Longterm survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol

Journal of Rheumatology

Volumn 36, Issue 10, 2009, Pages 2244-2249

  Badesch, David B ^a   McGoon, Michael D ^b   Barst, Robin J ^c   Tapson, Victor F ^d   Rubin, Lewis J ^e   Wigley, Fredrick M ^f   Kral, Kenneth M ^g   Raphiou, Ibrahim H ^g   Crater, Glenn D ^g  

^a UNIVERSITY OF COLORADO   (United States)

^b MAYO CLINIC   (United States)

^c Howard Hughes Medical Institute   (United States)

^d DUKE UNIVERSITY MEDICAL CENTER   (United States)

^e UCSD MEDICAL CENTER   (United States)

^f JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^g GLAXOSMITHKLINE   (United States)

Author keywords

6 Minute walk distance; Epoprostenol; Prostacyclin; Pulmonary arterial hypertension; Scleroderma; Therapy

Indexed keywords

PROSTACYCLIN;

ADULT; AGED; ANAMNESIS; ARTICLE; BRADYCARDIA; COMPARATIVE STUDY; CONTINUOUS INFUSION; DEPRESSION; DIARRHEA; DISEASE ASSOCIATION; DRUG FATALITY; DRUG WITHDRAWAL; DYSPNEA; EXERCISE; FEMALE; FLUSHING; GASTROINTESTINAL HEMORRHAGE; HEADACHE; HEART ARREST; HEART RIGHT VENTRICLE FAILURE; HUMAN; HYPERKALEMIA; HYPOTENSION; INFECTION; JAW DISEASE; KIDNEY FAILURE; LUNG DISEASE; MAJOR CLINICAL STUDY; MALE; NAUSEA; OPEN STUDY; OUTCOME ASSESSMENT; PAIN; PRIORITY JOURNAL; PROBABILITY; PULMONARY HYPERTENSION; RASH; RESPIRATORY TRACT DISEASE; SCLERODERMA; SEPSIS; SHOCK; SIDE EFFECT; SUDDEN DEATH; SURVIVAL RATE; SURVIVAL TIME; VASCULITIS; WALKING;

ADULT; AGED; ANTIHYPERTENSIVE AGENTS; EPOPROSTENOL; FEMALE; HUMANS; HYPERTENSION, PULMONARY; INFUSIONS, INTRAVENOUS; MALE; MIDDLE AGED; SCLERODERMA, SYSTEMIC; SURVIVAL RATE; TREATMENT OUTCOME;

EID: 70450162190     PISSN: 0315162X     EISSN: None     Source Type: Journal    
DOI: 10.3899/jrheum.081277     Document Type: Article

References (26)

1. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med 2004;351:1655-1665.
2. Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997;336:111-117.
3. Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet 2003;361:1533-1544.
4. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet 1998;352:719-725 [Erratum, Lancet 1999;353:74].
5. Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM, et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol 2004;43:25S-32S.
6. Hunzelmann N, Riemekasten G. Pulmonary arterial hypertension in collagen disease: experiences of the German Network for Systemic Scleroderma. Dtsch Med Wochenschr 2006;131 Suppl 9:S325-7.
7. Rolla G, Colagrande P, Scappaticci E, Chiavassa G, Dutto L, Cannizzo S, et al. Exhaled nitric oxide in systemic sclerosis: relationship with lung involvement and pulmonary hypertension. J Rheumatol 2000;27:1693-1698. (Pubitemid 30462813)
8. Stupi AM, Steen VD, Owens GR, Barnes EL, Rodman GP, Medsger Jr TA. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum 1986;4:515-524.
9. Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: A French nationwide prospective multicenter study. Arthritis Rheum 2005;52:3792-3800. (Pubitemid 41798217)
10. Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologist (the UNCOVER study). Arthritis Rheum 2005;52:2125-2132. (Pubitemid 40994336)
11. Mukerjee D, St. George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003;62:1088-1093.
12. Ungerer RG, Tashkin DP, Furst D, Clements PJ, Gong H, Bein M, et al. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med 1983;75:65-74.
13. Salneri R, Rodman GP, Leon DF, Shaver JA. Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). Ann Intern Med 1977;86:394-399.
14. Morse J, Barst R, Horn E, Cuervo N, Deng Z, Knowles J. Pulmonary hypertension in scleroderma spectrum of disease: Lack of bone morphogenic protein receptor 2 mutations. J Rheumatol 2002;29:2379-2381. (Pubitemid 35266029)
15. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43:2437-2444.
16. Highland KB, Garin MC, Brown KK. The spectrum of scleroderma lung disease. Semin Respir Crit Care Med 2007;28:418-429.
17. Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003;123:344-350. (Pubitemid 36269414)
18. Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: a randomized control trial. Ann Intern Med 2000;132:425-434.
19. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996;35:989-993. (Pubitemid 126719617)
20. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007;66:940-944.
21. Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, et al. Clinical difference between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006;54:3043-3050. (Pubitemid 44497786)
22. Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006;92:926-932. (Pubitemid 43974055)
23. Humbert M, Sanchez O, Fartoukh, M, Jagot JL, Le Gall C, Sitbon O, et al. Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study. Eur Respir J 1999;13:1351-1356. (Pubitemid 29357152)
24. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334:296-302.
25. Kuhn KP, Byrne DW, Arbogast PG, Doyle TP, Loyd JE, Robins IM. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving prostacyclin. Am J Respir Crit Care Med 2003;167:580-586.
26. Badesch DB, McLaughlin V, DeLacroix M, Vizza C, Olschewski H, Sitbon O, et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:56S-61S.