La asociación de hipertensión arterial pulmonar con lupus eritematoso sistémico es infrecuente. Presentamos el caso de una paciente puérpera con diagnóstico, sin tratamiento convencional, a quien debido a un agravamiento de su enfermedad se le diagnosticó hipertensión pulmonar clase III.

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Bibliografía del caso clínico
1. Pan TL, Thumboo J, Boey ML, primary and secondary hypertension in systemic lupus erythematous. Lupus 9:338-40, 2000.
2. Chung SM, Lee CK, Lee EY, et al. Clinical aspects of pulmonary hypertension y patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol 25:866-72, 2006.
3. Prabu A, Gordon C. Pulmonary arterial hypertension in SLE: what do we know? Lupus 22:1274-85, 2013.
4. Min H, Lee J, Jung S, et al. Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival. Korean J Intern Med 30(2):232-41, 2015.
5. Asherson RA, Higenbottam TW, Dinh Xuan AT, Khamashta MA, Hughes GR. Pulmonary hypertension in a lupus clinic: Experience with a twenty-four patients. J Rheumatol 17:1292-1298, 1990.
6. Robbins IM, Gaine SP, Schilz R, Tapson VF, Rubin LJ, Loyd JE. Epoprostenol for treatment of pulmonary Hypertension in patients with systemic lupus erythematous. Chest 117:14-8, 2000.
7. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogha A. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 896-903, 2002.
8. Gonzalez-López L, Cardona Muñoz EG, Celis A, et al. Therappy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematous. Lupus 105-12, 2004.
9. Cozzi F, Montisci R, Marotta H, Bobbo F, Durigon N, Ruscazio M, et al. Bosentan therapy of pulmonary arterial hypertension in connective tissue diseases. Eur J Clin Invest 36(suppl 3):49-53, 2006.
10. Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 169:441-447, 2004.
11. Wilkins MR, Paul GA, Strange JW, Tunariu N, Gin-Sing W, Banya WA, et al. Sildenafil Versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) study. Am J Respir Crit Care Med 171:1292-1297, 2005.
12. Sociedad Argentina de Cardiología, Asociación Argentina de Medicina Respiratoria, Sociedad Argentina de Reumatología. Consenso para el diagnóstico y tratamiento de la hipertensión de la arteria pulmonar. Revista Argentina de Cardiología 79(Supl 2), 2011.
13. McLaughlin V, McGoon M. Pulmonary Arterial Hypertension. Circulation 114:1417-31, 2006.
14. Tamborrini G, Distler O. Update in pulmonary hypertension associated with connective tissue diseases - a systematic literature review. Dtsch Med Wochenschr 133(Suppl 6):S199- 202, 2008.
15. Quismorio FP. Pulmonary manifestations of systemic lupus erythematous: in Walace D, Bebvra Hannahs H: Dubois' Lupus Erythematosus. Baltimore: Williams & Wilkins, 5th edition, pp. 673-92, 1997.
16. D´Alonso GE, Barst RJ, Yres SM, et al. Survival in patients with primary pulmonary. Results from a national prospective registry. Ann Intern Med 115:343-9, 1991.
17. Benza RL, Miller DP, Gomberg-Maitland, et al. Predicting survival in pulmonary arterial hypertension. Circulation 122:164-72, 2010.
18. Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 174:1034-41, 2006.
19. Tei C, Dujardin KS, Hodge DO, et al. Doppler echocardiographic index for assessment of global right ventricular function. J Am Soc Echocardiogr 838-47, 1996.
20. Kakouros N, Kakouros S, Lekakis J, et al. Tissue Doppler imaging of the tricuspid annulus and myocardical performance index in the evaluation of right ventricular involvement in the acute and late phase of a first inferior myocardical infarction. Echocardiography 28:311-9, 2011.
21. Guillinta P, Peterson KL, Yehuda O. Cardiac catheterization techniques in pulmonary hypertension. Cardiol Clin 22:401-15, 2004.
22. Paciocco G, Martinez F, Bossone E et al. Oxygen desaturationon the six minute walk test and mortality in untreated primary pulmonary hypertension. Eur Respir J 17:647-52, 2001.
23. Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six minute walk test in patients with primary pulmonary hypertension: comparision with cardiopulmonary exercise esting. Am J Respir Crit Care Med 161:487-92, 2000.
24. Fijalkowska A, Kurzyna M, Torbicki A, et al. Serum N terminalbrain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 129:1313-21, 2006.
25. Badesh DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54S55-S66, 2009.
26. Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with svere chronic pulmonary hypertension. Circulation 114:1482-9, 2006.
27. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al; American College of Cardiology Foundation Task Force on Expert Consensus Documents; American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. 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 53:1573-619, 2009.
28. Rich S, Kaufmann E, Levy PS. The effect of high doses of calciumchannel blockers on survival in primary pulmonary hypertension. N Engl J Med 327:76-81, 1992.
29. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis Circulation 70:580-7, 1984.
30. Sitbon O, Humbert M, Jais X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:3105-11, 2005.
31. Lee SH, Rubin LJ. Current treatment strategies for pulmonary arterial hypertension. J Intern Med 258:199-215, 2005.
32. Galie N, Rubin L, Hoeper M, Jansa P, Al Hiti H, Meyer G, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 371:2093-100, 2008.
33. Galie N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, et al; Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 114:48-54, 2006.
34. Galie N, Olschewski H, Oudiz RJ, et al; Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 117:3010-9, 2008.
35. Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353:2148-57, 2005.
36. Galiè N, Brundage BH, Ghofrani HA, et al; Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Tadalafil therapy for pulmonary arterial hypertension. Circulation 119:2894-903, 2009.
37. Jais X, Launay D, Yaici A, Le Pavec J, Tchérakian C, Sitbon O et al. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum 58:521-31, 2008.
37. 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 30:2493-537, 2009.
38. Tanaka E, Harigai M, Tanaka M, Kawaguchi Y, Hara M, Kamatani N. Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to inmunosuppresive treatment. J Rheumatol 29(2)282-7, 2002.
39. Sanchez O, Sitbon O, Jaïs X, Simanneau G, Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 130(1):182-9, 2006.

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