Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?
Journal
Current opinion in pulmonary medicine
ISSN: 1531-6971
Titre abrégé: Curr Opin Pulm Med
Pays: United States
ID NLM: 9503765
Informations de publication
Date de publication:
01 09 2023
01 09 2023
Historique:
medline:
3
8
2023
pubmed:
18
7
2023
entrez:
18
7
2023
Statut:
ppublish
Résumé
Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc), which confers significant morbidity and mortality. The current therapies and treatment strategies for SSc-associated PAH (SSc-PAH) are informed by those used to treat patients with idiopathic PAH (IPAH). There are, however, important differences between these two diseases that impact diagnosis, treatment, and outcomes. Both SSc-PAH and IPAH are incompletely understood with ongoing research into the underlying cellular biology that characterize and differentiate the two diseases. Additional research seeks to improve identification among SSc patients in order to diagnose patients earlier in the course of their disease. Novel therapies specifically for SSc-PAH such as rituximab and dimethyl fumarate are under investigation. Although patients with SSc-PAH and IPAH present with similar symptoms, there are significant differences between these two forms of PAH that warrant further investigation and characterization of optimal detection strategies, treatment algorithms, and outcomes assessment.
Identifiants
pubmed: 37461869
doi: 10.1097/MCP.0000000000001001
pii: 00063198-202309000-00008
doi:
Types de publication
Review
Journal Article
Research Support, N.I.H., Extramural
Research Support, U.S. Gov't, Non-P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
380-390Subventions
Organisme : NHLBI NIH HHS
ID : U01 HL125175
Pays : United States
Organisme : NHLBI NIH HHS
ID : T32 HL007534
Pays : United States
Informations de copyright
Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.
Références
De Almeida Chaves S, Porel T, Mounié M, et al. Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality. Arthritis Res Ther 2021; 23:295.
Haque A, Kiely DG, Kovacs G, et al. Pulmonary hypertension phenotypes in patients with systemic sclerosis. Eur Respir Rev 2021; 30:210053.
Chen J, Yang C, Pan J, et al. Clinical features and prognostic factors of systemic sclerosis in Guangxi, China: retrospective, single-center study of long-term survival in 470 patients. Int J Rheum Dis 2022; 25:182–191.
Chikhoune L, Brousseau T, Morell-Dubois S, et al. Association between routine laboratory parameters and the severity and progression of systemic sclerosis. J Clin Med 2022; 11:5087.
Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43:3618–3731.
Kolton Schneider L, Ali YS, Agnew JD. Scleroderma-associated pulmonary arterial hypertension: early detection for better outcomes. Treasure Island (FL): StatPearls Publishing; 2022.
Korman BD, Lachant DJ, Castelino FV. Pulmonary hypertension: how to best treat the different scleroderma phenotypes. Rheum Dis Clin North Am 2023; 49:345–357.
Mathai SC. Pulmonary hypertension associated with connective tissue disease. Cardiol Clin 2022; 40:29–43.
Yuan Y-P, Yuan P, Su Y-L, et al. Clinical phenotypes, hemodynamic characteristics and prognosis of Chinese patients with systemic sclerosis-associated precapillary pulmonary hypertension: a retrospective study. Clin Rheumatol 2022; 41:1675–1686.
Thomas CA, Anderson RJ, Condon DF. Diagnosis and management of pulmonary hypertension in the modern era: insights from the 6th world symposium. Pulm Ther 2020; 6:9–22.
Chennakesavulu PV, Uppaluri S, Koyi J, et al. Pulmonary hypertension in scleroderma- evaluation and management. Dis Mon 2022; 69:101468.
Calderon LM, Pope JE. Scleroderma epidemiology update. Curr Opin Rheumatol 2021; 33:122–127.
Saygin D, Domsic RT. Pulmonary arterial hypertension in systemic sclerosis: challenges in diagnosis, screening and treatment. Open Access Rheumatol 2019; 11:323–333.
Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol 2012; 24:165–170.
Puigrenier S, Giovannelli J, Lamblin N, et al. Mild pulmonary hemodynamic alterations in patients with systemic sclerosis: relevance of the new 2022 ESC/ERS definition of pulmonary hypertension and impact on mortality. Respir Res 2022; 23:284.
Ramjug S, Hussain N, Hurdman J, et al. Idiopathic and systemic sclerosis-associated pulmonary arterial hypertension: a comparison of demographic, hemodynamic, and MRI characteristics and outcomes. Chest 2017; 152:92–102.
Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ 2018; 360:j5492.
Zanin-Silva DC, Santana-Gonçalves M, Kawashima-Vasconcelos MY, Oliveira MC. Management of endothelial dysfunction in systemic sclerosis: current and developing strategies. Front Med (Lausanne) 2021; 8:788250.
Ranchoux B, Harvey LD, Ayon RJ, et al. Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series). Pulm Circ 2018; 8:1–17.
Hassoun PM. Pulmonary arterial hypertension. N Engl J Med 2021; 385:2361–2376.
Koumakis E, Wipff J, Dieudé P, et al. TGFβ receptor gene variants in systemic sclerosis-related pulmonary arterial hypertension: results from a multicentre EUSTAR study of European Caucasian patients. Ann Rheum Dis 2012; 71:1900–1903.
Hernandez-Gonzalez I, Tenorio-Castano J, Ochoa-Parra N, et al. Novel genetic and molecular pathways in pulmonary arterial hypertension associated with connective tissue disease. Cells 2021; 10:1488.
Xu B, Xu G, Yu Y, Lin J. The role of TGF-β or BMPR2 signaling pathway-related miRNA in pulmonary arterial hypertension and systemic sclerosis. Arthritis Res Ther 2021; 23:288.
Tabeling C, González Calera C, Lienau J, et al. Endothelin B receptor immunodynamics in pulmonary arterial hypertension. Front Immunol 2022; 13:895501.
Becker MO, Kill A, Kutsche M, et al. Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis. Am J Respir Crit Care Med 2014; 190:808–817.
Cardoneanu A, Burlui AM, Macovei LA, et al. Targeting systemic sclerosis from pathogenic mechanisms to clinical manifestations: why IL-6? Biomedicines 2022; 10:318.
Overbeek MJ, Vonk MC, Boonstra A, et al. Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy. Eur Respir J 2009; 34:371–379.
Dorfmüller 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; 38:893–902.
Dorfmüller P, Montani D, Humbert M. Beyond arterial remodelling: pulmonary venous and cardiac involvement in patients with systemic sclerosis-associated pulmonary arterial hypertension. Eur Respir J 2010; 35:6–8.
Hsu S, Kokkonen-Simon KM, Kirk JA, et al. Right ventricular myofilament functional differences in humans with systemic sclerosis-associated versus idiopathic pulmonary arterial hypertension. Circulation 2018; 137:2360–2370.
Overbeek MJ, Mouchaers KTB, Niessen HM, et al. Characteristics of interstitial fibrosis and inflammatory cell infiltration in right ventricles of systemic sclerosis-associated pulmonary arterial hypertension. Int J Rheumatol 2010; 2010:604615.
Mathai SC, Bueso M, Hummers LK, et al. Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension. Eur Respir J 2010; 35:95–104.
Syed Gaggatur N, Sange AH, Srinivas N, et al. Systemic sclerosis: highlighting respiratory complications and significance of early screening. Cureus 2021; 13:e17291.
Kiely DG, Lawrie A, Humbert M. Screening strategies for pulmonary arterial hypertension. Eur Heart J Suppl 2019; 21: (Suppl k): K9–K20.
Hewes JL, Lee JY, Fagan KA, Bauer NN. The changing face of pulmonary hypertension diagnosis: a historical perspective on the influence of diagnostics and biomarkers. Pulm Circ 2020; 10:1–26.
Lau EMT, Celermajer DS, Keogh A, Thakkar V. Screening of pulmonary arterial hypertension. Semin Respir Crit Care Med 2017; 38:596–605.
Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest 2011; 140:19–26.
Mukherjee M, Mercurio V, Balasubramanian A, et al. Defining minimal detectable difference in echocardiographic measures of right ventricular function in systemic sclerosis. Arthritis Res Ther 2022; 24:146.
Lu J, Jani V, Mercurio V, et al. Stress echocardiographic prediction of emerging pulmonary vascular disease in systemic sclerosis. J Am Soc Echocardiogr 2023; 36:259–261.
Mukherjee M, Mercurio V, Tedford RJ, et al. Right ventricular longitudinal strain is diminished in systemic sclerosis compared with idiopathic pulmonary arterial hypertension. Eur Respir J 2017; 50:1701436.
Coghlan JG, Denton CP, Grünig E, et al. DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014; 73:1340–1349.
Guillén-Del Castillo A, Callejas-Moraga EL, García G. High sensitivity and negative predictive value of the DETECT algorithm for an early diagnosis of pulmonary arterial hypertension in systemic sclerosis: application in a single center. Arthritis Res Ther 2017; 19:135.
Coirier V, Chabanne C, Jouneau S, et al. Impact of three different algorithms for the screening of SSc-PAH and comparison with the decisions of a multidisciplinary team. Diagnostics (Basel) 2021; 11:1738.
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016; 37:67–119.
Young A, Moles V, Jaafar S, et al. Performance of the DETECT algorithm for pulmonary hypertension screening in a systemic sclerosis cohort. Arthritis Rheumatol 2021; 73:1731–1737.
Alenezi F, Covington TA, Mukherjee M, et al. Novel approaches to imaging the pulmonary vasculature and right heart. Circ Res 2022; 130:1445–1465.
Bier EA, Alenezi F, Lu J, et al. Noninvasive diagnosis of pulmonary hypertension with hyperpolarised 129 Xe magnetic resonance imaging and spectroscopy. ERJ Open Res 2022; 8:00035-2022.
Bauer Y, de Bernard S, Hickey P, et al. Identifying early pulmonary arterial hypertension biomarkers in systemic sclerosis: machine learning on proteomics from the DETECT cohort. Eur Respir J 2021; 57:2002591.
Semalulu T, Rudski L, Huynh T, et al. An evidence-based strategy to screen for pulmonary arterial hypertension in systemic sclerosis. Semin Arthritis Rheum 2020; 50:1421–1427.
Colalillo A, Hoffmann-Vold AM, Pellicano C, et al. EUSTAR collaborators. The role of TAPSE/sPAP ratio in predicting pulmonary hypertension and mortality in the systemic sclerosis EUSTAR cohort. Autoimmun Rev 2023; 22:103290.
Hachulla E, Agard C, Allanore Y, et al. Collaborators. French recommendations for the management of systemic sclerosis. Orphanet J Rare Dis 2021; 16: (Suppl 2): 322.
Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting survival in patients with pulmonary arterial hypertension: the REVEAL Risk Score Calculator 2.0 and comparison with ESC/ERS-based risk assessment strategies. Chest 2019; 156:323–337.
Kuwana M, Blair C, Takahashi T, et al. Initial combination therapy of ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) in the modified intention-to-treat population of the AMBITION study: post hoc analysis. Ann Rheum Dis 2020; 79:626–634.
Mercurio V, Diab N, Peloquin G, et al. Risk assessment in scleroderma patients with newly diagnosed pulmonary arterial hypertension: application of the ESC/ERS risk prediction model. Eur Respir J 2018; 52:1800497.
Mullin CJ, Khair RM, Damico RL, et al. PHAROS Investigators. Validation of the REVEAL prognostic equation and risk score calculator in incident systemic sclerosis-associated pulmonary arterial hypertension. Arthritis Rheumatol 2019; 71:1691–1700.
Matsukawa Y, Saito O, Aoki M, et al. Long-term administration of beraprost, an oral prostacyclin analogue, improves pulmonary diffusion capacity in patients with systemic sclerosis. Prostaglandins Leukot Essent Fatty Acids 2002; 67:45–49.
Barst RJ, McGoon M, McLaughlin V, et al. Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41:2119–2125.
Galiè N, Humbert M, Vachiéry JL, et al. Arterial Pulmonary Hypertension and Beraprost European (ALPHABET) Study Group. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002; 39:1496–1502.
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; 132:425–434.
Olschewski H, Simonneau G, Galiè N, et al. Inhaled Iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347:322–329.
McLaughlin VV, Benza RL, Rubin LJ, et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 2010; 55:1915–1922.
Jing ZC, 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; 127:624–633.
White RJ, Jerjes-Sanchez C, Meyer GMB, et al. Combination therapy with oral treprostinil for pulmonary arterial hypertension: a double-blind placebo-controlled clinical trial. Am J Respir Crit Care Med 2020; 201:707–717.
Barst RJ, Galie N, Naeije R, et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 2006; 28:1195–1203.
Vlachou M, Fayed H, Dawson A, et al. Intravenous prostanoids in systemic sclerosis-associated pulmonary arterial hypertension: a single-centre experience. Rheumatology (Oxford) 2022; 61:1106–1114.
Sitbon O, Channick R, Chin KM, et al. GRIPHON Investigators. Selexipag for the treatment of pulmonary arterial hypertension. New Engl J Med 2015; 373:2522–2533.
Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353:2148–2157.
Galiè N, Brundage BH, Ghofrani HA, et al. Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119:2894–2903.
Jing ZC, Yu ZX, Shen JY, et al. Vardenafil in pulmonary arterial hypertension. Am J Respir Crit Care Med 2012; 183:1723–1729.
Fischer A, Denton CP, Matucci-Cerinic M, et al. Ambrisentan response in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) - a subgroup analysis of the ARIES-E clinical trial. Respir Med 2016; 117:254–263.
Galiè N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension. Circulation 2008; 117:265–280.
Galiè N, Rubin L, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008; 371:2093–2100.
Denton CP, Humbert M, Rubin L. 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; 65:1336–1340.
Pulido T, Adzerikho I, Channick RN, et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. New Engl J Med 2013; 369:809–818.
Langleben D, Brock T, Dixon R, Barst R. STRIDE 1: effects of the selective ETA receptor antagonist sitaxsentan sodium, in a patient population with pulmonary arterial hypertension that meets traditional inclusion criteria of previous pulmonary arterial hypertension trials. J Cardiovasc Pharmacol 2004; 44 Suppl 1:S80–S84.
Humbert M, Barst RJ, Robbins IM, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24:353–359.
McLaughlin VV, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006; 174:1257–1263.
Simonneau G, Rubin LJ, Galiè N, et al. PACES Study Group. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149:521–530.
Lei Y, Zhang X, Lin H, et al. The effects of oral treatment for systemic sclerosis related pulmonary arterial hypertension: a systematic review and meta-analysis. Mod Rheumatol 2021; 31:151–161.
White RJ, Jerjes-Sanchez C, Meyer GMB, et al. Combination therapy with oral treprostinil for pulmonary arterial hypertension. A double-blind placebo-controlled clinical trial. Am J Respir Crit Care Med 2020; 201:707–717.
Chin KM, Sitbon O, Doelberg M, et al. Three- versus two-drug therapy for patients with newly diagnosed pulmonary arterial hypertension. J Am Coll Cardiol 2021; 78:1393–1403.
Humbert M, McLaughlin V, Gibbs SR, et al. PULSAR Trial Investigators. Sotatercept for the treatment of pulmonary arterial hypertension. New Engl J Med 2021; 384:1204–1215.
Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 Trial of Sotatercept for treatment of pulmonary arterial hypertension. New Engl J Med 2023; 388:1478–1490.
Pope JE, Denton CP, Johnson SR, et al. State-of-the-art evidence in the treatment of systemic sclerosis. Nat Rev Rheumatol 2023; 19:212–226.
Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. New Engl J Med 2021; 384:325–334.
Yarkan Tuğsal H, Zengin B, Kenar G, et al. Rituximab on lung, skin, and joint involvement in patients with systemic sclerosis: a case series study and review of the literature. Int J Rheum Dis 2022; 25:755–768.
Zamanian RT, Badesch D, Chung L, et al. Safety and efficacy of B-cell depletion with rituximab for the treatment of systemic sclerosis-associated pulmonary arterial hypertension: a multicenter, double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2021; 204:209–221.
Kong K, Koontz D, Morse C, et al. A pilot study of dimethyl fumarate in pulmonary arterial hypertension associated with systemic sclerosis. J Scleroderma Relat Disord 2021; 6:242–246.
Mathai SC. Defense Technical Information Center. Sildenafil for Early Pulmonary Vascular Disease in Scleroderma, W81XWH-20-1-0768 2021.
ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). 2000 Feb 29 - . Identifier NCT00984932, Effect of rosuvastatin on systemic sclerosis-related pulmonary hypertension; 2009 Sep 25 [cited 2023 Jan 10]; [about 5 screens]. Available from: https://clinicaltrials.gov/ct2/show/NCT00984932 .
Saggar R, Khanna D, Shapiro S, et al. Brief report: effect of ambrisentan treatment on exercise-induced pulmonary hypertension in systemic sclerosis: a prospective single-center, open-label pilot study. Arthritis Rheum 2012; 64:4072–4077.
Hassoun PM, Zamanian RT, Damico R, et al. Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2015; 192:1102–1110.
Olsson KM, Delcroix M, Ghofrani A, et al. Anticoagulation and survival in pulmonary arterial hypertension. Circulation 2013; 129:57–65.
Sandoval J, Gaspar J, Peña H, et al. Effect of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension. Eur Respir J 2011; 38:1343–1348.
Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. Chest 2013; 144:1346.
Khan IY, Singer LG, De Perrot M, et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med 2013; 107:2081–2087.
Pradère P, Tudorache I, Magnusson J, et al. Lung transplantation for scleroderma lung disease: indications, survival and prognosis. J Heart Lung Transplant 2018; 37:S164.
Lee K-I, Manuntag LJ, Kifayat A, et al. Cardiovascular manifestations of systemic sclerosis: an overview of pathophysiology, screening modalities, and treatment options. Cardiol Rev 2023; 31:22–27.