Pulmonary hypertension screening in patients with systemic sclerosis, in a tertiary center, in Turkey; a cross-sectional original study

Yıl 2020, Cilt: 11 Sayı: 3, 146 - 155, 22.06.2020

Hilal Erken Pamukcu , Çağatay Tunca Cem Özişler Veysel Tanık , Bahar Tekin Tak , Saadet Demirtaş İnci , Ali Erhan Özdemirel Melih Pamukcu , Tolga Han Efe

https://doi.org/10.18663/tjcl.630633

Öz

Aim: Development of Pulmonary Hypertension (PH) in Systemic Sclerosis (SS) significantly reduces the survival of the disease and early diagnosis and treatment is very important.
The aim of this study was to investigate the presence of PH in patients who were followed and treated by rheumatology clinic with the diagnosis of SS and who did not have a known diagnosis of PH.
Materials and Methods: This cross-sectional study was completed with 51 patients with SS and a control group of 51 volunteers with similar characteristics in terms of gender and comorbidity. Demographic, laboratory and echocardiographic data were recorded.
Results: The median age of the patients with systemic sclerosis was 53 (46-60) years and the control group was 50 (45-55) years. 42 (82.4%) of the SS patients were female and 39 (76.5%) of the control group were female. Right heart catheterization was performed to 3 patients with high pulmonary artery pressure (>40 mmHg) on transthorasic echocardiography. Group 1 PH was diagnosed in two of three patients (3.9%); group 2 PH was diagnosed in one of three patients (1.9%).
Conclusion: In our study, we detected pulmonary hypertension in 5.8% of 51 patients with systemic sclerosis in a tertiary center. Although these patients have undergone PH screening at certain frequencies, it is noteworthy that we achieved this finding. We believe that we have detected patients with pulmonary systolic pressure at the border and showing rapid progression. Our study supports the more frequent screening of SS patients with borderline pulmonary artery pressure elevation.

Anahtar Kelimeler

systemic sclerosis, pulmonary hypertension, echocardiography, right heart catheterization

Türkiye’de tersiyer bir merkezde sistemik skleroz hastalarında pulmoner hipertansiyon taraması; kesitsel orjinal çalışma

Öz

Amaç: Sistemik Sklerozda (SS) Pulmoner Hipertansiyon (PH) gelişimi hastalığın sürvisini önemli ölçüde azaltmaktadır ve erken tanı ve tedavi çok önemlidir. Çalışmamızda SS tanısıyla romatoloji kliniği tarafından takip ve tedavi altında olan ve ve bilinen PH tanısı olmayan hastaların PH varlığı açısından taranması amaçlanmıştır.
Gereç ve Yöntemler: Bu kesitsel çalışma SS tanısı olan 51 hasta ve cinsiyet ve komorbidite açısından benzer özellikte 51 gönüllüden oluşan kontrol grubuyla tamamlandı. Demografik, laboratuvar ve ekokardiyografik verileri kayıt edildi.
Bulgular: Sistemik skleroz hasta grubunun ortanca yaşı 53 (46-60), kontrol grubunun 50 (45-55) idi. SS hastalarının 42 (%82,4)’si kadın, kontrol grubunun 39 (%76,5)’sı kadın cinsiyetten oluşmaktaydı. Transtorasik ekokardiyografide pulmoner arter basıncı yüksek saptanan (>40 mmHg) 3 hastaya sağ kalp kateterizasyonu yapıldı. İkisinde grup 1 PH (%3,9); birinde grup 2 PH (%1,9) saptandı
Sonuç: Çalışmamızda, tersiyer bir merkezde sistemik skleroz tanısıyla takipli ve tedavi altında olan 51 hastada %5,8 oranında pulmoner hipertansiyon saptamış bulunmaktayız. Bu hastaların belirli sıklıklarla PH taramasından geçirilmiş olmasına rağmen bu bulguya ulaşmamız dikkat çekicidir. Muhtemelen sınırda pulmoner arter basınç yüksekliği olan hızlı progresyon gösteren hastaları saptadığımızı düşünmekteyiz. Çalışmamız, sınırda pulmoner arter basınç yüksekliği olan SS hastalarının daha sık taranması gerektiğini desteklemektedir.

Anahtar Kelimeler

sistemik skleroz, pulmoner hipertansiyon, ekokardiografi, sağ kalp kateterizasyonu

Kaynakça

• 1. Le Pavec J, Launay D, Mathai SC, Hassoun PM, Humbert M. Scleroderma lung disease. Clinical reviews in allergy & immunology 2011; 40: 104-16.
• 2. Steen V, Chou M, Shanmugam V, Mathias M, Kuru T, Morrissey R. Exercise-induced pulmonary arterial hypertension in patients with systemic sclerosis. Chest 2008; 134: 146-51.
• 3. Yang X, Mardekian J, Sanders KN, Mychaskiw MA, Thomas J, 3rd. Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature. Clinical rheumatology 2013; 32: 1519-31.
• 4. Hachulla E, Gressin V, Guillevin L et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis and rheumatism 2005; 52: 3792-800.
• 5. 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 and rheumatism 2013; 65: 2412-23.
• 6. Coghlan JG, Galie N, Barbera JA et al. Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Annals of the rheumatic diseases 2017; 76: 1219-27.
• 7. Gaine S, Chin K, Coghlan G et al. Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. The European respiratory journal 2017; 50.
• 8. Simonneau G, Montani D, Celermajer DS et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. The European respiratory journal 2019; 53.
• 9. van den Hoogen F, Khanna D, Fransen J et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis and rheumatism 2013; 65: 2737-47.
• 10. Lang RM, Badano LP, Mor-Avi V et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 2015; 28: 1-39
• 11. 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. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 2010; 23: 685-713.
• 12. Meune C, Avouac J, Wahbi K et al. Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patients. Arthritis and rheumatism 2008; 58: 1803-09.
• 13. Galie 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). The European respiratory journal 2015; 46: 903-75.
• 14. Launay D, Sobanski V, Hachulla E, Humbert M. Pulmonary hypertension in systemic sclerosis: different phenotypes. European respiratory review : an official journal of the European Respiratory Society 2017; 26.
• 15. Denton CP, Humbert M, Rubin L, Black CM. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Annals of the rheumatic diseases 2006; 65: 1336-40.
• 16. Kowal-Bielecka O, Avouac J, Pittrow D et al. Echocardiography as an outcome measure in scleroderma-related pulmonary arterial hypertension: a systematic literature analysis by the EPOSS group. The Journal of rheumatology 2010; 37: 105-15.
• 17. Mandel J, Mark EJ, Hales CA. Pulmonary veno-occlusive disease. American journal of respiratory and critical care medicine 2000; 162: 1964-73.
• 18. Avouac J, Airo P, Meune C et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. The Journal of rheumatology 2010; 37: 2290-98.
• 19. McGoon MD, Benza RL, Escribano-Subias P et al. Pulmonary arterial hypertension: epidemiology and registries. Journal of the American College of Cardiology 2013; 62: 51-59.
• 20. Coghlan JG, Denton CP, Grunig E et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Annals of the rheumatic diseases 2014; 73: 1340-9.
• 21. Valerio CJ, Schreiber BE, Handler CE, Denton CP, Coghlan JG. Borderline mean pulmonary artery pressure in patients with systemic sclerosis: transpulmonary gradient predicts risk of developing pulmonary hypertension. Arthritis and rheumatism 2013; 65: 1074-84.
• 22. Champion HC. The heart in scleroderma. Rheumatic diseases clinics of North America 2008; 34: 181-90
• 23. Parks JL, Taylor MH, Parks LP, Silver RM. Systemic sclerosis and the heart. Rheumatic diseases clinics of North America 2014; 40: 87-102.
• 24. Roque MCF, Sampaio-Barros PD, Arruda AL et al. Evaluation of Left Ventricular Diastolic Function by Echocardiography with Tissue Doppler in Systemic Sclerosis. Arquivos brasileiros de cardiologia 2017; 109: 410-415.
• 25. LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. The Journal of rheumatology 2001; 28: 1573-76.
• 26. Hachulla E, Launay D. Diagnosis and classification of systemic sclerosis. Clinical reviews in allergy & immunology 2011; 40: 78-83.
• 27. 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 and rheumatism 2009; 60: 1831-39.
• 28. Young A, Nagaraja V, Basilious M et al. Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension. Seminars in arthritis and rheumatism 2019; 48: 1059-67.