Lung Transplantation in Sarcoidosis

ABSTRACT

Lung transplantation (LT) is an appropriate therapeutic option for patients with severe, fibrocystic pulmonary sarcoidosis refractory to medical therapy. Survival rates following LT for sarcoidosis are generally comparable to other indications. Timing of transplantation for patients with sarcoidosis is challenging because mortality rates are high (27 to 53%) among sarcoid patients awaiting LT. Deciding when to refer patients for LT is difficult because models predicting mortality have not been validated. Importantly, algorithms or parameters predicting mortality in idiopathic pulmonary fibrosis and other interstitial pneumonias may not apply to sarcoidosis. Pulmonary function tests do not correlate well with mortality risk in patients with sarcoidosis. However, retrospective studies have shown that the presence of pulmonary arterial hypertension in sarcoidosis is an ominous sign and warrants referral for LT. This article reviews indications and contraindications to LT among patients with sarcoidosis, examines risk factors for mortality, and discusses optimal timing of referral for LT. Early referral of patients allows for timely evaluation of patients for possible listing. If the risks specific to transplantation in sarcoidosis are considered and carefully evaluated, outcomes are reasonable and match those of other diagnoses. Recurrent sarcoidosis in the lung allografts can occur but does not affect survival or risk for complications.

KEYWORDS

Sarcoidosis - lung transplant - allograft - pulmonary arterial hypertension

REFERENCES

• 1 Hunninghake G W, Costabel U, Ando M et al.. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders.  Sarcoidosis Vasc Diffuse Lung Dis. 1999;  16 149-173
• 2 Trulock E P, Edwards L B, Taylor D O, Boucek M M, Keck B M, Hertz M I. Registry of the International Society for Heart and Lung Transplantation: twenty-third official adult lung and heart-lung transplantation report-2006.  J Heart Lung Transplant. 2006;  25 880-892
• 3 International guidelines for the selection of lung transplant candidates. The American Society for Transplant Physicians (ASTP)/American Thoracic Society (ATS)/European Respiratory Society (ERS)/International Society for Heart and Lung Transplantation (ISHLT).  Am J Respir Crit Care Med. 1998;  158 335-339
• 4 Shorr A F, Davies D B, Nathan S D. Outcomes for patients with sarcoidosis awaiting lung transplantation.  Chest. 2002;  122 233-238
• 5 Baughman R P, Winget D B, Bowen E H, Lower E E. Predicting respiratory failure in sarcoidosis patients.  Sarcoidosis Vasc Diffuse Lung Dis. 1997;  14 154-158
• 6 Shorr A F, Davies D B, Nathan S D. Predicting mortality in patients with sarcoidosis awaiting lung transplantation.  Chest. 2003;  124 922-928
• 7 Arcasoy S M, Christie J D, Pochettino A et al.. Characteristics and outcomes of patients with sarcoidosis listed for lung transplantation.  Chest. 2001;  120 873-880
• 8 Newman L S, Rose C S, Maier L A. Sarcoidosis.  N Engl J Med. 1997;  336 1224-1234
• 9 Judson M A, Baughman R P, Thompson B W et al.. Two year prognosis of sarcoidosis: the ACCESS experience.  Sarcoidosis Vasc Diffuse Lung Dis. 2003;  20 204-211
• 10 Kubanek M, Malek I, Kautzner J et al.. The value of B-type natriuretic peptide and big endothelin-1 for detection of severe pulmonary hypertension in heart transplant candidates.  Eur J Heart Fail. 2005;  7 1149-1155
• 11 Ishii J, Nomura M, Ito M et al.. Plasma concentration of brain natriuretic peptide as a biochemical marker for the evaluation of right ventricular overload and mortality in chronic respiratory disease.  Clin Chim Acta. 2000;  301 19-30
• 12 Judson M A. Lung transplantation for pulmonary sarcoidosis.  Eur Respir J. 1998;  11 738-744
• 13 Massard G, Roeslin N, Wihlm J M, Dumont P, Witz J P, Morand G. Pleuropulmonary aspergilloma: clinical spectrum and results of surgical treatment.  Ann Thorac Surg. 1992;  54 1159-1164
• 14 Hadjiliadis D, Sporn T A, Perfect J R, Tapson V F, Davis R D, Palmer S M. Outcome of lung transplantation in patients with mycetomas.  Chest. 2002;  121 128-134
• 15 Judson M A, Stevens D A. The treatment of pulmonary aspergilloma.  Curr Opin Investig Drugs. 2001;  2 1375-1377
• 16 Silverman K J, Hutchins G M, Bulkley B H. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis.  Circulation. 1978;  58 1204-1211
• 17 Bargout R, Kelly R F. Sarcoid heart disease: clinical course and treatment.  Int J Cardiol. 2004;  97 173-182
• 18 Padilla M L, Schilero G J, Teirstein A S. Sarcoidosis and transplantation.  Sarcoidosis Vasc Diffuse Lung Dis. 1997;  14 16-22
• 19 Sulica R, Teirstein A S, Kakarla S, Nemani N, Behnegar A, Padilla M L. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension.  Chest. 2005;  128 1483-1489
• 20 Whelan T P, Dunitz J M, Kelly R F et al.. Effect of preoperative pulmonary artery pressure on early survival after lung transplantation for idiopathic pulmonary fibrosis.  J Heart Lung Transplant. 2005;  24 1269-1274
• 21 Huerd S S, Hodges T N, Grover F L et al.. Secondary pulmonary hypertension does not adversely affect outcome after single lung transplantation.  J Thorac Cardiovasc Surg. 2000;  119 458-465
• 22 Conte J V, Borja M J, Patel C B, Yang S C, Jhaveri R M, Orens J B. Lung transplantation for primary and secondary pulmonary hypertension.  Ann Thorac Surg. 2001;  72 1673-1679 discussion 1679-1680
• 23 Barr M L, Kawut S M, Whelan T P et al.. Report of the ISHLT Working Group on Primary Lung Graft Dysfunction, IV: Recipient-related risk factors and markers.  J Heart Lung Transplant. 2005;  24 1468-1482
• 24 Gammie J S, Keenan R J, Pham S M et al.. Single- versus double-lung transplantation for pulmonary hypertension.  J Thorac Cardiovasc Surg. 1998;  115 397-402 discussion 402-403
• 25 Whitson B A, Nath D S, Johnson A C et al.. Risk factors for primary graft dysfunction after lung transplantation.  J Thorac Cardiovasc Surg. 2006;  131 73-80
• 26 Fitton T P, Kosowski T R, Barreiro C J et al.. Impact of secondary pulmonary hypertension on lung transplant outcome.  J Heart Lung Transplant. 2005;  24 1254-1259
• 27 Kennedy P T, Zakaria N, Modawi S B et al.. Natural history of hepatic sarcoidosis and its response to treatment.  Eur J Gastroenterol Hepatol. 2006;  18 721-726
• 28 Walker S, Mikhail G, Banner N et al.. Medium term results of lung transplantation for end stage pulmonary sarcoidosis.  Thorax. 1998;  53 281-284
• 29 Shorr A F, Helman D L, Davies D B, Nathan S D. Sarcoidosis, race, and short-term outcomes following lung transplantation.  Chest. 2004;  125 990-996
• 30 Nunley D R, Hattler B, Keenan R J et al.. Lung transplantation for end-stage pulmonary sarcoidosis.  Sarcoidosis Vasc Diffuse Lung Dis. 1999;  16 93-100
• 31 Milman N, Burton C, Andersen C B, Carlsen J, Iversen M. Lung transplantation for end-stage pulmonary sarcoidosis: outcome in a series of seven consecutive patients.  Sarcoidosis Vasc Diffuse Lung Dis. 2005;  22 222-228
• 32 Kazerooni E A, Jackson C, Cascade P N. Sarcoidosis: recurrence of primary disease in transplanted lungs.  Radiology. 1994;  192 461-464
• 33 Padilla M L, Schilero G J, Teirstein A S. Donor-acquired sarcoidosis.  Sarcoidosis Vasc Diffuse Lung Dis. 2002;  19 18-24
• 34 Ionescu D N, Hunt J L, Lomago D, Yousem S A. Recurrent sarcoidosis in lung transplant allografts: granulomas are of recipient origin.  Diagn Mol Pathol. 2005;  14 140-145
• 35 Yeatman M, McNeil K, Smith J A et al.. Lung transplantation in patients with systemic diseases: an eleven-year experience at Papworth Hospital.  J Heart Lung Transplant. 1996;  15 144-149