Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000075.xml
Download PDF
Semin Respir Crit Care Med 2014; 35(03): 362-371
DOI: 10.1055/s-0034-1376863
DOI: 10.1055/s-0034-1376863
Sarcoidosis-Associated Pulmonary Hypertension and Lung Transplantation for Sarcoidosis
Further Information
Publication History
Publication Date:
09 July 2014 (online)
Abstract
Pulmonary hypertension (PH) is a significant complication of sarcoidosis, occurring in approximately 6 to > 20% of cases, and markedly increases mortality among these patients. The clinician should exercise a high index of suspicion for sarcoidosis-associated PH (SAPH) given the nonspecific symptomatology and the limitations of echocardiography in this patient population. The pathophysiology of PH in sarcoidosis is complex and multifactorial. Importantly, there are inherent differences in the pathogenesis of SAPH compared with idiopathic pulmonary arterial hypertension, making the optimal management of SAPH controversial. In this article, we review the epidemiology, diagnosis, prognosis, and treatment considerations for SAPH. Lung transplantation (LT) is a viable therapeutic option for sarcoid patients with severe pulmonary fibrocystic sarcoidosis or SAPH refractory to medical therapy. We discuss the role for LT in patients with sarcoidosis, review the global experience with LT in this population, and discuss indications and contraindications to LT.
-
References
- 1 Cordova FC, D'Alonzo G. Sarcoidosis-associated pulmonary hypertension. Curr Opin Pulm Med 2013; 19 (5) 531-537
- 2 Battesti JP, Georges R, Basset F, Saumon G. Chronic cor pulmonale in pulmonary sarcoidosis. Thorax 1978; 33 (1) 76-84
- 3 Głuskowski J, Hawryłkiewicz I, Zych D, Wojtczak A, Zieliński J. Pulmonary haemodynamics at rest and during exercise in patients with sarcoidosis. Respiration 1984; 46 (1) 26-32
- 4 Handa T, Nagai S, Miki S , et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006; 129 (5) 1246-1252
- 5 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 2008; 27 (3) 329-334
- 6 Mitchell DN, Scadding JG. Sarcoidosis. Am Rev Respir Dis 1974; 110 (6) 774-802
- 7 Rizzato G, Pezzano A, Sala G , et al. Right heart impairment in sarcoidosis: haemodynamic and echocardiographic study. Eur J Respir Dis 1983; 64 (2) 121-128
- 8 Shorr AF, Davies DB, Nathan SD. Outcomes for patients with sarcoidosis awaiting lung transplantation. Chest 2002; 122 (1) 233-238
- 9 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) 1483-1489
- 10 Nunes H, Humbert M, Capron F , et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 2006; 61 (1) 68-74
- 11 Shorr AF, Davies DB, Nathan SD. Predicting mortality in patients with sarcoidosis awaiting lung transplantation. Chest 2003; 124 (3) 922-928
- 12 Shah L. Lung transplantation in sarcoidosis. Semin Respir Crit Care Med 2007; 28 (1) 134-140
- 13 Galiè N, Hoeper MM, Humbert M , et al; ESC Committee for Practice Guidelines (CPG). Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009; 30 (20) 2493-2537
- 14 Lahm T, Chakinala MM. World Health Organization group 5 pulmonary hypertension. Clin Chest Med 2013; 34 (4) 753-778
- 15 Preston IR, Klinger JR, Landzberg MJ, Houtchens J, Nelson D, Hill NS. Vasoresponsiveness of sarcoidosis-associated pulmonary hypertension. Chest 2001; 120 (3) 866-872
- 16 Maimon N, Salz L, Shershevsky Y, Matveychuk A, Guber A, Shitrit D. Sarcoidosis-associated pulmonary hypertension in patients with near-normal lung function. Int J Tuberc Lung Dis 2013; 17 (3) 406-411
- 17 Takemura T, Matsui Y, Oritsu M , et al. Pulmonary vascular involvement in sarcoidosis: granulomatous angiitis and microangiopathy in transbronchial lung biopsies. Virchows Arch A Pathol Anat Histopathol 1991; 418 (4) 361-368
- 18 Takemura T, Matsui Y, Saiki S, Mikami R. Pulmonary vascular involvement in sarcoidosis: a report of 40 autopsy cases. Hum Pathol 1992; 23 (11) 1216-1223
- 19 Rosen Y, Moon S, Huang CT, Gourin A, Lyons HA. Granulomatous pulmonary angiitis in sarcoidosis. Arch Pathol Lab Med 1977; 101 (4) 170-174
- 20 Carrington CB. Structure and function in sarcoidosis. Ann N Y Acad Sci 1976; 278: 265-283
- 21 Rosen Y. Pathology of sarcoidosis. Semin Respir Crit Care Med 2007; 28 (1) 36-52
- 22 Shorr AF, Helman DL, Davies DB, Nathan SD. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J 2005; 25 (5) 783-788
- 23 Damuth TE, Bower JS, Cho K, Dantzker DR. Major pulmonary artery stenosis causing pulmonary hypertension in sarcoidosis. Chest 1980; 78 (6) 888-891
- 24 Gaston B, Drazen JM, Loscalzo J, Stamler JS. The biology of nitrogen oxides in the airways. Am J Respir Crit Care Med 1994; 149 (2, Pt 1) 538-551
- 25 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) 214-221
- 26 Milman N, Svendsen CB, Iversen M, Videbaek R, Carlsen J. Sarcoidosis-associated pulmonary hypertension: acute vasoresponsiveness to inhaled nitric oxide and the relation to long-term effect of sildenafil. Clin Respir J 2009; 3 (4) 207-213
- 27 Galié N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 2004; 61 (2) 227-237
- 28 Giaid A, Yanagisawa M, Langleben D , et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993; 328 (24) 1732-1739
- 29 Sofia M, Mormile M, Faraone S, Alifano M, Carratù P, Carratù L. Endothelin-1 excretion in urine in active pulmonary sarcoidosis and in other interstitial lung diseases. Sarcoidosis 1995; 12 (2) 118-123
- 30 Reichenberger F, Schauer J, Kellner K, Sack U, Stiehl P, Winkler J. Different expression of endothelin in the bronchoalveolar lavage in patients with pulmonary diseases. Lung 2001; 179 (3) 163-174
- 31 Terashita K, Kato S, Sata M, Inoue S, Nakamura H, Tomoike H. Increased endothelin-1 levels of BAL fluid in patients with pulmonary sarcoidosis. Respirology 2006; 11 (2) 145-151
- 32 Letizia C, Danese A, Reale MG , et al. Plasma levels of endothelin-1 increase in patients with sarcoidosis and fall after disease remission. Panminerva Med 2001; 43 (4) 257-261
- 33 Barnett CF, Bonura EJ, Nathan SD , et al. Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience. Chest 2009; 135 (6) 1455-1461
- 34 Baughman RP, Engel PJ, Meyer CA, Barrett AB, Lower EE. Pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2006; 23 (2) 108-116
- 35 Foley RJ, Metersky ML. Successful treatment of sarcoidosis-associated pulmonary hypertension with bosentan. Respiration 2008; 75 (2) 211-214
- 36 Hoffstein V, Ranganathan N, Mullen JB. Sarcoidosis simulating pulmonary veno-occlusive disease. Am Rev Respir Dis 1986; 134 (4) 809-811
- 37 Jones RM, Dawson A, Jenkins GH, Nicholson AG, Hansell DM, Harrison NK. Sarcoidosis-related pulmonary veno-occlusive disease presenting with recurrent haemoptysis. Eur Respir J 2009; 34 (2) 517-520
- 38 Portier F, Lerebours-Pigeonniere G, Thiberville L , et al. [Sarcoidosis simulating a pulmonary veno-occlusive disease]. Rev Mal Respir 1991; 8 (1) 101-102
- 39 Wagenvoort CA, Wagenvoort N. The pathology of pulmonary veno-occlusive disease. Virchows Arch A Pathol Anat Histol 1974; 364 (1) 69-79
- 40 Montani D, O'Callaghan DS, Savale L , et al. Pulmonary veno-occlusive disease: recent progress and current challenges. Respir Med 2010; 104 (Suppl. 01) S23-S32
- 41 Roberts WC, McAllister Jr HA, Ferrans VJ. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 11). Am J Med 1977; 63 (1) 86-108
- 42 Mehta D, Lubitz SA, Frankel Z , et al. Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest 2008; 133 (6) 1426-1435
- 43 Perry A, Vuitch F. Causes of death in patients with sarcoidosis. A morphologic study of 38 autopsies with clinicopathologic correlations. Arch Pathol Lab Med 1995; 119 (2) 167-172
- 44 Iwai K, Sekiguti M, Hosoda Y , et al. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis 1994; 11 (1) 26-31
- 45 Simonneau G, Robbins IM, Beghetti M , et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54 (1, Suppl): S43-S54
- 46 Bourbonnais JM, Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 2008; 32 (2) 296-302
- 47 Arcasoy SM, Christie JD, Pochettino A , et al. Characteristics and outcomes of patients with sarcoidosis listed for lung transplantation. Chest 2001; 120 (3) 873-880
- 48 Saggar R, Saggar R, Aboulhosn J, Belperio JA, Zisman DA, Lynch III JP. Diagnosis and hemodynamic assessment of pulmonary arterial hypertension. Semin Respir Crit Care Med 2009; 30 (4) 399-410
- 49 Hoeper MM, Lee SH, Voswinckel R , et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006; 48 (12) 2546-2552
- 50 Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM. The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology 2008; 249 (3) 1042-1049
- 51 Emirgil C, Sobol BJ, Herbert WH, Trout K. The lesser circulation in pulmonary fibrosis secondary to sarcoidosis and its relationship to respiratory function. Chest 1971; 60 (4) 371-378
- 52 Arcasoy SM, Christie JD, Ferrari VA , et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003; 167 (5) 735-740
- 53 Nathan SD, Shlobin OA, Barnett SD , et al. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2008; 102 (9) 1305-1310
- 54 Zisman DA, Ross DJ, Belperio JA , et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2007; 101 (10) 2153-2159
- 55 Rich JD, Shah SJ, Swamy RS, Kamp A, Rich S. Inaccuracy of Doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice. Chest 2011; 139 (5) 988-993
- 56 Baughman RP, Sparkman BK, Lower EE. Six-minute walk test and health status assessment in sarcoidosis. Chest 2007; 132 (1) 207-213
- 57 Macfarlane JT. Prognosis in sarcoidosis. Br Med J (Clin Res Ed) 1984; 288 (6430) 1557-1558
- 58 Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007; 357 (21) 2153-2165
- 59 Lynch III JP, Kazerooni EA, Gay SE. Pulmonary sarcoidosis. Clin Chest Med 1997; 18 (4) 755-785
- 60 Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999; 160 (2) 736-755
- 61 Baughman RP. Pulmonary sarcoidosis. Clin Chest Med 2004; 25 (3) 521-530 , vi
- 62 Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc 2013; 10 (4) 362-370
- 63 Lynch III JP, Ma YL, Koss MN, White ES. Pulmonary sarcoidosis. Semin Respir Crit Care Med 2007; 28 (1) 53-74
- 64 Gluskowski J, Hawrylkiewicz I, Zych D, Zieliński J. Effects of corticosteroid treatment on pulmonary haemodynamics in patients with sarcoidosis. Eur Respir J 1990; 3 (4) 403-407
- 65 Pitsiou G, Papakosta D, Bouros D. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Respiration 2011; 82 (3) 294-304
- 66 Patel NM, Lederer DJ, Borczuk AC, Kawut SM. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest 2007; 132 (3) 998-1006
- 67 Fisher KA, Serlin DM, Wilson KC, Walter RE, Berman JS, Farber HW. Sarcoidosis-associated pulmonary hypertension: outcome with long-term epoprostenol treatment. Chest 2006; 130 (5) 1481-1488
- 68 Olschewski H, Ghofrani HA, Walmrath D , et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med 1999; 160 (2) 600-607
- 69 Olschewski H. Inhaled iloprost for the treatment of pulmonary hypertension. Eur Respir Rev 2009; 18 (111) 29-34
- 70 Baughman RP, Judson MA, Lower EE , et al. Inhaled iloprost for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc Diffuse Lung Dis 2009; 26 (2) 110-120
- 71 Baughman RP. Pulmonary hypertension associated with sarcoidosis. Arthritis Res Ther 2007; 9 (Suppl. 02) S8
- 72 Sharma S, Kashour T, Philipp R. Secondary pulmonary arterial hypertension: treated with endothelin receptor blockade. Tex Heart Inst J 2005; 32 (3) 405-410
- 73 Baughman RP, Culver DA, Cordova FC , et al. Bosentan for sarcoidosis associated pulmonary hypertension: A double-blind placebo controlled randomized trial. Chest 2013; (Oct) 31
- 74 Judson MA, Highland KB, Kwon S , et al. Ambrisentan for sarcoidosis associated pulmonary hypertension. Sarcoidosis Vasc Diffuse Lung Dis 2011; 28 (2) 139-145
- 75 Dobarro D, Schreiber BE, Handler C, Beynon H, Denton CP, Coghlan JG. Clinical characteristics, haemodynamics and treatment of pulmonary hypertension in sarcoidosis in a single centre, and meta-analysis of the published data. Am J Cardiol 2013; 111 (2) 278-285
- 76 Walker S, Mikhail G, Banner N , et al. Medium term results of lung transplantation for end stage pulmonary sarcoidosis. Thorax 1998; 53 (4) 281-284
- 77 Yusen RD, Christie JD, Edwards LB , et al; International Society for Heart and Lung Transplantation. The Registry of the International Society for Heart and Lung Transplantation: thirtieth adult lung and heart-lung transplant report—2013; focus theme: age. J Heart Lung Transplant 2013; 32 (10) 965-978
- 78 Orens JB, Estenne M, Arcasoy S , et al; Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. International guidelines for the selection of lung transplant candidates: 2006 update—a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006; 25 (7) 745-755
- 79 Baughman RP, Winget DB, Bowen EH, Lower EE. Predicting respiratory failure in sarcoidosis patients. Sarcoidosis Vasc Diffuse Lung Dis 1997; 14 (2) 154-158
- 80 Wollschlager C, Khan F. Aspergillomas complicating sarcoidosis. A prospective study in 100 patients. Chest 1984; 86 (4) 585-588
- 81 Patterson GA. Clinical-pathologic conference in general thoracic surgery: bilateral lung transplantation for sarcoidosis with aspergilloma. J Thorac Cardiovasc Surg 2002; 124 (1) 171-175
- 82 Hadjiliadis D, Sporn TA, Perfect JR, Tapson VF, Davis RD, Palmer SM. Outcome of lung transplantation in patients with mycetomas. Chest 2002; 121 (1) 128-134
- 83 Shorr AF, Helman DL, Davies DB, Nathan SD. Sarcoidosis, race, and short-term outcomes following lung transplantation. Chest 2004; 125 (3) 990-996
- 84 Young CJ, Gaston RS. African Americans and renal transplantation: disproportionate need, limited access, and impaired outcomes. Am J Med Sci 2002; 323 (2) 94-99
- 85 Nair S, Eustace J, Thuluvath PJ. Effect of race on outcome of orthotopic liver transplantation: a cohort study. Lancet 2002; 359 (9303) 287-293
- 86 Collins J, Hartman MJ, Warner TF , et al. Frequency and CT findings of recurrent disease after lung transplantation. Radiology 2001; 219 (2) 503-509
- 87 Martinez FJ, Orens JB, Deeb M, Brunsting LA, Flint A, Lynch III JP. Recurrence of sarcoidosis following bilateral allogeneic lung transplantation. Chest 1994; 106 (5) 1597-1599
- 88 Bjørtuft O, Foerster A, Boe J, Geiran O. Single lung transplantation as treatment for end-stage pulmonary sarcoidosis: recurrence of sarcoidosis in two different lung allografts in one patient. J Heart Lung Transplant 1994; 13 (1, Pt 1) 24-29