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Demographic and clinical differences between idiopathic and scleroderma-related pulmonary arterial hypertension: Russian National Registry analysis

Abstract

Pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) and idiopathic pulmonary arterial hypertension (IPAH) belong to group I in the clinical classification of PH, but there is evidence for significant differences in their survival due to current therapy. Objective: the objective of this report is to compare pts with (SSc-PAH) and (IPAH) based on data of Russian National Registry. Patients and methods: in the study we included 52 pts with IPAH and 50 with SSc-PAH. There were no differences in functional class (FC). Diagnosis was based on RHC. Results. At the moment of diagnosis average age of patients with SSc-PAH was 15 year higher (p<0.0001). 6MWD was somewhat more in IPAH group. Borg index was higher in SSc-APAH (4.2±2.1 vs 3.3±1.5); p<0.049. The degree of heart failure (HF) and respiratory failure (RF) did not differ. Dyspnea was the most common among the first symptoms (94% in SSc-PAH and 73% in IPAH). Syncope were observed only in IPAH. In both the diagnosis was verified quite late (in SSc-PAH group in 33 (13; 56.5mts), IPAH - in 30.5 (13.3; 76.3mts). Signs of HF were detected in both groups with equal frequency (76% and 65% respectively). Advanced stage of PAH including symptoms of HF were detected with equal frequency. Only ECHO revealed pericardial effusion more frequently in pts with SSc-PAH (56% vs 35%, p<0.05). Also they had more frequently weight lost (14% vs 2%, p<0.05). Hemodynamics estimation revealed significant haemodynamic differences: sRVP (73.4±19.1 mm Hg) and mPAP (49.1±11.5 mm Hg) in SSc-PAH were significantly lower, than in IPAH (85.9±25.6 mm Hg and 57.5±15.3 mm Hg). dRVP and mRVP were significantly higher in SSc (7.02±5.59 and 28.5±12.7 mm Hg), compared to 0.95±7.6 and 11.7±6.0 mm Hg respectively. Whereas CO values were equal, the average PVR was higher in IPAH (13.2 vs 10.6 WU, р<0.005). RAP levels were equal at both groups. FVC was within normal limits in groups, but DLCO was lower in SSc-PAH group (46.9±13.5% vs 68.5±12.8%). Hb in SSc-APAH group was significantly lower (136±22 g/l vs 149±19 g/l), erythrocyte count was almost same is both groups (4.9±0.6 and 5.1±0.7×1012/l). C-reactive protein in patients with SSc-PAH was higher (9.4±15.1 vs 1.1±1.0 mg/l); p<0.001. Significant decrease in survival during the second and third years of observation was revealed in SSc-PAH group. Difference in 5 years survival between the two groups was 15%, difference was close to statistically significant (p=0.06). Conclusion: although general clinical appearance of patients with SSc-APAH and IPAH looks similar, there are significant differences in hemodynamic indexes and laboratory signs between the two groups. This factor leads to difference in progress and outcome of the disease.

About the Authors

N. N. Yudkina
V.A.Nasonova Research Institute of Rheumatology
Russian Federation


E. G. Valeeva
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


I. N. Taran
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


E. V. Nikolaeva
V.A.Nasonova Research Institute of Rheumatology
Russian Federation


V. M. Paramonov
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


I. A. Kurmukov
V.A.Nasonova Research Institute of Rheumatology
Russian Federation


Z. S. Valieva
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


O. A. Arkhipova
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


T. V. Martynyuk
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


A. V. Volkov
V.A.Nasonova Research Institute of Rheumatology
Russian Federation


E. L. Nasonov
V.A.Nasonova Research Institute of Rheumatology
Russian Federation


I. E. Chazova
A.L.Myasnikov Institute of Clinical Cardiology Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation
Russian Federation


References

1. Легочная гипертензия. Под ред. И.Е.Чазовой, Т.В.Мартынюк. М.: Практика, 2015.

2. Волков А.В. Легочная артериальная гипертензия при системных заболеваниях соединительной ткани. Научно - практ. ревматология. 2015; 1 (53): 69-77.

3. Galié N, Humbert M, Vachiery J.L 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): 903-75.

4. Fisher M.R, Mathai M.C, Champion H.C et al. Clinical differences between idiopathic and scleroderma - related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043-50. Doi: 10.1002/art.22069.

5. Launay D, Sitbon O, Le Pavec J et al. Long - term outcome of systemic sclerosis - associated pulmonary arterial hypertension treated with bosentan as first - line monotherapy followed or not by the addition of prostanoid or sildenafil. Rheumatology 2010: 49 (3): 490-500.

6. Le Roy E.C, Black C, Fleischmajer R et. al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15 (2): 202-5.

7. Mayes M.D. Scleroderma epidemiology. Rheum Dis Clin North Am 2003; 29 (2): 239-54.

8. Maricq H.R, Weinrich M.C, Keil J.E et al. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum 1989; 32 (8): 998-1006.

9. 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: 461-6.

10. Hachulla E, De Groote P, Gressin V et al. ItinОr AIR-SclОrodermie Study Group. The three - year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 2009; 60 (6): 1831-9.

11. Волков А.В., Мартынюк Т.В., Юдкина Н.Н. и др. Выживаемость пациентов с легочной артериальной гипертензией, ассоциированной с системной склеродермией. Терапевт. арх. 2012; 5: 24-8

12. 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: 1940-6.

13. Overbeek M.J, Lankhaar J.W, Westerhof N et al. Right ventricular contractility in systemic sclerosis - associated and idiopathic pulmonary arterial hypertension. Eur Respir J 2008; 31 (6): 1160-6. Doi: 10.1183/09031936.00135407.

14. Tedford R.J, Mudd J.O, Girgis R.E et al. Right ventricular dysfunction in systemic sclerosis - associated pulmonary arterial hypertension. Circ Heart Fail 2013; 6 (5): 953-63.

15. Kelemen B.W, Mathai S.C, Tedford R.J et al. Right ventricular remodeling in idiopathic and scleroderma - associated pulmonary arterial hypertension: two distinct phenotypes. Pulm Circ 2015; 5 (2): 327-34. Doi: 10.1086/680356.

16. 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.

17. Волков А.В., Юдкина Н.Н., Николаева Е.В. и др. Бозентан: существенное увеличение продолжительности жизни пациентов с легочной артериальной гипертонией, ассоциированной с системными ревматическими заболеваниями. Терапевт. арх. 2014; 86 (5): 32-9.

18. Волков А.В., Николаева Е.В., Юдкина Н.Н. и др. Влияние терапии силденафилом на выживаемость пациентов с легочной артериальной гипертензией, ассоциированной с системными заболеваниями соединительной ткани (результаты проспективного наблюдения). Терапевт. арх. 2015; 87 (11): 62-7.

19. Авдеев С.Н., Царева Н.А., Неклюдова Г.В., Чучалин А.Г. Первый клинический опыт применения антагониста рецепторов эндотелина бозентана у пациентов с легочной артериальной гипертензией: результаты годичного исследования. Терапевт. арх. 2013; 85 (3): 38-43.

20. Чазова И.Е., Авдеев С.Н., Царева Н.А. и др. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевт. арх. 2014; 9: 4-23.

21. Van den Hoogen F, Khanna D, Fransen J et al. Classification Criteria for Systemic Sclerosis: An ACR-EULAR Collaborative Initiative. Arthritis Rheum 2013; 65 (11): 2737-47.

22. ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six - minute walk test. Am J Respir Crit Care Med 2002; 166: 111-7.

23. Benza R.L, Gomberg-Maitland M, Miller D.P et al. The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension. Chest 2012; 141 (2): 354-62.

24. Chemla D, Castelain V, Herv О P et al. Haemodynamic evaluation of pulmonary hypertension. Eur Respir J 2002; 20: 1314-31.

25. Fisher M.R, Mathai S.C, Champion H.C et al. Clinical differences between idiopathic and scleroderma - related pulmonary hypertension. Arthritis Rheum 2006; 54 (9): 3043-50.

26. Clements P.J, Tan M, Mc Laughlin V.V et al. The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis - associated PAH. Ann Rheum Dis 2012; 71 (2): 249-52.

27. Rubin L.J, Badesch D.B, Barst R.J et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903.

28. Kuhn K.P, Byrne D.W, Arbogast P.G et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 2003; 167: 580-6.

29. Fernandes F, Ramires F.J, Arteaga E et al. Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J Card Fail 2003; 9: 311-7.

30. Chin K.M, Kim N.H, Rubin L.J. The right ventricle in pulmonary hypertension. Coron Artery Dis 2005; 16: 13-8.

31. Hinderliter A.L, Willis P.W, Long W et al. For the PPH Study Group. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension: primary pulmonary hypertension. Am J Cardiol 1999; 84: 481-4.

32. Miller A.J, Pick R, Johnson P.J. The production of acute pericardial effusion: the effects of various degrees of interference with venous blood and lymph drainage from the heart muscle in the dog. Am J Cardiol 1971; 28: 463-6.

33. Mellins R.B, Levine O.R, Fishman A.P. Effect of systemic and pulmonary venous hypertension on pleural and pericardial fluid accumulation. J Appl Physiol 1970; 29: 564-9.

34. Thompson A.E, Pope J.E. A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 1998; 37: 1320-3.

35. Simeon C.P, Armadans L, Fonollosa V et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 2003; 42: 71-5.

36. Ruiter G, Lankhorst S, Boonstra A et al. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 2011; 37 (6): 1386-91.

37. Rhodes C.J, Howard L.S, Busbridge M et al. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: Clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011; 58: 300-9.

38. Ruiter G, Lanser I.J, De Man F.S et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014; 53 (2): 285-92.

39. Takeda Y, Takeda Y, Tomimoto S et al. Bilirubin as a prognostic marker in patients with pulmonary arterial hypertension. BMC Pulm Med 2010; 10: 22.

40. Kubo S.H, Walter B.A, John D.H et al. Liver function abnormalities in chronic heart failure. Influence of systemic hemodynamics. Arch Intern Med 1987; 147: 1227-30.

41. Giallourakis C.C, Rosenberg P.M, Friedman L.S. The liver in heart failure. Clin Liver Dis 2002; 6: 947-67.

42. Nagaya N, Uematsu M, Satoh T et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160 (2): 487-92.

43. Leyva F, Anker S.D, Godsland I.F et al. Uric acid in chronic heart failure: A marker of chronic inflammation. Eur Heart J 1998; 19: 1814-22.

44. Kawut S.M, Taichman D.B, Archer-Chicko C.L et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123: 344-50.

45. Koh E.T, Lee P, Gladman D.D, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996; 35: 989-93.


Review

For citations:


Yudkina N.N., Valeeva E.G., Taran I.N., Nikolaeva E.V., Paramonov V.M., Kurmukov I.A., Valieva Z.S., Arkhipova O.A., Martynyuk T.V., Volkov A.V., Nasonov E.L., Chazova I.E. Demographic and clinical differences between idiopathic and scleroderma-related pulmonary arterial hypertension: Russian National Registry analysis. Systemic Hypertension. 2016;13(2):65-72. (In Russ.)

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