Sarcoidosis associated pulmonary hypertension
DOI:
https://doi.org/10.18203/2320-6012.ijrms20242972Keywords:
Sarcoidosis, Pulmonary hypertension, Sarcoidosis associated pulmonary hypertensionAbstract
Sarcoidosis is a multisystem inflammatory disease of unknown etiology that can be manifested as non-caseating granulomas, predominantly in the lungs and intrathoracic lymph nodes. Pulmonary hypertension is known as one of the complications of sarcoidosis and is associated with increased mortality. The incidence of sarcoidosis associated pulmonary hypertension (SAPH) varies between 5 to 20% and the prevalence is unknown. SAPH is classified as group V because of its multifactorial mechanism, it can be caused by pulmonary arterial hypertension, sarcoid cardiomyopathy, hypoxia, chronic thromboembolic pulmonary hypertension disease, and other mechanism like thoracic lymphadenopathy and fibrosing mediastinal, and increasing pulmonary vasoreactivity. SAPH patients have worsening dyspnea or sometimes signs of right-sided heart failure. Patients with SAPH reduced in pulmonary function test (6-minute walk distance (6MWD), diffusing capacity for carbon monoxide (DLCO), another test can be done are chest X-ray, high-resolution chest computed tomography (CT) scan, echocardiography, and right heart catheterization (RHC) as gold standard. The optimal management strategy for pulmonary hypertension associated with sarcoidosis is still unknown. The treatment involved in use of supplemental oxygen, systemic anti-inflammatory medication, pulmonary vasodilators, prostacyclin, endothelin receptor antagonists, phosphodiesterase 5 inhibitors even lung transplantation. SAPH is associated with significant morbidity and mortality. Patients with SAPH have 7-fold increase in risk for all-cause mortality when compare to sarcoidosis patients without PH, even when adjusted for age and pulmonary function.
References
Medscape. Pulmonary Arterial Hypertension: Practice Essentials, Pathophysiology, Etiology. Available at: https://emedicine.medscape.com/ article/303098-overview. Accessed on 09 July 2024.
Duong HT, Bonham CA. Sarcoidosis-associated Pulmonary Hypertension: Pathophysiology, Diagnosis, and Treatment. Clin Pulm Med. 2018;25(2):52.
Bonham CA, Oldham JM, Gomberg-Maitland M, Vij R. Prostacyclin and oral vasodilator therapy in sarcoidosis-associated pulmonary hypertension: a retrospective case series. Chest. 2015;148(4):1055-62.
Dickinson MG, Bartelds B, Borgdorff MAJ, Berger RMF. The role of disturbed blood flow in the development of pulmonary arterial hypertension: lessons from preclinical animal models. Am J Physiol Lung Cell Mol Physiol. 2013;305(1):L1-14.
Cool CD, Stewart JS, Werahera P, Miller GJ, Williams RL, Voelkel NF, et al. Three-dimensional reconstruction of pulmonary arteries in plexiform pulmonary hypertension using cell-specific markers. Evidence for a dynamic and heterogeneous process of pulmonary endothelial cell growth. Am J Pathol. 1999;155(2):411-9.
Siasos G, Tousoulis D, Gialafos E, Oikonomou E, Zaromitidou M, Aggeli C, et al. Association of Sarcoidosis With Endothelial Function, Arterial Wall Properties, and Biomarkers of Inflammation. Am J Hypertens. 2011;24(6):647-53.
Patel MR, Cawley PJ, Heitner JF, Klem I, Parker MA, Jaroudi WA, et al. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009;120(20):1969-77.
Trivieri MG, Spagnolo P, Birnie D, Liu P, Drake W, Kovacic JC, et al. Challenges in Cardiac and Pulmonary Sarcoidosis: JACC State-of-the-Art Review. J Am Coll Cardiol. 2020;76(16):1878-901.
Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest. 2010;138(5):1078-85.
Sulica R, Teirstein AS, Kakarla S, Nemani N, Behnegar A, Padilla ML. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest. 2005;128(3):1483-9.
Zisman DA, Karlamangla AS, Ross DJ, Keane MP, Belperio JA, Saggar R, et al. High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2007;132(3):773-9.
Lal C, Medarov BI, Judson MA. Interrelationship between sleep-disordered breathing and sarcoidosis. Chest. 2015;148(4):1105-14.
Charolidi N, Carroll VA, Charolidi N, Carroll VA. Hypoxia and Pulmonary Hypertension. In: Hypoxia and Human Diseases. IntechOpen. 2017. Available at: https://www.intechopen.com/chapters/53817. Accessed on 09 July 2024.
Ball MK, Waypa GB, Mungai PT, Nielsen JM, Czech L, Dudley VJ, et al. Regulation of Hypoxia-induced Pulmonary Hypertension by Vascular Smooth Muscle Hypoxia-Inducible Factor-1α. Am J Respir Crit Care Med. 2014;189(3):314-24.
Goljan-Geremek A, Geremek M, Puscinska E, Sliwinski P. Venous thromboembolism and sarcoidosis: co-incidence or coexistence? Cent-Eur J Immunol. 2015;40(4):477-80.
16. Alvarez RA, Barbash IJ, Rose JJ. Bosentan for sarcoidosis-associated pulmonary hypertension, age-adjusted D-dimer levels in pulmonary embolism, and mean arterial blood pressure targets in septic shock. Am J Respir Crit Care Med. 2014;190(8):948-9.
Marc K, Bourkadi JE, Benamor J, Iraqi G. Thoracic venous thrombosis in the course of sarcoidosis. Rev Mal Respir. 2008;25(1):105-6.
Damuth TE, Bower JS, Cho K, Dantzker DR. Major pulmonary artery stenosis causing pulmonary hypertension in sarcoidosis. Chest. 1980;78(6):888-91.
Preston IR, Klinger JR, Landzberg MJ, Houtchens J, Nelson D, Hill NS. Vasoresponsiveness of sarcoidosis-associated pulmonary hypertension. Chest. 2001;120(3):866-72.
Salazar A, Mañá J, Sala J, Landoni BR, Manresa F. Combined portal and pulmonary hypertension in sarcoidosis. Respir Int Rev Thorac Dis. 1994;61(2):117-9.
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D42-50.
Kabitz HJ, Lang F, Walterspacher S, Sorichter S, Müller-Quernheim J, Windisch W. Impact of impaired inspiratory muscle strength on dyspnea and walking capacity in sarcoidosis. Chest. 2006;130(5):1496-502.
Colman R, Whittingham H, Tomlinson G, Granton J. Utility of the physical examination in detecting pulmonary hypertension. A mixed methods study. PloS One. 2014;9(10):e108499.
Crouser ED, Maier LA, Wilson KC, Bonham CA, Morgenthau AS, Patterson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;201(8):e26-51.
Bourbonnais JM, Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J. 2008;32(2):296-302.
Jose A, Delio J, Gwizdala J, Goulart H, Ahari JE. Predictive value of pulmonary function testing in the evaluation of pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2018;35(4):308-16.
Crapo RO, Forster RE. Carbon monoxide diffusing capacity. Clin Chest Med. 1989;10(2):187-98.
Lower EE, Smith JT, Martelo OJ, Baughman RP. The anemia of sarcoidosis. Sarcoidosis. 1988;5(1):51-5.
Farha S, Asosingh K, Xu W, Sharp J, George D, Comhair S, et al. Hypoxia-inducible factors in human pulmonary arterial hypertension: a link to the intrinsic myeloid abnormalities. Blood. 2011;117(13):3485-93.
Ng CS, Wells AU, Padley SP. A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary artery to aortic diameter. J Thorac Imaging. 1999;14(4):270-8.
Nunes H, Humbert M, Capron F, Brauner M, Sitbon O, Battesti JP, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006;61(1):68-74.
Handa T, Nagai S, Miki S, Fushimi Y, Ohta K, Mishima M, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006;129(5):1246-52.
Bossone E, Bodini BD, Mazza A, Allegra L. Pulmonary arterial hypertension: the key role of echocardiography. Chest. 2005;127(5):1836-43.
Diaz-Guzman E, Farver C, Parambil J, Culver DA. Pulmonary hypertension caused by sarcoidosis. Clin Chest Med. 2008;29(3):549.
Nair R, Lamaa N. Pulmonary Capillary Wedge Pressure. Treasure Island (FL): StatPearls Publishing. 2023.
Savale L, Huitema M, Shlobin O, Kouranos V, Nathan SD, Nunes H, et al. WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension. Eur Respir Rev. 2022;31:163.
Galiè 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.
Preston IR, Klinger JR, Landzberg MJ, Houtchens J, Nelson D, Hill NS. Vasoresponsiveness of sarcoidosis-associated pulmonary hypertension. Chest. 2001;120(3):866-72.
Abston E, Moll M, Hon S, Govender P, Berman J, Farber H. Long-term outcomes of epoprostenol therapy in sarcoid associated pulmonary hypertension. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(2):184-91.
Baughman RP, Judson MA, Lower EE, Highland K, Kwon S, Craft N, et al. Inhaled iloprost for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc Diffuse Lung Dis Off J WASOG. 2009;26(2):110-20.
Palmer SM, Robinson LJ, Wang A, Gossage JR, Bashore T, Tapson VF. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest. 1998;113(1):237-40.
Judson MA, Highland KB, Kwon S, Donohue JF, Aris R, Craft N, et al. Ambrisentan for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc Diffuse Lung Dis Off J WASOG. 2011;28(2):139-45.
Mathijssen H, Huitema MP, Bakker ALM, Mager JJ, Snijder RJ, Grutters JC, et al. Safety of macitentan in sarcoidosis-associated pulmonary hypertension: a case-series. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(1):74-8.
Milman N, Burton CM, Iversen M, Videbaek R, Jensen CV, Carlsen J. Pulmonary hypertension in end-stage pulmonary sarcoidosis: therapeutic effect of sildenafil? J Heart Lung Transplant Off Publ Int Soc Heart Transplant. 2008;27(3):329-34.
Ford HJ, Baughman RP, Aris R, Engel P, Donohue JF. Tadalafil Therapy for Sarcoidosis-Associated Pulmonary Hypertension. Pulm Circ. 2016;6(4):557-62.
Baughman RP, Shlobin OA, Gupta R, Engel P, Stewart JI, Lower EE, et al. Riociguat Was Effective in Increasing Time until Clinical Worsening in Sarcoidosis Associated Pulmonary Hypertension: Results of a One Year Double Blind, Placebo Controlled Trial. In: TP25 TP025 SARCOIDOSIS: DIAGNOSIS, PROGNOSIS, AND TREATMENT. American Thoracic Society. 2021. Available at: https://www.atsjournals.org/doi/10.1164/ajrccm-conference.2021.203.1_MeetingAbstracts.A1828. Accessed on 09 July 2024.
Shino MY, Lynch Iii JP, Fishbein MC, McGraw C, Oyama J, Belperio JA, et al. Sarcoidosis-associated pulmonary hypertension and lung transplantation for sarcoidosis. Semin Respir Crit Care Med. 2014;35(3):362-71.
Shorr AF, Davies DB, Nathan SD. Predicting mortality in patients with sarcoidosis awaiting lung transplantation. Chest. 2003;124(3):922-8.
Tiosano S, Versini M, Dar Antaki L, Spitzer L, Yavne Y, Watad A, et al. The long-term prognostic significance of sarcoidosis-associated pulmonary hypertension – A cohort study. Clin Immunol. 2019;199:57-61.
