journal    
DOI: 10.1016/j.rdc.2015.04.009     Document Type: Review

References (101)

1. Mukerjee D., St George D., Coleiro B., et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003, 62(11):1088-1093.
2. Chung L., Domsic R.T., 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):489-495.
3. Launay D., Sitbon O., Hachulla E., et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann Rheum Dis 2013, 72(12):1940-1946.
4. Badesch D.B., Champion H.C., Sanchez M.A., et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009, 54(1 Suppl):S55-S66.
5. Simonneau G., Robbins I.M., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009, 54(1 Suppl):S43-S54.
6. Simonneau G., Gatzoulis M.A., Adatia I., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013, 62(25 Suppl):D34-D41.
7. Domsic R.T., Chung L., Gomberg-Maitland M., et al. Pulmonary hypertension assessment and recognition of outcomes in scleroderma (PHAROS): comparison of outcomes in subtypes of pulmonary hypertension. Arthritis Rheum 2010, 62(10S):S245.
8. Johnson S.R., Patsios D., Hwang D.M., et al. Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension. J Rheumatol 2006, 33(11):2347-2350.
9. McGuire F., Kennelly T., Tillack T., et al. Pulmonary capillary hemangiomatosis associated with CREST syndrome: a case report and review of the literature. Respiration 2010, 80(5):435-438.
10. Montani D., Price L.C., Dorfmuller P., et al. Pulmonary veno-occlusive disease. Eur Respir J 2009, 33(1):189-200.
11. Montani D., Achouh L., Dorfmuller P., et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine 2008, 87(4):220-233.
12. O'Callaghan D.S., Dorfmuller P., Jais X., et al. Pulmonary veno-occlusive disease: the bete noire of pulmonary hypertension in connective tissue diseases?. Presse Med 2011, 40(1 Pt 2):e65-e78.
13. Hachulla E., Gressin V., Guillevin L., et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005, 52(12):3792-3800.
14. Hachulla E., de Groote P., Gressin V., 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(6):1831-1839.
15. Hsu V.M., Chung L., Hummers L.K., et al. Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. Semin Arthritis Rheum 2014, 44(1):55-62.
16. Avouac J., Airo P., Meune C., et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 2010, 37(11):2290-2298.
17. Schachna L., Wigley F.M., Chang B., et al. Age and risk of pulmonary arterial hypertension in scleroderma. Chest 2003, 124(6):2098-2104.
18. Scorza R., Caronni M., Bazzi S., et al. Post-menopause is the main risk factor for developing isolated pulmonary hypertension in systemic sclerosis. Ann N Y Acad Sci 2002, 966:238-246.
19. Beretta L., Caronni M., Origgi L., et al. Hormone replacement therapy may prevent the development of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Scand J Rheumatol 2006, 35(6):468-471.
20. Cox S.R., Walker J.G., Coleman M., et al. Isolated pulmonary hypertension in scleroderma. Intern Med J 2005, 35(1):28-33.
21. Hachulla E., Launay D., Mouthon L., et al. Is pulmonary arterial hypertension really a late complication of systemic sclerosis?. Chest 2009, 136(5):1211-1219.
22. Shah A.A., Wigley F.M., Hummers L.K. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. J Rheumatol 2010, 37(1):98-104.
23. Steen V.D. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 2005, 35(1):35-42.
24. Kampolis C., Plastiras S., Vlachoyiannopoulos P., et al. The presence of anti-centromere antibodies may predict progression of estimated pulmonary arterial systolic pressure in systemic sclerosis. Scand J Rheumatol 2008, 37(4):278-283.
25. Aggarwal R., Lucas M., Fertig N., et al. Anti-U3 RNP autoantibodies in systemic sclerosis. Arthritis Rheum 2009, 60(4):1112-1118.
26. Steen V.D., Lucas M., Fertig N., et al. Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody. J Rheumatol 2007, 34(11):2230-2235.
27. Assous N., Allanore Y., Batteux F., et al. Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. Clin Exp Rheumatol 2005, 23(2):199-204.
28. Steen V., Medsger T.A. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003, 48(2):516-522.
29. Yaqub A., Chung L. Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis. Curr Rheumatol Rep 2013, 15(1):302.
30. Dib H., Tamby M.C., Bussone G., et al. Targets of anti-endothelial cell antibodies in pulmonary hypertension and scleroderma. Eur Respir J 2012, 39(6):1405-1414.
31. Negi V.S., Tripathy N.K., Misra R., et al. Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension. J Rheumatol 1998, 25(3):462-466.
32. Becker M.O., Kill A., Kutsche M., et al. Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis. Am J Respir Crit Care Med 2014, 190(7):808-817.
33. Steen V.D., Graham G., Conte C., et al. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 1992, 35(7):765-770.
34. Allanore Y., Borderie D., Avouac J., et al. High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum 2008, 58(1):284-291.
35. Steen V., Chou M., Shanmugam V., et al. Exercise-induced pulmonary arterial hypertension in patients with systemic sclerosis. Chest 2008, 134(1):146-151.
36. Shah A.A., Chung S.E., Wigley F.M., et al. Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients. Ann Rheum Dis 2013, 72(7):1136-1140.
37. Valerio C.J., Schreiber B.E., Handler C.E., et al. Borderline mean pulmonary artery pressure in patients with systemic sclerosis: transpulmonary gradient predicts risk of developing pulmonary hypertension. Arthritis Rheum 2013, 65(4):1074-1084.
38. Bae S., Saggar R., Bolster M.B., et al. Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry. Ann Rheum Dis 2012, 71(8):1335-1342.
39. Galie N., Hoeper M.M., Humbert M., et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2009, 34(6):1219-1263.
40. Saukkonen K., Tan T.C., Sharma A., et al. Case 9-2014: a 34-year-old woman with increasing dyspnea (case records of the Massachusetts General Hospital). N Engl J Med 2014, 370(12):1149-1157.
41. Condliffe R., Kiely D.G., Peacock A.J., et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009, 179(2):151-157.
42. Humbert M., Yaici A., de Groote P., et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 2011, 63(11):3522-3530.
43. Coghlan J.G., Denton C.P., Grunig E., et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014, 73(7):1340-1349.
44. Khanna D., Gladue H., Channick R., et al. Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension. Arthritis Rheum 2013, 65(12):3194-3201.
45. Hachulla E., Gressin V., Guillevin L., et al. Pulmonary arterial hypertension in systemic sclerosis: definition of a screening algorithm for early detection (the ItinerAIR-Sclerodermie Study). Rev Med Interne 2004, 25(5):340-347. [in French].
46. Williams M.H., Handler C.E., Akram R., et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 2006, 27(12):1485-1494.
47. Cavagna L., Caporali R., Klersy C., et al. Comparison of brain natriuretic peptide (BNP) and NT-proBNP in screening for pulmonary arterial hypertension in patients with systemic sclerosis. J Rheumatol 2010, 37(10):2064-2070.
48. Thakkar V., Stevens W.M., Prior D., et al. N-terminal pro-brain natriuretic peptide in a novel screening algorithm for pulmonary arterial hypertension in systemic sclerosis: a case-control study. Arthritis Res Ther 2012, 14(3):R143.
49. Gladue H., Steen V., Allanore Y., et al. Combination of echocardiographic and pulmonary function test measures improves sensitivity for diagnosis of systemic sclerosis-associated pulmonary arterial hypertension: analysis of 2 cohorts. J Rheumatol 2013, 40(10):1706-1711.
50. Fischer A., Bull T.M., Steen V.D. Practical approach to screening for scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res 2012, 64(3):303-310.
51. Hoeper M.M., Bogaard H.J., Condliffe R., et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013, 62(25 Suppl):D42-D50.
52. Wilsher M., Good N., Hopkins R., et al. The six-minute walk test using forehead oximetry is reliable in the assessment of scleroderma lung disease. Respirology 2012, 17(4):647-652.
53. Mathai S.C., Puhan M.A., Lam D., et al. The minimal important difference in the 6-minute walk test for patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2012, 186(5):428-433.
54. Tzelepis G.E., Kelekis N.L., Plastiras S.C., et al. Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum 2007, 56(11):3827-3836.
55. Vacca A., Meune C., Gordon J., et al. Cardiac arrhythmias and conduction defects in systemic sclerosis. Rheumatology (Oxford) 2014, 53(7):1172-1177.
56. Gunther S., Jais X., Maitre S., et al. Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension. Arthritis Rheum 2012, 64(9):2995-3005.
57. Hsu V.M., Moreyra A.E., Wilson A.C., et al. Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right-heart catheterization. J Rheumatol 2008, 35(3):458-465.
58. Mathai S.C., Sibley C.T., Forfia P.R., et al. Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension. J Rheumatol 2011, 38(11):2410-2418.
59. Gopal D.M., Doldt B., Finch K., et al. Relation of novel echocardiographic measures to invasive hemodynamic assessment in scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res 2014, 66(9):1386-1394.
60. Le Pavec J., Girgis R.E., Lechtzin N., et al. Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease: impact of pulmonary arterial hypertension therapies. Arthritis Rheum 2011, 63(8):2456-2464.
61. Mathai S.C., Hummers L.K., Champion H.C., et al. Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum 2009, 60(2):569-577.
62. Gargani L., Pignone A., Agoston G., et al. Clinical and echocardiographic correlations of exercise-induced pulmonary hypertension in systemic sclerosis: a multicenter study. Am Heart J 2013, 165(2):200-207.
63. Bernstein E.J., Mandl L.A., Gordon J.K., et al. Submaximal heart and pulmonary evaluation: a novel noninvasive test to identify pulmonary hypertension in patients with systemic sclerosis. Arthritis Care Res 2013, 65(10):1713-1718.
64. Williams M.H., Das C., Handler C.E., et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006, 92(7):926-932.
65. Tyndall A.J., 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):1809-1815.
66. Hesselstrand R., Wildt M., Ekmehag B., et al. Survival in patients with pulmonary arterial hypertension associated with systemic sclerosis from a Swedish single centre: prognosis still poor and prediction difficult. Scand J Rheumatol 2011, 40(2):127-132.
67. 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(6):1383-1394.
68. Chung L., Liu J., Parsons L.S., et al. Response to 'survival in pulmonary hypertension registries: the importance of incident cases'. Chest 2011, 139(6):1548-1549.
69. Lefevre G., Dauchet L., Hachulla E., et al. Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis. Arthritis Rheum 2013, 65(9):2412-2423.
70. Chung L., Farber H.W., 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):1494-1504.
71. Schioppo T., Artusi C., Ciavarella T., et al. N-TproBNP as biomarker in systemic sclerosis. Clin Rev Allergy Immunol 2012, 43(3):292-301.
72. van Bon L., Affandi A.J., Broen J., et al. Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med 2014, 370(5):433-443.
73. Pendergrass S.A., Hayes E., Farina G., et al. Limited systemic sclerosis patients with pulmonary arterial hypertension show biomarkers of inflammation and vascular injury. PLoS One 2010, 5(8):e12106.
74. Barnes T., Gliddon A., Dore C.J., et al. Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis. Rheumatology (Oxford) 2012, 51(9):1606-1609.
75. Chung L., Cramb C., Robinson W., et al. Differential expression of hepatocyte growth factor (HGF) in patients with systemic sclerosis-associated pulmonary arterial hypertension. Arthritis Rheum 2012, 64(10):S632.
76. Dimitroulas T., Giannakoulas G., Karvounis H., et al. Biomarkers in systemic sclerosis-related pulmonary arterial hypertension. Curr Vasc Pharmacol 2011, 9(2):213-219.
77. Coral-Alvarado P., Quintana G., Garces M.F., et al. Potential biomarkers for detecting pulmonary arterial hypertension in patients with systemic sclerosis. Rheumatol Int 2009, 29(9):1017-1024.
78. Hassoun P.M. Therapies for scleroderma-related pulmonary arterial hypertension. Expert Rev Respir Med 2009, 3(2):187-196.
79. Taichman D.B., Ornelas J., Chung L., et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest 2014, 146(2):449-475.
80. Nikpour M., Stevens W., Proudman S.M., et al. Should patients with systemic sclerosis-related pulmonary arterial hypertension be anticoagulated?. Intern Med J 2013, 43(5):599-603.
81. Johnson S.R., Granton J.T., Tomlinson G.A., et al. Warfarin in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. A Bayesian approach to evaluating treatment for uncommon disease. J Rheumatol 2012, 39(2):276-285.
82. Badesch D.B., Tapson V.F., McGoon M.D., et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000, 132(6):425-434.
83. Badesch D.B., McGoon M.D., Barst R.J., et al. Longterm survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol. J Rheumatol 2009, 36(10):2244-2249.
84. Oudiz R.J., Schilz R.J., Barst R.J., et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004, 126(2):420-427.
85. Tapson V.F., Gomberg-Maitland M., McLaughlin V.V., et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest 2006, 129(3):683-688.
86. McLaughlin V.V., Benza R.L., Rubin L.J., et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 2010, 55(18):1915-1922.
87. Tapson V.F., Torres F., Kermeen F., et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial. Chest 2012, 142(6):1383-1390.
88. Jing Z.C., Parikh K., Pulido T., et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation 2013, 127(5):624-633.
89. Olschewski H., Simonneau G., Galie N., et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002, 347(5):322-329.
90. Caramaschi P., Volpe A., Tinazzi I., et al. Does cyclically iloprost infusion prevent severe isolated pulmonary hypertension in systemic sclerosis? Preliminary Results. Rheumatol Int 2006, 27(2):203-205.
91. Rubin L.J., Badesch D.B., Barst R.J., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002, 346(12):896-903.
92. Denton C.P., Pope J.E., Peter H.H., et al. 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(9):1222-1228.
93. Pulido T., Adzerikho I., Channick R.N., et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013, 369(9):809-818.
94. Macchia A., Mariani J., Tognoni G. Macitentan and pulmonary arterial hypertension. N Engl J Med 2014, 370(1):82.
95. Galie N., Olschewski H., Oudiz R.J., 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(23):3010-3019.
96. Badesch D.B., Hill N.S., Burgess G., et al. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 2007, 34(12):2417-2422.
97. Galie N., Brundage B.H., Ghofrani H.A., et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009, 119(22):2894-2903.
98. Ghofrani H.A., Galie N., Grimminger F., et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013, 369(4):330-340.
99. Schachna L., Medsger T.A., Dauber J.H., et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006, 54(12):3954-3961.
100. Khan I.Y., Singer L.G., de Perrot M., et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med 2013, 107(12):2081-2087.
101. Bernstein E., Peterson E., Bathon J., et al. One-year survival of adults with systemic sclerosis following lung transplantation: a nationwide cohort study. Arthritis Rheumatol 2014, 66(11):S789.