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Vol 79, Supp. III (2021): Zeszyty Edukacyjne 3/2021
Opinie i stanowiska ekspertów
Published online: 2021-09-16

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Opinia ekspertów Sekcji Krążenia Płucnego Polskiego Towarzystwa Kardiologicznego oraz Polskiego Towarzystwa Reumatologicznego dotycząca diagnostyki i leczenia nadciśnienia płucnego u pacjentów z układowymi chorobami tkanki łącznej

Tatiana Mularek-Kubzdela1, Michał Ciurzyński2, Otylia Kowal-Bielecka3, Jarosław D Kasprzak4, Grzegorz Kopeć5, Katarzyna Mizia-Stec6, Ewa Mroczek7, Ewa Lewicka8, Ilona Skoczylas9, Marek Grabka10, Michał Furdal11, Michał Florczyk12, Marek Brzosko13, Bogdan Batko14, Hanna Przepiera-Będzak13, Włodzimierz Samborski15, Eugeniusz J Kucharz16
DOI: 10.33963/v.kp.85930

Abstract

Układowe choroby tkanki łącznej (CTD, connective tissue diseases) obejmują dużą grupę chorób, które cechują się zajęciem wielu układów i narządów, i mają charakter autoimmunologiczny. W przebiegu CTD może dochodzić do rozwoju nadciśnienia płucnego (PH, pulmonary hypertension) o różnej etiologii: tętnicze nadciśnienie płucne (PAH, pulmonary arterial hypertension), PH wtórne do chorób płuc, postkapilarne nadciśnienie płucne w przebiegu chorób lewej części serca oraz przewlekłe zakrzepowo-zatorowe nadciśnienie płucne (CTEPH, chronic thromboembolic pulmonary hypertension). Ponadto poszczególne postaci PH mogą ze sobą współistnieć. U chorych z CTD, PAH występuje najczęściej w przebiegu twardziny układowej (SSc, systemic sclerosis), u ok. 8%–12% pacjentów. Rokowanie u pacjentów z nieleczonym PAH jest bardzo poważne, dlatego z uwagi na możliwość prowadzenia terapii celowanej na tętniczki płucne, szczególnie istotne jest wyodrębnienie populacji wysokiego ryzyka CTD-PAH oraz sprawna i precyzyjna diagnostyka. Badaniem przesiewowym w diagnostyce PH jest echokardiografia, jednak kliniczne i echokardiograficzne podejrzenie PH zawsze wymaga potwierdzenia cewnikowaniem prawego serca (RHC, right heart catheterisation). Potwierdzenie PAH umożliwia włączenie leczenia farmakologicznego, wydłużającego życie w tej grupie chorych, które powinno być stosowane w ośrodkach referencyjnych. W postępowaniu farmakologicznym dostępne są następujące grupy leków: antagoniści receptora endoteliny (ERA, endothelin receptor antagonists), inhibitory fosfodiesterazy 5 (PDE-5) oraz prostacykliny.

Keywords: nadciśnienie płucnetętnicze nadciśnienie płucnechoroby układowe tkanki łącznejtwardzina układowabadania przesiewowe

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References

  1. Mani P, Gonzalez D, Chatterjee S, et al. Cardiovascular complications of systemic sclerosis: what to look for. Cleve Clin J Med. 2019; 86(10): 685–695.
    CrossRefPubMed
  2. Attanasio U, Cuomo A, Pirozzi F, et al. Pulmonary hypertension phenotypes in systemic sclerosis: the right diagnosis for the right treatment. Int J Mol Sci. 2020; 21(12): 4430.
    CrossRefPubMed
  3. Aithala R, Alex AG, Danda D. Pulmonary hypertension in connective tissue diseases: an update. Int J Rheum Dis. 2017; 20(1): 5–24.
    CrossRefPubMed
  4. Kucharz EJ. Reumatoidalne zapalenie stawów. In: Puszczewicz MJ. ed. Wielka Interna. Reumatologia. Medical Tribune, Warszawa 2016: 79–95.
  5. Majdan M. Toczeń rumieniowaty układowy. Termedia, Poznań 2015.
  6. Kucharz EJ. Zapalenia naczyń. In: Zimmermann-Górska I. ed. Terapia w chorobach reumatycznych. PZWL Wydawnictwo Lekarskie, Warszawa 2018: 204–250.
  7. Hachulla E, Czirják L. EULAR Textbook on systemic sclerosis. BMJ Group, London 2013: London.
  8. Kucharz EJ. Idiopatyczne miopatie zapalne. In: Puszczewicz MJ. ed. Wielka Interna. Reumatologia. Medical Tribune, Warszawa 2016: 158–168.
  9. Zawilska K, Zimmermann-Górska I. Zespól antyfosfolipidowy. In: Zimmermann-Górska I. ed. Terapia w chorobach reumatycznych. PZWL Wydawnictwo Lekarskie, Warszawa 2018: 270–287.
  10. Kucharz EJ. Zespół Sjögrena. In: Olesińska MJ. ed. Reumatologia w gabinecie lekarza Podstawowej Opieki Zdrowotnej. PZWL Wydawnictwo Lekarskie, Warszawa 2019: 359–367.
  11. Olesińska M, Felis-Giemza A. Mieszana choroba tkanki łącznej. In: Zimmermann-Górska I, Felis-Giemza A. ed. Terapia w chorobach reumatycznych. PZWL Wydawnictwo Lekarskie, Warszawa 2018: 171–189.
  12. Kucharz EJ. Chronic inflammation-enhanced atherosclerosis: can we consider it as a new clinical syndrome? Med Hypotheses. 2012; 78(3): 396–397.
    CrossRefPubMed
  13. Galiè N, Humbert M, Vachiery JL, et al. ESC Scientific Document Group. 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 Heart J. 2016; 37(1): 67–119.
    CrossRefPubMed
  14. Simonneau G, Montani D, Celermajer D, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019; 53(1): 1801913.
    CrossRefPubMed
  15. 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(7): 1340–1349.
    CrossRefPubMed
  16. Kopeć G, Kurzyna M, Mroczek E, et al. Characterization of patients with pulmonary arterial hypertension: data from the Polish Registry of Pulmonary Hypertension (BNP-PL). J Clin Med. 2020; 9(1): 173.
    CrossRefPubMed
  17. Kwiatkowska J, Zuk M, Migdal A, et al. Children and adolescents with pulmonary arterial hypertension: baseline and follow-up data from the Polish Registry of Pulmonary Hypertension (BNP-PL). J Clin Med. 2020; 9(6): 1717.
    CrossRefPubMed
  18. Kopeć G, Kurzyna M, Mroczek E, et al. Database of pulmonary hypertension in the polish population (BNP‑PL): design of the registry. Kardiol Pol. 2019; 77(10): 972–974.
    CrossRefPubMed
  19. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013; 62(Suppl 25): D51–D59.
    CrossRefPubMed
  20. Niklas K, Niklas A, Mularek-Kubzdela T, et al. Prevalence of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease. Medicine (Baltimore). 2018; 97(28): e11437.
    CrossRefPubMed
  21. Weatherald J, Montani D, Jevnikar M, et al. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev. 2019; 28(153): 190023.
    CrossRefPubMed
  22. Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol. 2014; 66(6): 1625–1635.
    CrossRefPubMed
  23. Kanecki K, Goryński P, Tarka P, et al. Incidence and prevalence of Systemic Sclerosis (SSc) in Poland — differences between rural and urban regions. Ann Agric Environ Med. 2017; 24(2): 240–244.
    CrossRefPubMed
  24. Sierakowski S, Sierakowska M. Systemic scleroderma. In: Gajewski P, Szczeklik M. ed. Interna Szczekilka — Internal Medicine Textbook. Medycyna Praktyczna, Kraków 2020: 2060–2069.
  25. Weatherald J, Boucly A, Launay D, et al. Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension. Eur Respir J. 2018; 52(4): 1800678.
    CrossRefPubMed
  26. Kolstad KD, Li S, Steen V, et al. PHAROS Investigators. Long-term outcomes in systemic sclerosis-associated pulmonary arterial hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS). Chest. 2018; 154(4): 862–871.
    CrossRefPubMed
  27. Vonk MC, Vandecasteele E, van Dijk AP. Pulmonary hypertension in connective tissue diseases, new evidence and challenges. Eur J Clin Invest. 2021; 51(4): e13453.
    CrossRefPubMed
  28. Jiang Y, Turk MA, Pope JE. Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). Autoimmun Rev. 2020; 19(9): 102602.
    CrossRefPubMed
  29. 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.
    CrossRefPubMed
  30. Tyndall AJ, 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.
    CrossRefPubMed
  31. Galiè N, Humbert M, Vachiery JL, 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(4): 903–975.
    CrossRefPubMed
  32. Frost A, Badesch D, Gibbs JS, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019; 53(1): 1801904.
    CrossRefPubMed
  33. Young A, Nagaraja V, Basilious M, et al. Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension. Semin Arthritis Rheum. 2019; 48(6): 1059–1067.
    CrossRefPubMed
  34. Schoenfeld SR, Choi HK, Sayre EC, et al. Risk of pulmonary embolism and deep venous thrombosis in systemic sclerosis: a general population-based study. Arthritis Care Res (Hoboken). 2016; 68(2): 246–253.
    CrossRefPubMed
  35. Rudski LG, Lai WW, Afilalo J, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2010; 23(7): 685–713; quiz 786–788.
    CrossRefPubMed
  36. Kowal-Bielecka O, Fransen J, Avouac J, et al. EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017; 76(8): 1327–1339.
    CrossRefPubMed
  37. Montani D, Savale L, Natali D, et al. Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension. Eur Heart J. 2010; 31(15): 1898–1907.
    CrossRefPubMed
  38. Olsson K, Delcroix M, Ghofrani H, et al. Anticoagulation and survival in pulmonary arterial hypertension. Circulation. 2014; 129(1): 57–65.
    CrossRef
  39. 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(2): 323–337.
    CrossRefPubMed
  40. Kylhammar D, Kjellström B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018; 39(47): 4175–4181.
    CrossRefPubMed
  41. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006; 173(9): 1023–1030.
    CrossRefPubMed
  42. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017; 50(2): 1700740.
    CrossRefPubMed
  43. Hachulla E, Launay D, Yaici A, et al. French PAH-SSc Network. Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome. Rheumatology (Oxford). 2010; 49(5): 940–944.
    CrossRefPubMed
  44. Morrisroe K, Stevens W, Huq M, et al. Australian Scleroderma Interest Group (ASIG). Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension. Arthritis Res Ther. 2017; 19(1): 122.
    CrossRefPubMed
  45. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019; 53(1): 1801889.
    CrossRefPubMed
  46. Gadre SK, 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.
    CrossRefPubMed
  47. Shah RJ, Boin F. Lung transplantation in patients with systemic sclerosis. Curr Rheumatol Rep. 2017; 19(5): 23.
    CrossRefPubMed
  48. Lefèvre 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.
    CrossRefPubMed
  49. 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.
    CrossRefPubMed
  50. Khanna D, Zhao C, Saggar R, et al. Long-Term outcomes in patients with connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era: meta-analyses of randomized, controlled trials and observational registries. Arthritis Rheumatol. 2021; 73(5): 837–847.
    CrossRefPubMed
  51. 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(6): 425–434.
    CrossRefPubMed
  52. Almaaitah S, Highland KB, Tonelli AR. Management of pulmonary arterial hypertension in patients with systemic sclerosis. Integr Blood Press Control. 2020; 13: 15–29.
    CrossRefPubMed
  53. Sobanski V, Launay D, Hachulla E, et al. Current approaches to the treatment of systemic-sclerosis-associated pulmonary arterial hypertension (ssc-pah). Curr Rheumatol Rep. 2016; 18(2): 10.
    CrossRefPubMed
  54. Pulido T, Adzerikho I, Channick RN, et al. SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013; 369(9): 809–818.
    CrossRefPubMed
  55. Gaine S, Chin K, Coghlan G, et al. Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. Eur Respir J. 2017; 50(2): 1602493.
    CrossRefPubMed
  56. Coghlan JG, Galiè N, Barberà JA, et al. AMBITION investigators. Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Ann Rheum Dis. 2017; 76(7): 1219–1227.
    CrossRefPubMed
  57. Zizzo G, De Santis M, Ferraccioli GF. Mycophenolic acid in rheumatology: mechanisms of action and severe adverse events. Reumatismo. 2010; 62(2): 91–100.
    CrossRefPubMed
  58. Nawata M, Nagayasu A, Fujita Y, et al. Severe pulmonary arterial hypertension and interstitial pneumonia related to systemic lupus erythematosus successfully treated with mycophenolate mofetil: a novel case report. Lupus. 2020; 29(14): 1955–1960.
    CrossRefPubMed
  59. Donate PB, Cunha TM, Verri WA, et al. Bosentan, an endothelin receptor antagonist, ameliorates collagen-induced arthritis: the role of TNF-α in the induction of endothelin system genes. Inflamm Res. 2012; 61(4): 337–348.
    CrossRefPubMed
  60. Coirier V, Lescoat A, Chabanne C, et al. Pulmonary arterial hypertension in four patients treated by leflunomide. Joint Bone Spine. 2018; 85(6): 761–763.
    CrossRefPubMed
  61. McGee M, Whitehead N, Martin J, et al. Drug-associated pulmonary arterial hypertension. Clin Toxicol (Phila). 2018; 56(9): 801–809.
    CrossRefPubMed
  62. Quah Ed, Amoasii C, Mudawi T, et al. Systematic literature review investigating whether methotrexate causes chronic pulmonary fibrosis. Future Healthc J. 2019; 6(Suppl 2): 4.
    CrossRefPubMed
  63. Grossi O, Horeau-Langlard D, Agard C, et al. Low-dose methotrexate in PAH related to T-cell large granular lymphocyte leukaemia. Eur Respir J. 2012; 39(2): 493–494.
    CrossRefPubMed
  64. Dwyer N, Jones G, Kilpatrick D. Severe hepatotoxicity in a patient on bosentan upon addition of methotrexate: reversible with resumption of methotrexate without bosentan. J Clin Rheumatol. 2009; 15(2): 88–89.
    CrossRefPubMed

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