Advanced search

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

open access

View PDF (Polish) Download PDF file View HTML (Polish)
Page views 582
Article views/downloads 830
Get Citation

Connect on Social Media

Connect on Social Media

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

Full content:

View HTML (Polish) View PDF (Polish) Download PDF file

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

About cookies

Necessary cookies

Allways active

These cookies are necessary for the proper display and operation of the website. Blocking them may cause the website to malfunction.

Analytical cookies

As part of our website, we use analytical tools, such as Google Analytics, that allow us to verify website traffic. These tools may require the use of cookies.

Community cookies

These are cookies associated with the social networks we use. Accepting them will allow us to associate your visit to the site with our social media profiles.

Marketing cookies

These are cookies responsible for carrying out advertising and marketing activities - consenting to their use will allow us to target you with advertisements based on your previous activities within our website.

Copyright © 2023-2024 Via Medica Regulations Cookie policy Privacy Statement Legal Note