Chronic pulmonary complications of sickle cell disease

Chest

Volumn 149, Issue 5, 2016, Pages 1313-1324

  Mehari, Alem ^a   Klings, Elizabeth S ^b  

^a HOWARD UNIVERSITY COLLEGE OF MEDICINE   (United States)

^b BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Airway hyperresponsiveness; Pulmonary embolism; Pulmonary hypertension; Sickle cell disease; Sleep disordered breathing

Indexed keywords

ANTICOAGULANT AGENT; BETA 2 ADRENERGIC RECEPTOR STIMULATING AGENT; BOSENTAN; CORTICOSTEROID; DIURETIC AGENT; HYDROXYUREA; OXYGEN; PLACEBO; SILDENAFIL;

6 MINUTE WALK TEST; ACUTE CHEST SYNDROME; ANTICOAGULANT THERAPY; ARTICLE; ASTHMA; DIAGNOSTIC TEST; ECHOCARDIOGRAPHY; ERYTHROCYTE TRANSFUSION; FORCED EXPIRATORY VOLUME; HEADACHE; HUMAN; HYPERTRANSAMINASEMIA; HYPOXEMIA; LUNG ALVEOLUS OXYGEN TENSION; LUNG COMPLICATION; LUNG DIFFUSION CAPACITY; LUNG FUNCTION TEST; NOCTURNAL HYPOXEMIA; OXYGEN SATURATION; PERIPHERAL EDEMA; PREVALENCE; PRIORITY JOURNAL; PROGNOSIS; PULMONARY HYPERTENSION; SICKLE CELL ANEMIA; SLEEP DISORDERED BREATHING; SURVIVAL; TOTAL LUNG CAPACITY; VENOUS THROMBOEMBOLISM; WHEEZING; COMPLICATION; HYPERTENSION, PULMONARY; LUNG; PATHOPHYSIOLOGY; PULMONARY EMBOLISM; RESPIRATORY SOUNDS; SLEEP APNEA SYNDROMES;

ANEMIA, SICKLE CELL; ASTHMA; HUMANS; HYPERTENSION, PULMONARY; LUNG; PULMONARY EMBOLISM; RESPIRATORY FUNCTION TESTS; RESPIRATORY SOUNDS; SLEEP APNEA SYNDROMES;

EID: 84966525936     PISSN: 00123692     EISSN: 19313543     Source Type: Journal    
DOI: 10.1016/j.chest.2015.11.016     Document Type: Review

References (93)

1. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med. 1997;337(11):762-769.
2. Miller AC, Gladwin MT. Pulmonary complications of sickle cell disease. Am J Respir Crit Care Med. 2012;185(11):1154-1165.
3. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D42-D50.
4. Mehari A, Gladwin MT, Tian X, et al. Mortality in adults with sickle cell disease and pulmonary hypertension. JAMA. 2012;307(12): 1254-1256.
5. Parent F, Bachir D, Inamo J, et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011;365(1):44-53.
6. Fonseca GHH, Souza R, Salemi VMC, et al. Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease. Eur Respir J. 2012;39(1):112-118.
7. Mehari A, Alam S, Tian X, et al. Hemodynamic predictors of mortality in adults with sickle cell disease. Am J Respir Crit Care Med. 2013;187(8):840-847.
8. Klings ES, Machado RF, Barst RJ, et al. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014;189(6):727-740.
9. Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62(11): 1088-1093.
10. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D34-D41.
11. Klings ES. Pulmonary hypertension of sickle cell disease: more than just another lung. Am J Hematol. 2008;83(1):4-5.
12. Anthi A, Machado RF, Jison ML, et al. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Am J Respir Crit Care Med. 2007;175(12):1272-1279.
13. Naeije R. Physiology of the pulmonary circulation and the right heart. Curr Hypertens Rep. 2013;15(6):623-631.
14. Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood. 2003;101(4):1257-1261.
15. Forfia PR, Vachiery JL. Echocardiography in pulmonary arterial hypertension. Am J Cardiol. 2012;110(6 suppl):16S-24S.
16. Fitzgerald M, Fagan K, Herbert DE, et al. Misclassification of pulmonary hypertension in adults with sickle hemoglobinopathies using Doppler echocardiography. South Med J. 2012;105(6):300-305.
17. Machado RF, Hildesheim M, Mendelsohn L, et al. NT-pro brain natriuretic peptide levels and the risk of death in the cooperative study of sickle cell disease. Br J Haematol. 2011;154(4):512-520.
18. Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of Six-Minute Walk Test in patients with primary pulmonary hypertension: comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000;161(2):487-492.
19. Machado RF, Mack AK, Martyr S, et al. Severity of pulmonary hypertension during vaso-occlusive pain crisis and exercise in patients with sickle cell disease. Br J Haematol. 2007;136(2):319-325.
20. Mekontso Dessap A, Leon R, Habibi A, et al. Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2008;177(6):646-653.
21. Potoka KP, Gladwin MT. Vasculopathy and pulmonary hypertension in sickle cell disease. Am J Physiol Lung Cell Mol Physiol. 2015;308(4):L314-L324.
22. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312(10):1033-1048.
23. Lezcano NE, Odo N, Kutlar A, et al. Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke. Stroke. 2006;37(6):1424-1426.
24. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573-1619.
25. Barst RJ, Mubarak KK, MacHado RF, et al. Exercise capacity and haemodynamics in patients with sickle cell disease with pulmonary hypertension treated with bosentan: results of the ASSET studies. Br J Haematol. 2010;149(3):426-435.
26. Machado RF, Barst RJ, Yovetich NA, et al. Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity. Blood. 2011;118(4):855-864.
27. Machado RF, Martyr S, Kato GJ, et al. Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension. Br J Haematol. 2005;130(3):445-453.
28. Derchi G, Forni GL, Formisano F, et al. Efficacy and safety of sildenafil in the treatment of severe pulmonary hypertension in patients with hemoglobinopathies. Haematologica. 2005;90(4): 452-458.
29. Minniti CP, MacHado RF, Coles WA, et al. Endothelin receptor antagonists for pulmonary hypertension in adult patients with sickle cell disease. Br J Haematol. 2009;147(5):737-743.
30. Naik RP, Streiff MB, Haywood C Jr., et al. Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complication. Am J Med. 2013;126(5):443-449.
31. Novelli EM, Huynh C, Gladwin MT, et al. Pulmonary embolism in sickle cell disease: a case-control study. J Thromb Haemost. 2012;10(5):760-766.
32. Ataga KI. Hypercoagulability and thrombotic complications in hemolytic anemias. Haematologica. 2009;94(11):1481-1484.
33. Stein PD, Beemath A, Meyers FA, et al. Deep venous thrombosis and pulmonary embolism in hospitalized patients with sickle cell disease. Am J Med. 2006;119(10):897.e7-897.e11.
34. Lijfering WM, Brouwer JL, Veeger NJ, et al. Selective testing for thrombophilia in patients with first venous thrombosis: results from a retrospective family cohort study on absolute thrombotic risk for currently known thrombophilic defects in 2479 relatives. Blood. 2009;113(21):5314-5322.
35. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855-1865.
36. Mekontso Dessap A, Deux JF, Abidi N, et al. Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2011;184(9):1022-1029.
37. Miller ST. How I treat acute chest syndrome in children with sickle cell disease. Blood. 2011;117(20):5297-5305.
38. Adedeji MO, Cespedes J, Allen K, et al. Pulmonary thrombotic arteriopathy in patients with sickle cell disease. Arch Pathol Lab Med. 2001;125(11):1436-1441.
39. Jerath A, Murphy P, Madonik M, et al. Pulmonary endarterectomy in sickle cell haemoglobin C disease. Eur Respir J. 2011;38(3):735-737.
40. Ataga KI, Key NS. Hypercoagulability in sickle cell disease: new approaches to an old problem. Hematology Am Soc Hematol Educ Program. 2007;2007(1):91-96.
41. Kearon C, Akl EA, Comerota AJ, et al. Antithrombotic therapy for VTE disease: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines [published correction appears in Chest. 2012;142(6):1698-1704]. Chest. 2012;141(suppl 2):e419S-e494S.
42. Kabrhel C, Mark Courtney D, Camargo CA Jr., et al. Factors associated with positive D-dimer results in patients evaluated for pulmonary embolism. Acad Emerg Med. 2010;17(6):589-597.
43. Falck-Ytter Y, Francis CW, Johanson NA, et al. Prevention of VTE in orthopedic surgery patients: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2012;141(suppl 2):e278S-e325S.
44. Naik RP, Lanzkron S. Baby on board: what you need to know about pregnancy in the hemoglobinopathies. Hematology Am Soc Hematol Educ Program. 2012;2012:208-214.
45. Taher AT, Musallam KM, Karimi M, et al. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood. 2010;115(10):1886-1892.
46. Colella MP, De Paula EV, Conran N, et al. Hydroxyurea is associated with reductions in hypercoagulability markers in sickle cell anemia. J Thromb Haemost. 2012;10(9):1967-1970.
47. Rogers VE, Lewin DS, Winnie GB, et al. Polysomnographic characteristics of a referred sample of children with sickle cell disease. J Clin Sleep Med. 2010;6(4):374-381.
48. Strauss T, Sin S, Marcus CL, et al. Upper airway lymphoid tissue size in children with sickle cell disease. Chest. 2012;142(1):94-100.
49. Kaleyias J, Mostofi N, Grant M, et al. Severity of obstructive sleep apnea in children with sickle cell disease. J Pediatr Hematol Oncol. 2008;30(9):659-665.
50. Salles C, Ramos RT, Daltro C, et al. Prevalence of obstructive sleep apnea in children and adolescents with sickle cell anemia. J Bras Pneumol. 2009;35(11):1075-1083.
51. Rosen CL, Debaun MR, Strunk RC, et al. Obstructive sleep apnea and sickle cell anemia. Pediatrics. 2014;134(2):273-281.
52. Sharma S, Efird JT, Knupp C, et al. Sleep disorders in adult sickle cell patients. J Clin Sleep Med. 2015;11(3):219-223.
53. Needleman JP, Franco ME, Varlotta L, et al. Mechanisms of nocturnal oxyhemoglobin desaturation in children and adolescents with sickle cell disease. Pediatr Pulmonol. 1999;28(6):418-422.
54. Caboot JB, Allen JL. Hypoxemia in sickle cell disease: significance and management. Paediatr Respir Rev. 2014;15(1):17-23.
55. Spivey JF, Uong EC, Strunk R, et al. Low daytime pulse oximetry reading is associated with nocturnal desaturation and obstructive sleep apnea in children with sickle cell anemia. Pediatr Blood Cancer. 2008;50(2):359-362.
56. Kirkham FJ, Hewes DK, Prengler M, et al. Nocturnal hypoxaemia and central-nervous-system events in sickle-cell disease. Lancet. 2001;357(9269):1656-1659.
57. Lehmann GC, Bell TR, Kirkham FJ, et al. Enuresis associated with sleep disordered breathing in children with sickle cell anemia. J Urol. 2012;188(4 suppl):1572-1576.
58. Roizenblatt M, Figueiredo MS, Cancado RD, et al. Priapism is associated with sleep hypoxemia in sickle cell disease. J Urol. 2012;188(4):1245-1251.
59. Marcus CL, Brooks LJ, Draper KA, et al. Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2012;130(3):e714-e755.
60. Ambrusko SJ, Gunawardena S, Sakara A, et al. Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatr Blood Cancer. 2006;47(7): 907-913.
61. Roland PS, Rosenfeld RM, Brooks LJ, et al. Clinical practice guideline: polysomnography for sleep-disordered breathing prior to tonsillectomy in children. Otolaryngol Head Neck Surg. 2011;145(1 suppl):S1-S15.
62. Akinbami LJ, Moorman JE, Bailey C, et al. Trends in asthma prevalence, health care use, and mortality in the United States, 2001-2010. NCHS Data Brief. 2012;94:1-8.
63. Field JJ, Stocks J, Kirkham FJ, et al. Airway hyperresponsiveness in children with sickle cell anemia. Chest. 2011;139(3):563-568.
64. Koumbourlis AC, Zar HJ, Hurlet-Jensen A, et al. Prevalence and reversibility of lower airway obstruction in children with sickle cell disease. J Pediatr. 2001;138(2):188-192.
65. Ozbek OY, Malbora B, Sen N, et al. Airway hyperreactivity detected by methacholine challenge in children with sickle cell disease. Pediatr Pulmonol. 2007;42(12):1187-1192.
66. Knight-Madden JM, Barton-Gooden A, Weaver SR, et al. Mortality, asthma, smoking and acute chest syndrome in young adults with sickle cell disease. Lung. 2013;191(1):95-100.
67. Strunk RC, Cohen RT, Cooper BP, et al. Wheezing symptoms and parental asthma are associated with a physician diagnosis of asthma in children with sickle cell anemia. J Pediatr. 2014;164(4):821-826.e1.
68. Boyd JH, Macklin EA, Strunk RC, et al. Asthma is associated with increased mortality in individuals with sickle cell anemia. Haematologica. 2007;92(8):1115-1118.
69. Gladwin MT, Machado RF. Pulmonary hypertension in sickle cell disease. N Engl J Med. 2011;365(17):1646-1647; author reply 1648-1649.
70. Knight-Madden JM, Forrester TS, Lewis NA, et al. Asthma in children with sickle cell disease and its association with acute chest syndrome. Thorax. 2005;60(3):206-210.
71. Cohen RT, Madadi A, Blinder MA, et al. Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease. Am J Hematol. 2011;86(9):756-761.
72. Glassberg JA, Chow A, Wisnivesky J, et al. Wheezing and asthma are independent risk factors for increased sickle cell disease morbidity. Br J Haematol. 2012;159(4):472-479.
73. National Asthma Education and Prevention Program. Expert Panel Report 3 (EPR-3): guidelines for the diagnosis and management of asthma-summary report 2007. J Allergy Clin Immunol. 2007;120(5): S94-S138.
74. Darbari DS, Castro O, Taylor JGt, et al. Severe vaso-occlusive episodes associated with use of systemic corticosteroids in patients with sickle cell disease. J Natl Med Assoc. 2008;100(8):948-951.
75. Joos S, Miksch A, Szecsenyi J, et al. Montelukast as add-on therapy to inhaled corticosteroids in the treatment of mild to moderate asthma: a systematic review. Thorax. 2008;63(5):453-462.
76. Knight-Madden JM, Forrester TS, Lewis NA, et al. The impact of recurrent acute chest syndrome on the lung function of young adults with sickle cell disease. Lung. 2010;188(6):499-504.
77. Klings ES, Wyszynski DF, Nolan VG, et al. Abnormal pulmonary function in adults with sickle cell anemia. Am J Respir Crit Care Med. 2006;173(11):1264-1269.
78. Lunt A, Desai SR, Wells AU, et al. Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease. Thorax. 2014;69(8):746-751.
79. Field JJ, Glassberg J, Gilmore A, et al. Longitudinal analysis of pulmonary function in adults with sickle cell disease. Am J Hematol. 2008;83(7):574-576.
80. Ong BA, Caboot J, Jawad A, et al. Respiratory muscle force and lung volume changes in a population of children with sickle cell disease. Br J Haematol. 2013;163(1):112-117.
81. Hosenpud JD, Stibolt TA, Atwal K, et al. Abnormal pulmonary function specifically related to congestive heart failure: comparison of patients before and after cardiac transplantation. Am J Med. 1990;88(5):493-496.
82. van Beers EJ, van der Plas MN, Nur E, et al. Exercise tolerance, lung function abnormalities, anemia, and cardiothoracic ratio in sickle cell patients. Am J Hematol. 2014;89(8):819-824.
83. Intzes S, Kalpatthi RV, Short R, et al. Pulmonary function abnormalities and asthma are prevalent in children with sickle cell disease and are associated with acute chest syndrome. Pediatr Hematol Oncol. 2013;30(8):726-732.
84. Arteta M, Campbell A, Nouraie M, et al. Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 2014;36(3): 185-189.
85. Klings ES, Anton Bland D, Rosenman D, et al. Pulmonary arterial hypertension and left-sided heart disease in sickle cell disease: clinical characteristics and association with soluble adhesion molecule expression. Am J Hematol. 2008;83(7):547-553.
86. Brousseau DC, Owens PL, Mosso AL, et al. Acute care utilization and rehospitalizations for sickle cell disease. JAMA. 2010;303(13): 1288-1294.
87. Piccin A, Murphy C, Eakins E, et al. Protein C and free protein S in children with sickle cell anemia. Ann Hematol. 2012;91(10): 1669-1671.
88. Raj A, Bertolone S, Bond S, et al. Cathlink 20: a subcutaneous implanted central venous access device used in children with sickle cell disease on long-term erythrocytapheresis-A report of low complication rates. Pediatr Blood Cancer. 2005;44(7):669-672.
89. Kucuk O, Gilman-Sachs A, Beaman K, et al. Antiphospholipid antibodies in sickle cell disease. Am J Hematol. 1993;42(4):380-383.
90. Noubouossie DF, Le PQ, Corazza F, et al. Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children. Am J Hematol. 2012;87(2):145-149.
91. Caboot JB, Jawad AF, McDonough JM, et al. Non-invasive measurements of carboxyhemoglobin and methemoglobin in children with sickle cell disease. Pediatr Pulmonol. 2012;47(8): 808-815.
92. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008;359(21):2254-2265.
93. Blake K, Lima J. Asthma in sickle cell disease: implications for treatment. Anemia. 2011;2011:740235.