Pulmonary hypertension in ANCA-associated vasculitis: a retrospective analysis Pulmonary Hypertension in AAV
Main Article Content
Keywords
antineutrophil cytoplasmic antibody, pulmonary hypertension, vasculitis
Abstract
Background: Little is known about pulmonary hypertension (PH) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Objectives: The aims of this retrospective study, in which echocardiography was used for detection of PH, were to identify the potential causes of PH in AAV and to analyze the risk factors for mortality.
Methods: We performed a retrospective descriptive review of 97 patients who had AAV with PH at our institution from January 1, 1997, through December 31, 2015. These patients with PH were compared with 558 patients who had AAV without PH. Demographic and clinical data were abstracted from electronic health records.
Results: Among the patients who had PH, 61% were men; mean (SD) age was 70.5 (14.1) years at the time of PH diagnosis. The majority of patients with PH (73.2%) had more than 1 potential cause of PH, with left heart disease and chronic lung disease being the most common causes. Older age, male sex, smoking history, and kidney involvement were associated with the presence of PH. PH was associated with an increased risk of death (hazard ratio, 3.15; 95% CI, 2.37-4.18). On multivariate analysis, PH, age, smoking status, and kidney involvement were independent risk factors for death. Median survival after the diagnosis of PH was 25.9 months (95% CI, 12.2-49.9).
Conclusions: PH in AAV is often multifactorial, is commonly associated with left heart disease, and is associated with a poor prognosis.
References
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8. Fukumoto Y. Takayasu arteritis-associated pulmonary hypertension. Eur Heart J. 2021;42(42):4306-8.
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23. Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003;41(4):776-84.
24. Mahr A, Girard T, Agher R, Guillevin L. Analysis of factors predictive of survival based on 49 patients with systemic Wegener's granulomatosis and prospective follow-up. Rheumatology (Oxford). 2001;40(5):492-8.
25. Wang Q, van Timmeren MM, Petersen AH, Yuan J, Moser J, Brouwer E, et al. Age-determined severity of anti-myeloperoxidase autoantibody-mediated glomerulonephritis in mice. Nephrol Dial Transplant. 2017;32(2):254-64.
26. Chen M, Yu F, Zhang Y, Zhao MH. Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients. Medicine (Baltimore). 2008;87(4):203-9.
27. Weiner M, Goh SM, Mohammad AJ, Hruskova Z, Tanna A, Bruchfeld A, et al. Outcome and treatment of elderly patients with ANCA-associated vasculitis. Clin J Am Soc Nephrol. 2015;10(7):1128-35.
28. Hoganson DD, From AM, Michet CJ. ANCA vasculitis in the elderly. J Clin Rheumatol. 2008;14(2):78-81.
29. Jayne D. Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 2003;26(4):231-9.
30. Wallace ZS, Fu X, Harkness T, Stone JH, Zhang Y, Choi H. All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type. Rheumatology (Oxford). 2020;59(9):2308-15.
31. Berti A, Felicetti M, Monti S, Ortolan A, Padoan R, Brunori G, et al. Disease and treatment-related morbidity in young and elderly patients with granulomatosis with polyangiitis and microscopic polyangiitis. Semin Arthritis Rheum. 2020;50(6):1441-8.
32. Berti A, Caporali R, Montecucco C, Paolazzi G, Monti S. Aging in Primary Systemic Vasculitis: Implications for Diagnosis, Clinical Manifestations, and Management. Drugs Aging. 2019;36(1):53-63.
33. Higgins RM, Goldsmith DJ, Connolly J, Scoble JE, Hendry BM, Ackrill P, et al. Vasculitis and rapidly progressive glomerulonephritis in the elderly. Postgrad Med J. 1996;72(843):41-4.
34. Strange G, Playford D, Stewart S, Deague JA, Nelson H, Kent A, et al. Pulmonary hypertension: prevalence and mortality in the Armadale echocardiography cohort. Heart. 2012;98(24):1805-11.
35. Schiess R, Senn O, Fischler M, Huber LC, Vatandaslar S, Speich R, et al. Tobacco smoke: a risk factor for pulmonary arterial hypertension? A case-control study. Chest. 2010;138(5):1086-92.
36. Wright JL, Dai J, Zay K, Price K, Gilks CB, Churg A. Effects of cigarette smoke on nitric oxide synthase expression in the rat lung. Lab Invest. 1999;79(8):975-83.
37. Wright JL, Farmer SG, Churg A. A neutrophil elastase inhibitor reduces cigarette smoke-induced remodelling of lung vessels. Eur Respir J. 2003;22(1):77-81.
38. Santos S, Peinado VI, Ramirez J, Morales-Blanhir J, Bastos R, Roca J, et al. Enhanced expression of vascular endothelial growth factor in pulmonary arteries of smokers and patients with moderate chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2003;167(9):1250-6.
39. Barbera JA, Peinado VI, Santos S, Ramirez J, Roca J, Rodriguez-Roisin R. Reduced expression of endothelial nitric oxide synthase in pulmonary arteries of smokers. Am J Respir Crit Care Med. 2001;164(4):709-13.
40. Wright JL, Tai H, Churg A. Vasoactive mediators and pulmonary hypertension after cigarette smoke exposure in the guinea pig. J Appl Physiol (1985). 2006;100(2):672-8.
41. Goerre S, Staehli C, Shaw S, Luscher TF. Effect of cigarette smoking and nicotine on plasma endothelin-1 levels. J Cardiovasc Pharmacol. 1995;26 Suppl 3:S236-8.
42. Santos S, Peinado VI, Ramirez J, Melgosa T, Roca J, Rodriguez-Roisin R, et al. Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J. 2002;19(4):632-8.
43. Akmal M, Barndt RR, Ansari AN, Mohler JG, Massry SG. Excess PTH in CRF induces pulmonary calcification, pulmonary hypertension and right ventricular hypertrophy. Kidney Int. 1995;47(1):158-63.
44. Yigla M, Fruchter O, Aharonson D, Yanay N, Reisner SA, Lewin M, et al. Pulmonary hypertension is an independent predictor of mortality in hemodialysis patients. Kidney Int. 2009;75(9):969-75.
45. Havlucu Y, Kursat S, Ekmekci C, Celik P, Serter S, Bayturan O, et al. Pulmonary hypertension in patients with chronic renal failure. Respiration. 2007;74(5):503-10.
46. Issa N, Krowka MJ, Griffin MD, Hickson LJ, Stegall MD, Cosio FG. Pulmonary hypertension is associated with reduced patient survival after kidney transplantation. Transplantation. 2008;86(10):1384-8.
47. Abdelwhab S, Elshinnawy S. Pulmonary hypertension in chronic renal failure patients. Am J Nephrol. 2008;28(6):990-7.
48. Pabst S, Hammerstingl C, Hundt F, Gerhardt T, Grohe C, Nickenig G, et al. Pulmonary hypertension in patients with chronic kidney disease on dialysis and without dialysis: results of the PEPPER-study. PLoS One. 2012;7(4):e35310.
49. Nakhoul F, Yigla M, Gilman R, Reisner SA, Abassi Z. The pathogenesis of pulmonary hypertension in haemodialysis patients via arterio-venous access. Nephrol Dial Transplant. 2005;20(8):1686-92.
50. Ressl J, Urbanova D, Widimsky J, Ostadal B, Pelouch V, Prochazka J. Reversibility of pulmonary hypertension and right ventricular hypertrophy induced by intermittent high altitude hypoxia in rats. Respiration. 1974;31(1):38-46.
51. Sica AL, Greenberg HE, Ruggiero DA, Scharf SM. Chronic-intermittent hypoxia: a model of sympathetic activation in the rat. Respir Physiol. 2000;121(2-3):173-84.
52. Wijeratne DT, Lajkosz K, Brogly SB, Lougheed MD, Jiang L, Housin A, et al. Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension: A Population-Based Cohort Study in Ontario, Canada. Circ Cardiovasc Qual Outcomes. 2018;11(2):e003973.
53. Yanda RJ, Guis MS, Rabkin JM. Aortic valvulitis in a patient with Wegener's granulomatosis. West J Med. 1989;151(5):555-6.
54. Gerbracht DD, Savage RW, Scharff N. Reversible valvulitis in Wegener's granulomatosis. Chest. 1987;92(1):182-3.
2. Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, 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 Heart J. 2016;37(1):67-119.
3. Denton CP, Cailes JB, Phillips GD, Wells AU, Black CM, Bois RM. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol. 1997;36(2):239-43.
4. Gabbay E, Reed A, Williams TJ. Assessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006. Intern Med J. 2007;37(1):38-48.
5. Mukerjee D, St George D, Knight C, Davar J, Wells AU, Du Bois RM, et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford). 2004;43(4):461-6.
6. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med. 1995;333(4):214-21.
7. Jiang X, Zhu YJ, Zhou YP, Peng FH, Wang L, Ma W, et al. Clinical features and survival in Takayasu's arteritis-associated pulmonary hypertension: a nationwide study. Eur Heart J. 2021;42(42):4298-305.
8. Fukumoto Y. Takayasu arteritis-associated pulmonary hypertension. Eur Heart J. 2021;42(42):4306-8.
9. Marette P, Molle B, Mas JL, Kahan A, Devaux JY, Toussaint M, et al. [Angina without involvement of large coronary trunks and precapillary pulmonary hypertension in a patient with periarteritis nodosa]. Ann Cardiol Angeiol (Paris). 1989;38(6):305-8.
10. Launay D, Souza R, Guillevin L, Hachulla E, Pouchot J, Simonneau G, et al. Pulmonary arterial hypertension in ANCA-associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23(3):223-8.
11. Li Y, Yi Q. Pulmonary arterial hypertension associated with rare cause of ANCA-associated vasculitis misdiagnosed as idiopathic one. Int J Clin Exp Med. 2015;8(9):16850-3.
12. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11.
13. Augustine DX, Coates-Bradshaw LD, Willis J, Harkness A, Ring L, Grapsa J, et al. Echocardiographic assessment of pulmonary hypertension: a guideline protocol from the British Society of Echocardiography. Echo Res Pract. 2018;5(3):G11-G24.
14. Nagueh SF, Smiseth OA, Appleton CP, Byrd BF, 3rd, Dokainish H, Edvardsen T, et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29(4):277-314.
15. Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671-8.
16. Stone JH, Hoffman GS, Merkel PA, Min YI, Uhlfelder ML, Hellmann DB, et al. A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum. 2001;44(4):912-20.
17. McDermott G, Fu X, Stone JH, Wallwork R, Zhang Y, Choi HK, et al. Association of Cigarette Smoking With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. JAMA Intern Med. 2020;180(6):870-6.
18. Mohammad AJ, Segelmark M. A population-based study showing better renal prognosis for proteinase 3 antineutrophil cytoplasmic antibody (ANCA)-associated nephritis versus myeloperoxidase ANCA-associated nephritis. J Rheumatol. 2014;41(7):1366-73.
19. Berti A, Cornec-Le Gall E, Cornec D, Casal Moura M, Matteson EL, Crowson CS, et al. Incidence, prevalence, mortality and chronic renal damage of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in a 20-year population-based cohort. Nephrol Dial Transplant. 2019;34(9):1508-17.
20. Bai YH, Li ZY, Chang DY, Chen M, Kallenberg CG, Zhao MH. The BVAS is an independent predictor of cardiovascular events and cardiovascular disease-related mortality in patients with ANCA-associated vasculitis: A study of 504 cases in a single Chinese center. Semin Arthritis Rheum. 2018;47(4):524-9.
21. Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009;48(12):1560-5.
22. Harper L, Savage CO. ANCA-associated renal vasculitis at the end of the twentieth century--a disease of older patients. Rheumatology (Oxford). 2005;44(4):495-501.
23. Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003;41(4):776-84.
24. Mahr A, Girard T, Agher R, Guillevin L. Analysis of factors predictive of survival based on 49 patients with systemic Wegener's granulomatosis and prospective follow-up. Rheumatology (Oxford). 2001;40(5):492-8.
25. Wang Q, van Timmeren MM, Petersen AH, Yuan J, Moser J, Brouwer E, et al. Age-determined severity of anti-myeloperoxidase autoantibody-mediated glomerulonephritis in mice. Nephrol Dial Transplant. 2017;32(2):254-64.
26. Chen M, Yu F, Zhang Y, Zhao MH. Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients. Medicine (Baltimore). 2008;87(4):203-9.
27. Weiner M, Goh SM, Mohammad AJ, Hruskova Z, Tanna A, Bruchfeld A, et al. Outcome and treatment of elderly patients with ANCA-associated vasculitis. Clin J Am Soc Nephrol. 2015;10(7):1128-35.
28. Hoganson DD, From AM, Michet CJ. ANCA vasculitis in the elderly. J Clin Rheumatol. 2008;14(2):78-81.
29. Jayne D. Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 2003;26(4):231-9.
30. Wallace ZS, Fu X, Harkness T, Stone JH, Zhang Y, Choi H. All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type. Rheumatology (Oxford). 2020;59(9):2308-15.
31. Berti A, Felicetti M, Monti S, Ortolan A, Padoan R, Brunori G, et al. Disease and treatment-related morbidity in young and elderly patients with granulomatosis with polyangiitis and microscopic polyangiitis. Semin Arthritis Rheum. 2020;50(6):1441-8.
32. Berti A, Caporali R, Montecucco C, Paolazzi G, Monti S. Aging in Primary Systemic Vasculitis: Implications for Diagnosis, Clinical Manifestations, and Management. Drugs Aging. 2019;36(1):53-63.
33. Higgins RM, Goldsmith DJ, Connolly J, Scoble JE, Hendry BM, Ackrill P, et al. Vasculitis and rapidly progressive glomerulonephritis in the elderly. Postgrad Med J. 1996;72(843):41-4.
34. Strange G, Playford D, Stewart S, Deague JA, Nelson H, Kent A, et al. Pulmonary hypertension: prevalence and mortality in the Armadale echocardiography cohort. Heart. 2012;98(24):1805-11.
35. Schiess R, Senn O, Fischler M, Huber LC, Vatandaslar S, Speich R, et al. Tobacco smoke: a risk factor for pulmonary arterial hypertension? A case-control study. Chest. 2010;138(5):1086-92.
36. Wright JL, Dai J, Zay K, Price K, Gilks CB, Churg A. Effects of cigarette smoke on nitric oxide synthase expression in the rat lung. Lab Invest. 1999;79(8):975-83.
37. Wright JL, Farmer SG, Churg A. A neutrophil elastase inhibitor reduces cigarette smoke-induced remodelling of lung vessels. Eur Respir J. 2003;22(1):77-81.
38. Santos S, Peinado VI, Ramirez J, Morales-Blanhir J, Bastos R, Roca J, et al. Enhanced expression of vascular endothelial growth factor in pulmonary arteries of smokers and patients with moderate chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2003;167(9):1250-6.
39. Barbera JA, Peinado VI, Santos S, Ramirez J, Roca J, Rodriguez-Roisin R. Reduced expression of endothelial nitric oxide synthase in pulmonary arteries of smokers. Am J Respir Crit Care Med. 2001;164(4):709-13.
40. Wright JL, Tai H, Churg A. Vasoactive mediators and pulmonary hypertension after cigarette smoke exposure in the guinea pig. J Appl Physiol (1985). 2006;100(2):672-8.
41. Goerre S, Staehli C, Shaw S, Luscher TF. Effect of cigarette smoking and nicotine on plasma endothelin-1 levels. J Cardiovasc Pharmacol. 1995;26 Suppl 3:S236-8.
42. Santos S, Peinado VI, Ramirez J, Melgosa T, Roca J, Rodriguez-Roisin R, et al. Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J. 2002;19(4):632-8.
43. Akmal M, Barndt RR, Ansari AN, Mohler JG, Massry SG. Excess PTH in CRF induces pulmonary calcification, pulmonary hypertension and right ventricular hypertrophy. Kidney Int. 1995;47(1):158-63.
44. Yigla M, Fruchter O, Aharonson D, Yanay N, Reisner SA, Lewin M, et al. Pulmonary hypertension is an independent predictor of mortality in hemodialysis patients. Kidney Int. 2009;75(9):969-75.
45. Havlucu Y, Kursat S, Ekmekci C, Celik P, Serter S, Bayturan O, et al. Pulmonary hypertension in patients with chronic renal failure. Respiration. 2007;74(5):503-10.
46. Issa N, Krowka MJ, Griffin MD, Hickson LJ, Stegall MD, Cosio FG. Pulmonary hypertension is associated with reduced patient survival after kidney transplantation. Transplantation. 2008;86(10):1384-8.
47. Abdelwhab S, Elshinnawy S. Pulmonary hypertension in chronic renal failure patients. Am J Nephrol. 2008;28(6):990-7.
48. Pabst S, Hammerstingl C, Hundt F, Gerhardt T, Grohe C, Nickenig G, et al. Pulmonary hypertension in patients with chronic kidney disease on dialysis and without dialysis: results of the PEPPER-study. PLoS One. 2012;7(4):e35310.
49. Nakhoul F, Yigla M, Gilman R, Reisner SA, Abassi Z. The pathogenesis of pulmonary hypertension in haemodialysis patients via arterio-venous access. Nephrol Dial Transplant. 2005;20(8):1686-92.
50. Ressl J, Urbanova D, Widimsky J, Ostadal B, Pelouch V, Prochazka J. Reversibility of pulmonary hypertension and right ventricular hypertrophy induced by intermittent high altitude hypoxia in rats. Respiration. 1974;31(1):38-46.
51. Sica AL, Greenberg HE, Ruggiero DA, Scharf SM. Chronic-intermittent hypoxia: a model of sympathetic activation in the rat. Respir Physiol. 2000;121(2-3):173-84.
52. Wijeratne DT, Lajkosz K, Brogly SB, Lougheed MD, Jiang L, Housin A, et al. Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension: A Population-Based Cohort Study in Ontario, Canada. Circ Cardiovasc Qual Outcomes. 2018;11(2):e003973.
53. Yanda RJ, Guis MS, Rabkin JM. Aortic valvulitis in a patient with Wegener's granulomatosis. West J Med. 1989;151(5):555-6.
54. Gerbracht DD, Savage RW, Scharff N. Reversible valvulitis in Wegener's granulomatosis. Chest. 1987;92(1):182-3.
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Baqir M, Singam NS, DuBrock H. Pulmonary hypertension in ANCA-associated vasculitis: a retrospective analysis: Pulmonary Hypertension in AAV. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2023 Jun. 29 [cited 2024 Jul. 17];40(2):e2023020. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/13631
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Baqir M, Singam NS, DuBrock H. Pulmonary hypertension in ANCA-associated vasculitis: a retrospective analysis: Pulmonary Hypertension in AAV. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2023 Jun. 29 [cited 2024 Jul. 17];40(2):e2023020. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/13631
