Modern tactics of managing patients with pulmonary arterial hypertension associated with systemic connective tissue diseases.
Keywords:pulmonary arterial hypertension, systemic connective tissue diseases, systemic scleroderma
A review of the literature on the problem of one of the rare manifestations of systemic diseases - pulmonary arterial hypertension is presented. The necessity of early and thorough differential diagnosis, verification of the diagnosis using invasive methods for assessing central hemodynamics is substantiated. A model for screening patients with systemic scleroderma, which simplifies the determination of indications to set the research methods that allow to verify the diagnosis is proposed. Modern approaches to drug therapy are described, and questions of improving the patients’ survival with this prognostically unfavorable pathology are discussed.
Syniachenko OV, Yehudina YeD, Mikukst VYa, Yermolaiva MV, et al. [Angiopathy in systemic scleroderma]. Ukrainskyi revmatolohichnyi zhurnal. 2017;67(1):5-12. Russian.
Volkov AV. [Diagnosis of pulmonary hypertension in systemic scleroderma]. Sovremenaya revmatologiya. 2008;4:22-25. Russian.
Van den Hoogen F, Khanna D, Fransen J, [et al.] 2013 classification criteria for systemic sclerosis: anAmericanCollegeof Rheumatology/European League Against Rheumatism Collaborative Initiative. Ann Rheum Dis. 2013;72(11):1747–55. doi: 10.1136/annrheumdis-2013-204424
Syniachenko OV, Yehudina YeD, Mikukst VYa, Yermolaiva MV. [Rheological properties of blood serum in limited and diffuse forms of systemic scleroderma]. Ukrainskyi zhurnal dermatolohii, venerolohii, kosmetolohii. 2017;64(1):43-46. Russian.
Saad YeO, Ananeva LP, Novikova DS, Ajkeperova RT. [Traditional risk factors for cardiovascular disease in systemic scleroderma and their relationship with heart structural changes]. Nauchno-prakticheskaya revmatologiya.2016;54(6):687-692. Russian.
MathaiSC, Girgis RE, Fisher MR, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J.2007; 29:469-75.
Francesco B, Franchini S, Colantuoni E, et al. Anti-β2 glycoprotein I antibodies are independently associated with macrovascular disease and mortality in scleroderma patients Arthritis Rheum. 2009;60(8):2480-89. doi: 10.1002/art.24684.
Hurdman J, Condliffe R, Elliot CA, et al. ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a Referral Centre. Eur Respir J. 2012;39(4):945-55. doi: 10.1183 / 09031936.00078411
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):1383-94. doi: 10.1378/chest.10-0260
Hoeper MM, Faulenbach C, Golpon H, et al. Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension. Eur Respir J 2004;24:1007-10.
Gü nther S, Jais X, Maitre S, et al. Computed tomography find- ings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension. Arthritis Rheum. 2012;64(9):2995-3005. doi: 10.1002 / art.34501
Denton CP. Systemic sclerosis: from pathogenesis to targeted therapy. Clin. Exp. Rheumatol. 2015;33(4):3-7.
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):3792-800. doi: 10.1002/art.21433
Mukerjee D, St George D, Knight C, et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford). 2004;43(4):461-6. doi: 10.1093/rheumatology/keh067.
Ranchoux B, Harvey LD, Ayon RJ, et al. Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape. Pulm Circ. 2018;8(1):2045893217752912. doi: 10.1177/2045893217752912
McMahan ZH, Cottrell TR, Wigley FM, et al.Enrichment of Scleroderma Vascular Disease-Associated Autoantigens in Endothelial Lineage Cells. Arthritis Rheumatol. 2016;68(10):2540-9. doi: 10.1002/art.39743
Coghlan JC, Denton CP, Grü nig E, et al. Evidence-based detec- tion of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340-9. doi: 10.1136/annrheumdis-2013-203301
Ho YY, Lagares D, Tager AM, Kapoor M Fibrosis – a lethal component of systemic sclerosis. Nat. Rev. Rheumatol. 2014;10(7):390-402. doi: 10.1038/nrrheum.2014.53
Dorfmuller P, Humbert M, Perros F, et al. Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol. 2007;38(6):893-902. doi: 10.1016/j.humpath.2006.11.022
Campo A,MathaiSC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2016;182(2):252-60. doi: 10.1164/rccm.200912-1820OC
Allanore Y, Borderie D, Avouac J, Zerkk D, 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. doi: 10.1002/art.23187
GadreSK, Minai OA, Wang XF, et al. Lung or Heart-Lung Transplant in Pulmonary Arterial Hypertension: What Is the Impact of Systemic Sclerosis? Exp Clin Transplant. 2017;15(6):676-684. doi: 10.6002 / ect.2016.0209
Badesch DB, McGoon MD, Barst RJ, et al. Longterm survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol. J Rheumatol. 2009;36(10):2244-9. doi: 10.3899/jrheum.081277
Mathai SP, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413-25. doi: 10.1016/j.hfc.2012.04.001
Manetti M, Guiducci S, Matucci-Cerinic M. The crowded crossroad to angiogenesis in systemic sclerosis: where is the key to the problem? Arthritis Res. Ther. 2016;18(5);36-46. doi: 10.1186/s13075-016-0937-x
Launay D, Mouthon L, Hachulla E, et al. Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol. 2007;34(5):1005-11.
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;62:1088-93.
Avouac J, Airo P, Meune C, Beretta L, et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and meta-analysis of 5 studies. J Rheumatol. 2010;37(11):2290-8. doi: 10.3899/jrheum.100245
Connoly MJ, Abdullah S, Ridout DA, et al. Prognostic significance of computed tomography criteria for pulmonary veno-occlusive disease in systemic sclerosis-pulmonary arterial hypertension. Rheumatology (Oxford). 2017;56(12):2197-203. doi: 10.1093/rheumatology/kex351
Badesch DB, Raskob GE, Elliott CG, Krichman AM, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376-87. doi: 10.1378/chest.09-1140
Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension inFrance: results from a national registry. Am. J. Respir. Crit. Care Med. 2006;173(9):1023-30.
Hayato A, Konno T, Motohiko A, et al. Pulmonary hypertension associated with venoocclusive disease in systemic sclerosis: Insight into the mechanism of resistance to vasodilator. Journal of Cardiology Cases. 2012;5(1):e44-e47.
Iudici M, Codullo V, Giuggioli D, et al. Pulmonary hypertension in systemic sclerosis: prevalence, incidence and predictive factors in a large multicentric Italian cohort. Clin Exp Rheumatol. 2013;31(76):31-6.
MacGregor AJ, Canavan R, Knight C, et al. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford). 2001;40:453-9.
Daraban AM, Enache R, Predescu L, et al. Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. Case presentation and review of the literature. Rom J Intern Med. 2015;53(2):175-83.
Johnson SR, Patsios D, Hwang DM, Granton JT. Pulmonary venoocclusive disease and scleroderma associated pulmonary hypertension. J Rheumatol. 2006;33(11):2347-50.
Williams M, Handler C, 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):1485-94.
Rosenkranz S, Ioana R. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension Preston European Respiratory Review. 2015;24:642-52. doi: 10.1183 / 16000617.0062-2015
Schwaiger JP, Khanna D, Gerry Coghlan J. Screening patients with scleroderma for pulmonary arterial hypertension and implications for other at risk populations. Eur Respir Rev. 2013;22(130):515-25. doi: 10.1183/09059180.00006013
Tsou PS, Rabquer BJ, Ohara RA, et al. Scleroderma dermal microvascular endothelial cells exhibit defective response to pro-angiogenic chemokines. Rheumatology. 2016;55(4):745-754. doi: 10.1093/rheumatology/kev399
Dimitroulas T, Sarafidis P, Roma V, et al. Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature. Inflamm. Allergy Drug. Targets. 2010;9(4);313-318.
Njaman W, Iesaki T, Iwama Y, et al. Serum uric acid as a prognostic predictor in pulmonary arterial hypertension with connective tissue disease. Inter. Heart J.2007;48(4):523-32.
Ami A Shah, Fredrick M Wigley, Laura K. Hummers.Telangiectases in Scleroderma: A Potential Clinical Marker of Pulmonary Arterial Hypertension. J Rheumatol. 2010;37(1):98-104. doi: 10.3899/jrheum.090697
Steen V, Medsger TA Jr. Predictors of isolated pulmonary hyper- tension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum. 2003;48(2):516-22. doi: 10.1002 / art.10775
Gü ndü z OH, Fertig N, Lucas M, Medsger TA. Systemic sclerosis with renal crisis and pulmonary hypertension: a report of eleven cases. Arthritis Rheum. 2001;44(7):1663-6.
Kawut SM, Horn EM, Berekashvili KK, et al. Von Willibrand factor independently predicts long-term pulmonary arterial hypertension. Chest. 2005;128:2355-62.
How to Cite
Copyright (c) 2018 Medicni perspektivi (Medical perspectives)
This work is licensed under a Creative Commons Attribution 4.0 International License.
Submitting manuscript to the journal "Medicni perspektivi" the author(s) agree with transferring copyright from the author(s) to publisher (including photos, figures, tables, etc.) editor, reproducing materials of the manuscript in the journal, Internet, translation into other languages, export and import of the issue with the author’s article, spreading without limitation of their period of validity both on the territory of Ukraine and other countries. This and other mutual duties of the author and all co-authors separately and editorial board are secured by written agreement by special form to use the article, the sample of which is presented on the site.
Author signs a written agreement and sends it to Editorial Board simultaneously with submission of the manuscript.