Clinical case of pulmonary hypertension, associated with rare lung disease
Article Sidebar
Main Article Content
Abstract
This article is clinical presentation of pulmonary hypertension case, associated with rare lung disease – lymphangioleiomyomatosis. There are elucidated: diagnostic criteria of this disease, its prevalence, pathogenesis, including mechanisms of pulmonary hypertension development, clinical presentations and methods of treatment. Authors concluded some special features of this case: rare disease that could be diagnosed by only experienced staff; late patient’s age is atypical for this disease (usual this disease is diagnosed in younger women (childbirth potential age); moderate lung function disorders were accompanied by severe pulmonary hypertension, that needed additional examination in expert center; in spite of general recommendations not to use specific therapy in patients with lung disease the prostaglandins were used in this patient with improving of clinical symptoms. Based on summary of 6th World Symposium on Pulmonary Hypertension (Nice, 2018) there were discussed the particularities of pulmonary hypertension diagnosis and treatment in patients with lung disease or chronic hypoxia (group 3) and their differences with pulmonary arterial hypertension (group 1). It was stressed the necessity of individual approaches in specific therapy using in patients with lung diseases and high pulmonary artery pressure and providing the clinical trials for evaluation of this therapy influence on prognosis.
Article Details
Keywords:
References
Редкие интерстициальные заболевания легких / Под ред. В. К. Гаврисюка.– К.: ООО Велес, 2012.– 148 с.
Уніфікований клінічний протокол екстреної, первинної, вторинної (спеціалізованої) та третинної (високоспеціалізованої) медичної допомоги. Легенева гіпертензія у дорослих. 2016 // Артеріальна гіпертензія.– 2016.– № 3.–С. 51–83.
Alkukhun L., Wang X.F., Ahmed M.K. et al. Non-invasive screening for pulmonary hypertension in idiopathic pulmonary fibrosis // Respir. Med.– 2016.– Vol. 117.– P. 65–72. doi: https://doi.org/10.1016/j.rmed.2016.06.001.
Andersen K.H., Iversen M., Kjaergaard J. et al. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease // J. Heart Lung Transplant.– 2012.– Vol. 31.– P. 373–380. doi: https://doi.org/10.1016/j.healun.2011.11.020.
Barbera J.A., Blanco I. Pulmonary hypertension in patients with chronic obstructive pulmonary disease: advances in pathophysiology and management // Drugs.– 2009.– Vol. 69.– P. 1153–1171 doi: https://doi.org/10.2165/00003495-200969090-00002.
Barnett C.F., Bonura E.J., Nathan S.D. et al. Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience // Chest.– 2009.– Vol. 135.– P. 1455–1461. doi: https://doi.org/10.1378/chest.08-1881.
Baughman R.P., Culver D.A., Cordova F.C. et al. Bosentan for sarcoidosis-associated pulmonary hypertension: a double-blind placebo controlled randomized trial // Chest.– 2014.– Vol. 145.– P. 810–817. doi: https://doi.org/10.1378/chest.13-1766.
Baughman R.P., Judson M.A., Lower E.E. et al. Inhaled iloprost for sarcoidosis associated pulmonary hypertension. Sarcoidosis // Vasc. Diffuse Lung Dis.– 2009.– Vol. 26.– P. 110–120. PMID:20560291.
Blanco I., Ribas J., Xaubet A. et al. Effects of inhaled nitric oxide at rest and during exercise in idiopathic pulmonary fibrosis // J. Appl. Physiol.– 2011.– Vol. 110.– P. 638–645. doi: https://doi.org/10.1152/japplphysiol.01104.2010.
Calcaianu G., Canuet M., Schuller A. et al. Pulmonary arterial hypertension-specific drug therapy in COPD patients with severe pulmonary hypertension and mild-to-moderate airflow limitation // Respiration.– 2016.– Vol. 91.– P. 9–17. doi: https://doi.org/10.1159/000441304.
Chaouat A., Bugnet A.–S., Kadaoui N. et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease // Amer. J. Respir. Crit. Care Med.– 2005.– 172.– P. 189–194. doi: https://doi.org/10.1164/rccm.200401-006OC.
Chen X., Tang S., Liu K. et al. Therapy in stable chronic obstructive pulmonary disease patients with pulmonary hypertension: a systematic review and meta-analysis // J. Thorac. Dis.– 2015.– Vol. 7.– P. 309–319. doi: https://doi.org/10.3978/j.issn.2072-1439.2015.02.08.
Corte T.J., Keir G.J., Dimopoulos K. et al.; BPHIT Study Group. Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia // Am. J. Respir. Crit Care. Med.– 2014.– Vol. 190.– P. 208–217. doi: https://doi.org/10.1164/rccm.201403-0446OC.
Cottin V., Harari S., Humbert M. et al.; Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM”O”P). Pulmonary hypertension in lymphangio leiomyomatosis: characteristics in 20 patients // Eur. Respir. J.– 2012.– Vol. 40.– P. 630–640. doi: https://doi.org/10.1183/09031936.00093111.
D’Andrea A., Stanziola A., Di Palma E. et al. Right ventricular structure and function in idiopathic pulmonary fibrosis with or without pulmonary hypertension // Echocardiography.– 2016.– Vol. 33.– P. 57–65. doi: https://doi.org/10.1111/echo.12992.
Fartoukh M., Humbert M., Capron F. et al. Severe pulmonary hypertension in histiocytosis X // Am. J. Respir. Crit. Care Med.– 2000.– Vol. 161.– P. 216–223. doi: https://doi.org/10.1164/ajrccm.161.1.9807024.
Furukawa T., Kondoh Y., Taniguchi H. et al. A scoring system to predict the elevation of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis // Eur. Respir. J.– 2018.– Vol. 51.– P. 1701311. doi: https://doi.org/10.1183/13993003.01311-2017.
Galiè 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. Heart J.– 2016.– Vol. 37 (1).– P. 67–119. doi: https://doi.org/10.1093/eurheartj/ehv317.
Galiè N., McLaughlin V.V., Rubin L.J., Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension // Eur. Respir. J.– 2019.– Vol. 53 (1).– P. 1802148. Published 2019 Jan 24. doi: https://doi.org/10.1183/13993003.02148-2018.
Ghofrani H.A., Wiedemann R., Rose F. et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial // Lancet.– 2002.– Vol. 360.– P. 895–900. doi: https://doi.org/10.1016/S0140-6736(02)11024-5.
Glaser S., Obst A., Koch B. et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis – the predictive value of exercise capacity and gas exchange efficiency // PLoS One.– 2013.– Vol. 8.– P. e65643. doi: https://doi.org/10.1371/journal.pone.0065643.
Greiner S., Jud A., Aurich M. et al. Reliability of noninvasive assessment of systolic pulmonary artery pressure by Doppler echocardiography compared to right heart catheterization: analysis in a large patient population // J. Am. Heart Assoc.– 2014.– Vol. 3.– P. e001103. doi: https://doi.org/10.1161/JAHA.114.001103.
Hoeper M.M., Behr J., Held M. et al. Pulmonary hypertension in patients with chronic fibrosing idiopathic interstitial pneumonias // PLoS One.– 2015.– Vol. 10.– P. e0141911. doi: https://doi.org/10.1371/journal.pone.0141911.
Hoeper M.M., Halank M., Wilkens H. et al. Riociguat for interstitial lung disease and pulmonary hypertension: a pilot trial // Eur. Respir. J.– 2013.– Vol. 41.– P. 853–860. doi: https://doi.org/10.1183/09031936.00213911.
Harari S., Torre O., Moss J. Lymphangioleiomyomatosis: What do we know and what we looking for? // Eur. Respir. Rev.– 2011.– Vol. 20 (119).– P. 34–44. doi: https://doi.org/10.1183/09059180.00011010.
Johnson S.R., Cordier J.F., Lazor R. et al. European Respira-tory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis // Eur. Respir. J.– 2010.– Vol. 35.– P. 14–26. doi: https://doi.org/10.1183/09031936.00076209.
Lange T.J., Baron M., Seiler I. et al. Outcome of patients with severe PH due to lung disease with and without targeted therapy // Cardiovasc. Ther.– 2014.– Vol. 32.– P. 202–208. doi: https://doi.org/10.1111/1755-5922.12084.
Le Pavec J., Lorillon G., Jais X. et al. Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies // Chest.– 2012.– Vol. 142.– P. 1150–1157. doi: https://doi.org/10.1378/chest.11-2490.
Lederer D.J., Bartels M.N., Schluger N.W. et al. Sildenafil for chronic obstructive pulmonary disease: a randomized crossover trial // COPD.– 2012.– Vol. 9.– P. 268–275. doi: https://doi.org/10.3109/15412555.2011.651180.
Leuchte H.H., Baumgartner R.A., Nounou M.E. et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease // Am. J. Respir. Crit. Care Med.– 2006.– Vol. 173.– P. 744–750. doi: 10.1164/rccm.200510-1545OC.
Nadrous H.F., Pellikka P.A., Krowka M.J. et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis // Chest.– 2005.– Vol. 128.– P. 2393–2399. doi: https://doi.org/10.1378/chest.128.4.2393.
Nathan S., Barbera J., Gaine S. et al. Number 10 in the series «Proceedings of the 6th World Symposium on Pulmonary Hypertension» / Ed. N. Galiè, V.V. McLaughlin, L.J. Rubin, G. Simonneau. Pulmonary hypertension in chronic lung disease and hypoxia // Eur. Respir. J.– 2019.– Vol. 53.– P. 1801–1914. doi: https://doi.org/10.1183/13993003.01914-2018.
Nathan S.D., Behr J., Collard H.R. et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study // Lancet Respir. Med.– 2019.– Vol. 7 (9).– P. 780–790. doi: https://doi.org/10.1016/S2213-2600(19)30250-4.
Nowak J., Hudzik B., Jastrze Bski D. et al. Pulmonary hypertension in advanced lung diseases: echocardiography as an important part of patient evaluation for lung transplantation // Clin. Respir. J.– 2018.– Vol. 12.– P. 930–938. doi: https://doi.org/10.1111/crj.12608.
Nunes H., Humbert M., Capron F. et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis // Thorax.– 2006.– Vol. 61.– P. 68–74. doi: https://doi.org/10.1136/thx.2005.042838.
Olschewski H., Ghofrani H.A., Walmrath D. et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis // Am. J. Respir. Crit Care Med.– 1999.– Vol. 160.– P. 600–607. doi: https://doi.org/10.1164/ajrccm.160.2.9810008.
Prins K.W., Duval S., Markowitz J. et al. Chronic use of PAH-specific therapy in World Health Organization Group III Pulmonary Hypertension: a systematic review and meta-analysis // Pulm. Circ.– 2017.– Vol. 7.– P. 145–155. doi: https://doi.org/10.1086/690017.
Raghu G., Behr J., Brown K.K. et al. ARTEMIS-IPF Investigators. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial // Ann. Intern. Med.– 2013.– Vol. 158.– P. 641–649. doi: https://doi.org/10.7326/0003-4819-158-9-201305070-00003.
Raghu G., Nathan S.D., Behr J. et al. Pulmonary hypertension in idiopathic pulmonary fibrosis with mild to moderate restriction // Eur. Respir. J.– 2015.– Vol. 46.– P. 1370–1377. doi: https://doi.org/10.1183/13993003.01537-2014.
Ryu J.H., Moss J., Beck G. The NHLBI lymphangioleiomyomatosis registry: Characteristies of 230 patients at enrollment // Am. J. Respir. Crit. Care Med.– 2006.– Vol. 173 (1).– P. 105–111. doi: https://doi.org/10.1164/rccm.200409-1298OC.
Saggar R., Khanna D., Vaidya A. et al. Changes in right heart haemodynamics and echocardiographic function in an advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis // Thorax.– 2014.– Vol. 69.– P. 123–129. doi: https://doi.org/10.1136/thoraxjnl-2013-204150.
Shorr A.F., Wainright J.L., Cors C.S. et al. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant // Eur. Respir. J.– 2007.– Vol. 30.– P. 715–721. doi: https://doi.org/10.1183/09031936.00107206.
The Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman D.A., Schwarz M., Anstrom K.J., Collard H.R., Flaherty K.R., Hunninghake G.W. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis // New Engl. J. Med.– 2010.– Vol. 363.– P. 620–628. doi: https://doi.org/10.1056/NEJMoa1002110.
Valerio G., Bracciale P., Grazia D.A. Effect of bosentan upon pulmonary hypertension in chronic obstructive pulmonary disease // Ther. Adv. Respir. Dis.– 2009.– Vol. 3.– P. 15–21. doi: https://doi.org/10.1177/1753465808103499.
Vitulo P., Stanziola A., Confalonieri M. et al. Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: a randomized controlled multicenter clinical trial // J. Heart Lung. Transplant.– 2017.– Vol. 36.– P. 166–174. doi: https://doi.org/10.1016/j.healun.2016.04.010
Zhang X., Travis W. Pulmonary Lymphangioleiomyomatosis // Arch. Pathol. Lab. Med.– 2010.– Vol. 134.– P. 1823–1828. doi: https://doi.org/10.1043/2009-0576-RS.1.