2004, Número S1
<< Anterior
Rev Inst Nal Enf Resp Mex 2004; 17 (S1)
Hipertensión pulmonar en las neumopatías intersticiales difusas
Curbelo FP, Meerovich AE, Allietti MC, Torres EV
Idioma: Español
Referencias bibliográficas: 93
Paginas: 83-95
Archivo PDF: 121.41 Kb.
RESUMEN
La hipertensión pulmonar es una complicación de las neumopatías intersticiales difusas. En el presente trabajo se realiza una revisión de los aspectos patogénicos, diagnósticos y terapéuticos de ellas. La hipertensión pulmonar no ha sido suficientemente estudiada en este grupo de pacientes.
En su patogenia se vinculan múltiples factores como la hipoxemia, la inflamación que promueve un desequilibrio a favor de mediadores con acción proliferativa y vasoconstrictora, la fibrosis intersticial, y la trombosis
in situ, entre otros.
El estudio de los pacientes incluye el screening no invasivo con ecocardiografía Doppler a lo que se agregan aspectos de la tomografía de alta resolución. El estudio invasivo hemodinámico no se plantea de rutina; actualmente, se realiza con fines de investigación para valorar la respuesta a nuevas drogas y en aquéllos con posibilidad de ser incluidos en lista de espera para trasplante pulmonar.
El tratamiento clásico incluye oxigenoterapia y, si no hay contraindicaciones la anticoagulación, pero con calcio antagonistas puede tener más efectos deletéreos que beneficios.
Se están ensayando nuevas drogas a la luz de los avances terapéuticos en el tratamiento de la hipertensión arterial pulmonar primaria. En ese campo, el tratamiento con vasodilatadores con acción antiproliferativa como los antagonistas de la endotelina 1, o el uso de sildenafil han mostrado resultados alentadores. Dichos fármacos podrían mejorar la calidad y sobrevida de los pacientes, o bien, ser usados como un puente a la espera de un eventual trasplante pulmonar.
REFERENCIAS (EN ESTE ARTÍCULO)
Presberg K, Dincer E. Pathophisiology of Pulmonary Hypertension due to Lung Disease. Curr Opin Pulm Med 2003;9:1-12.
Channick R, Rubin L. Pulmonary Hypertension: Pathogenesis and Treatment. Manual Clin Problems Pulmonary Med 2001:354-358.
Zamudio T. Hypertension Arterial Pulmonary Primary. Consideraciones actuales. Arch Inst Cardiol Mex 2001;71:142-144.
Vancheeswaran R, Magoulas T, Efrat G. Circulating Endothelin –1 Levels in Systemics Sclerosis Subsets, a Marker of Vascular Dysfunction? J Rheumatology 1994;21:1838-1844.
5 Trakada G, Spiropoulos K. Arterial Endothelin –1 in Interstitial Lung Disease Patients with Pulmonary Hypertension. Monaldi Arch Chest Dis 2001;56:379-383.
Rubin L. Primary Pulmonary Hypertension. N Engl J Med 1997;336:111-117.
Colby T. Pulmonary Pathology in Patients with Systemic Autoinmune Diseases. Clin Chest Med 1998; 19:587-613.
Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The Epidemiology of Interstitial Lung Disease. Am J Respir Crit Care Med 1994;150:967-972.
Pérez-Padilla R, Salas J, Chapela R, Sánchez M, Carrillo G, Pérez R, et al. Mortality in Mexican Patients with Chronic Pigeon Breeder´s Lung Comparated with those with Usual Interstitial Pneumonia. Am Rev Respir Dis 1993;148:49-53.
Noble P, Morris D. Time with tell. Predicting Survival in Idiopathic Interstitial Pneumonia. Am J Respir Crit Care Med 2003;168:1510-1511.
Shwartz DA, van Fossen DS, Davis CS, Helmers RA, Dayton CS, Burmeister LF, et al. Determinants of Progression in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 1994;2:444-449.
Flaherty K, Mumford J, Murray S, Kazerooni E, Gross BH, Colby TV. Prognosis Implications of Physiologic and Radiographic Changes in Idiopathic Interstitial Pneumonia. Am J Respir Crit Care Med 2003;168:543-548.
King TE Jr, Tooze JA, Schwarz M, Brown K, Cherniack R. Predicting Survival in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2001;164:1171-1181.
Cottin V, Cordier JF. Pneumopathie Interstitielle Diffuse. Rev Practicien 2001;51:1221-1229.
Panos RJ, Mortenson RL, Niccoli SA, King TE. Clinical Deterioration in Patients with Idiopathic Pulmonary Fibrosis: Causes and Assessment. Am J Med 1990; 88:396-398.
Lamblin C, Bergoin C, Saelens T, Wallert B. Interstitial Lung Diseases in Collagen Vascular Diseases. Eur Respir J 2001;32:69-80.
Gurtej S, Cheema M, Quismorio JR. Interstitial Lung Disease in Systemic Sclerosis. Curr Opin Pulm Med 2001;7:283-290.
Keneth W, Presberg M, Erhan H. Pathophysiology of Pulmonary Hypertension Due to Lung Disease. Curr Opin Pulm Med 2003;9:1-12.
Kasukawa R, Nishimaki T, Takagi T, Miyawaki S, Sokohari R, Tsunematsu T. Pulmonary Hypertension in Connective Tissue Disease. Clin Rheumatol 1990; 9:56-62.
Battle RW, Davitt MA, Cooper SM, Buckley LM, Leib ES, Beglin PA, et al. Prevalence of Pulmonary Hypertension in Limited and Diffuse Scleroderma. Chest 1996;110:1515-1519.
Steen VD, Conte C, Owens GR, Medsger TA Jr. Severe Restrictive Lung Disease in Systemic Sclerosis. Arthritis Rheum 1994;37:1283-1289.
Yousem SA. The Pulmonary Pathologic Manifestations of the CREST Syndrome. Hum Pathol 1990;21:467–474.
Yamane K, Ihn H, Asano Y, Yazawa N, Kubo M, Kikuchi K, et al. Clinical and Laboratory Features of Scleroderma Patients with Pulmonary Hypertension. Rheumatology 2000;39:1269-1271.
Stupi AM, Steen VD, Owens GR, Barnes EL, Rondan GP, Beglin, et al. Pulmonary Hypertension in CREST Syndrome Variant of Systemic Sclerosis. Arthritis Rheum 1986;29:515-524.
Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr. Isolated Diffusing Capacity Reduction in Systemic Sclerosis. Arthritis Rheum 1992;35:765-770.
Fenlon HM, Doran M, Sant SM, Breatnach E. High Resolution Chest CT in Systemic Lupus Erythematosus. Am J Roentgen 1996;166:301.
Orens J, Martinez JB, Lynch FJ. Pleuropulmonary Manifestation of Systemic Lupus Eythematosus. Rheum Dis Clin North Am 1994;20:159-193.
Pérez HD, Kramer N. Pulmonary Hypertension in Systemic Lupus Erythematosus. Semin Arthritis Rheum 1981;11:177-182.
Winslow TM, Ossipov MA, Fazio GP, Simonson JS, Redberg RF, Schiller NB. Five Year Follow- up Study of Prevalence and Progression of Pulmonary Hypertension in Systemic Lupus Erythematosus. Am Heart J 1995;129:510.
Yoshio T, Masuyama J, Sumiya M. Antiendothelial Cell Antibodies and the Relation to Pulmonary Hypertension in Systemic Lupus Erythematosus. J Rheumatol 1994;21:2058-2063.
Prakash UB. Respiratory Complications in Mixed Connective Tissue Disease. Clin Chest Med 1998;19:733-746.
Burdt MA, Hoffman RW, Deutscher SL, Wang GS, Johnson JC, Sharp GC. Long Term Outcome in Mixed Connective Tissue Disease. Arthritis Rheum 1999;42:899-909.
Nishimaki T, Aotsuka S, Kondo H, Yamamoto D, Takasaki Y, Sumiya M, et al. Inmunological Analysis of Pulmonary Hypertension in Connective Tissue Diseases. J Rheum 1999;26:2357-2363.
Helmers R, Galvin J, Hunninghake GW. Pulmonary Manifestations Associated with Rheumatoid Arthritis. Chest 1991;100:235-238.
Murray JF, Nadel JA. Textbook of Respiratory Medicine. 2nd Philadelphia, Saunders, 1994:1879-1880.
Gluskowski J, Hawrylkiewicz I, Zych D, Wojtczad A, Zielinski J. Pulmonary Hemodynamics at Rest and During Exercise in Patients with Sarcoidosis. Respiration 1984;46:26-32.
Smith LJ, Lawrence JB, Katzenstein AA. Vascular Sarcoidosis: a Rare Cause of Pulmonary Hypertension. Am J Med Sci 1983;285:38-44.
Barst RJ, Ratner SJ. Sarcoidosis and Reactive Pulmonary Hypertension. Arch Inter Med 1985;145:2112-2114.
39.Jones K, Higenbottan T, Wallwork J. Pulmonary Vasodilation with Prostacyclin in Primary and Secondary Pulmonary Hypertension. Chest 1989; 96:784-789.
Weitzenblum E, Hirth J, Rasaholinjanahary, Oudet P. Clinical Functional and Pulmonary Hemodinamic Course of Patients with Chronic Obstructive Pulmonary Disease Followed-up Over 3 Years. Respiration 1978;36:1-9.
Keogh B, Lakatos E, Price D, Cristal R. Importance of the Lower Respiratory Tract in Oxygen Transfer: Exercise Testing in Patients with Interstitial and Destructive Lung Disease. Am Rev Respir Dis 1984;129:76-80.
Travis W, Borok J, Ferrans V, Crystal R. Pulmonary Langerhans Cell Granulomatosis (Histiocytosis X). A Clinical Study of 48 Cases. Am J Surg Pathol 1993;17:971-986.
Colby I, Lombard C. Histiocytosis X in the Lung. Hum Pathol 2000;14:847-856.
Fartoukh M, Humbert M, Capron F, Maitre S, Parent F. Severe Pulmonary Hypertension in Histiocytosis X. Am J Respir Crit Care Med 2000;161:216-223.
Tejeda A, Atencio C, Sandoval J, Lupi Herrera E. Pulmonary Alveolar Microlithiasis. Arch Inst Cardiol Mex 1982;52:51-54.
Selman M, Chapela R, Salas J, Sansores R. Hypersensitivity Pneumonitis: Clinical Approach and an Integral Concept About its Pathogenesis. A Mexican Point of View. In: Selman M, Barrios R. editors. Interstitial Pulmonary Diseases: Selected Topics. Boca Raton, Florida: CRC Press, 1991:171.
Lupi E, Sandoval J, Bialostozki D. Extrinsic Allergic Alveolitis Caused by Pigeon Breeding at High Altitude. Hemodynamics Behavior of Pulmonary Circulation. Am Rev Respir Dis 1981;124: 602.
Yock PG, Popp RL. Noninvasive Evaluation of Right Ventricular Systolic Pressure by Doppler Ultrasound in Patients with Tricuspid Regurgitation. Circulation 1984;70:657-662.
Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, et al. Echocardiographic Assessment of Pulmonary Hypertension in Patients with Advanced Lung Disease. Am J Respir Crit Care Med 2003;167:735-740.
Homma A, Anzueto A, Peters JI, Susanto I, Sako E, Zabalgoitia M, et al. Pulmonary Artery Systolic Pressures Estimated by Echocardiogram vs Cardiac Catheterization in Patients Awaiting Lung Transplantation. J Heart Lung Transplant 2001;20:833-839.
Engelke C, Prokop C, Schirg E, Freihorst J, Grubnic S, Prokop M. High-resolution CT and CT Angiography of Peripheral Pulmonary Vascular Disorders. Radiographics 2002;22:739-764.
Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT Scan Evaluation for Predicting Pulmonary Hypertension in Patients with Parenchymal Lung Disease. Chest 1998;113:1250-1256.
Resten A, Maitre S, Humbert M, Sitbon O, Capron F, Simoneau G, et al. Pulmonary Arterial Hypertension: Thin-section CT Predictors of Epoprostenol Therapy Failure. Radiology 2002;222:782-788.
Miyamoto S, Nagaya N, Satoh T, Kyotani S, Sakamaki F, Fujita M, 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;161:487-492.
Timmer SJ, Karamzadeh AM, Yung GL, Kriett J, Jamieson SW, Smith CM. Predicting Survival of Lung Transplantation Candidates with Idiopathic Interstitial Pneumonia. Does PaO2 Predict Survival? Chest 2002;122:779-784.
Lama VN, Flaherty KR, Toews GB, Colby T, Travis WD, Long Q, et al. Prognostic Value of Desaturation During A Six Walk Minute Test in Idiopathic Interstitial Pneumonia. Am J Respir Crit Care Med. In press.
Agusti C, Xaubet A, Agusti AGM, Roca J, Ramirez J, Rodríguez-Roisin R. Clinical an Functional Assessment of Patients with Idiopathic Pulmonary Fibrosis: Results of a 3 Year Follow up. Eur Respir J 1994;7:643-650.
Rick C, Epler GR, Gaensler EA. Exercise Alveolar-arterial Oxygen Pressure Difference in Interstitial Lung Disease. Chest 1984;85:69-74.
Gaensler EA, Carrington CB, Coutu RE, Fitzgerald MX. Radiographic-physiologic, Pathologic Correlations in Interstitial Pneumonia. Prog Respir Res 1995;8:223-241.
Wells AU, King AD, Rubens MB, Cramer D, du Bois RM, Hansell DM. Lone Cryptogenic Fibrosing Alveolitis: a Functional-morphologic Correlation Based on Extent of Disease on Thin-section TC. Am J Respir Crit Care Med 1997;155:1367-1375.
WHO World conference on PPH in Evian, France 1998.
Agusti A, Rodríguez RR. Effect of Pulmonary Hypertension on Gas Exchange. Eur Respir J 1993;6:1371-7.
Weitzemblum E, Sautegeau A, Ehrhart M. Long Term Oxygen Therapy can Reverse the Progression of Pulmonary Hypertension in Patients with Chronic Obstructive Pulmonary Disease. Am Rev Respir Dis 1985;131:493-498.
ATS/ACCP Statement on Cardiopulmonary Exercise Testing. Am J Respir Crit Care Med 2003;167:211-277.
Dunn TL, Watters LC, Hendrix C, Schwarz MI, King TE. Gas Exchange at a Given Degree of Volume Restriction is Different in Sarcoidosis and Idiopathic Pulmonary Fibrosis. Am J Med 1988;85:221-224.
Wells AU, Hansel DM, Rubens MB, Cailes JB, Black CM, du Bois RM. Functional Impairment in Lone Cryptogenic Fibrosing Alveolitis and Fibrosing Alveolitis Associated with Systemic Sclerosis: a Comparison. Am J Respir Crit Care Med 1997;155:1657-1664.
Clark M, Cooper B, Singh S, Cooper M, Carr A, Hubbard R. A Survey of Nocturnal Hipoxaemia and Health Related Quality of Life in Patients with Cryptogenic Fibrosing Alveolitis. Thorax 2001;56:482-486.
Olschewski H, Seeger W. The therapy of severe pulmonary hypertension. In: UNI-MED Verlag AG. Pulmonary Hypertension. Bremen, Germany: International Medical Publishers, 2002:74-86.
Pietra GG, Edwards WD, Kay JM, Rich S, Kernis J, Schloo B. Primary Pulmonary Hypertension. A Qualitative and Quantitative Study of Pulmonary Blood Vessels from 58 Patients in the National Heart, Lung, and Blood Institute. Primary Pulmonary Hypertension Registry. Circulation 1989;80:1198-1206.
Fuster V, Steele PM, Edwards WD, Gersh BJ, Mc Goon MD, Frye RL. Primary Pulmonary Hypertension: Natural History and the Importance of Thrombosis. Circulation 1984;70:580-587.
Ogata M, Ohe M, Shirato K, Takishima T. Effects of a Combination Therapy of Anticoagulant and Vasodilator on the Long Term Prognosis of Primary Pulmonary Hypertension. Jpn Circ J 1993;57:63-69.
Badesch D, Tapson V, Mc Goon M, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous Intravenous Epoprostenol for Pulmonary Hypertension due to the Scleroderma Spectrum of Disease. A Randomized Controlled Trial. Ann Intern Med 2000; 132:425-434.
Tso E, Rafanan A, Hague K. Epoprostenol Therapy for Pulmonary Hipertension in Sarcoidosis. Chest 2000;118:1145-1165.
Galié N, Manes A, Branzi A. The New Clinical Trials on Pharmacological Treatment in Pulmonary Arterial Hipertension. Eur Respir J 2002;20:1037-1049.
Olschewski H, Ghofrani HA, Walmrath D, Schermuly R, Temmesfeld-Wollbruck B, Grimminger F, et al. Inhaled Prostacyclin and Iloprost in Severe Pulmonary Hypertension Secondary to Lung Fibrosis. Am J Respir Crit Care Med 1999;160:600-607.
Moloney ED, Daly A, Kirwan M, Anthony N, Corris PA, Egan JJ. Inhaled Iloprost as a Bridge to Trasplant in Patients with Pulmonary Fibrosis and Secondary Pulmonary Hypertension. J Heart Lung Transplant 2003;22:194S.
Warren JB, Higenbottam T. Caution with Use of Inhaled Nitric Oxide. Lancet 1996;348:629-630.
Riley M, Pórszász J, Miranda J, Engelen M, Brundage B, Wasserman K. Exhaled Nitric Oxide During Excercise in Primary Pulmonary Hypertension and Pulmonary Fibrosis. Chest 1997;111:44-50.
Channick RN, Hoch RC, Newhart JW, Johnson FW, Smith CM. Improvement in Pulmonary Hypertension and Hipoxemia during Nitric Oxide Inhalation in a Patient with End-stage Pulmonary Fibrosis. Am J Respir Crit Care Med 1994;149:811-814.
Yung GL, Kriett JM, Jamieson SW, Johnson FW, Newhart J, Kinninger K, et al. Outpatient Inhaled Nitric Oxide in a Patient with Idiopathic Pulmonary Fibrosis: a Bridge to Lung Transplantation. J Heart Lung Transplant 2001;20:1224-1227.
Preston IR, Klinger JR, Landzberg MJ, Houtchens J, Nelson D, Hills NS. Vasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension. Chest 2001;120:866-872.
Yamakami T, Taguchi O, Gabazza EC, Yoshida M, Kobayashi T, Yasui H. Arterial Endothelin-1 Level in Pulmonary Emphysema and Interstitial Lung Disease. Relation with Pulmonary Hypertension during Exercise. Eur Respir J 1997;10:2055-2060.
Channick R, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF. Effects of the Dual Endothelin-receptor Antagonist Bosentan in Patients with Pulmonary Hypertension: a Randomized Placebo-Controlled Study. Lancet 2001;358:1119-1123.
Rubin L, Badesch D, Barst R, Galié N, Black C, Keogh A, et al. Bosentan Therapy for Pulmonary Arterial Hypertension. N Engl J Med 2002;346:896-903.
Park S, Saleh D, Giaid A, Michel R. Increased Endothelin-1 in Bleomycin-induced Pulmonary Fibrosis and the Effect of an Endothelial Receptor Antagonist. Am J Respir Crit Care Med 1997;156:600-608.
Chen S, Chen Y, Meng Q, Durand J, Dicarlo V, Oparil S. Endothelin-receptor Antagonist Bosentan Prevents and Reverses Hypoxic Pulmonary Hypertension in Rats. J Appl Physiol 1995;79:2122-2131.
Giaid A, Michel R, Stewart D, Sheppard M, Corrin B, Hamid Q. Expression of Endothelin-1 in Lungs of Patients with Cryptogenic Fibrosing Alveolitis. Lancet 1995;341:1550-1554.
Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral Sildenafil is an Effective Specific Pulmonary Vasodilator in Patients with Pulmonary Arterial Hypertension. Circulation 2002;105:2398-2403.
Ghofrani H, Wiedemann R, Rose F, Schermuly R, Olschewski H, Weissmann N, et al. Sildenafil for Treatment of Lung Fibrosis and Pulmonary Hypertension: a Randomized Controlled Trial. Lancet 2002;360:895-900.
Hertz MI, Taylor DO, Trulock EP. The Registry of the International Society for Heart and Lung Transplantation. Nineteenth Official Report 2002. J Heart Lung Transplant 2002;21:950-970.
Hayden AM, Robert RC, Kriett JM, Smith CM, Nicholson K, Jamieson SW. Primary Diagnosis Predicts Prognosis of Lung Transplant Candidates. Transplantation 1993;55:1048-1050.
Turner Warwick M, Burrows B, Johnson A. Cryptogenic Fibrosing Alveolitis: Clinical Features and their Influence on Survival. Thorax 1980;35:171-180.
Doig JC, Corris PA, Hilton CJ, Dark JH, Bexton RS. Effect of Single Lung Transplantation on Pulmonary Hypertension in Patients with end Stage Fibrosing Lung Disease. Br Heart J 1991;66:431-443.