Pulmonary hypertension secondary to systemic lupus erythematosus: prolonged survival following treatment with intermittent low dose iloprost

Abstract

Pulmonary hypertension (PHT) associated with systemic lupus erythematosus (SLE) has a dismal prognosis. Vasodilators and immunosuppressive therapy have been tried over the years with discouraging results. Prostacyclin (PGI₂) which has potent vasodilatatory and anti-platelet effects has been demonstrated to significantly decrease pulmonary arterial pressure and pulmonary vascular resistance during acute infusion. Satisfactory response has been reported in SLE patients with PHT treated with short-term intravenous continuous PGI₂ infusion. We report here a 48-month experience of the use of monthly low dose infusion of a PGI₂ analogue, iloprost, in a SLE patient with pulmonary hypertension in New York Heart Association functional Class III. There was an initial haemodynamic response to an acute infusion of iloprost. Repeated infusions were followed by marked improvement in her functional status and her mean pulmonary arterial pressure dropped from 80 mmHg in the first few months and remained static at around 55 mmHg for the subsequent years.

Keywords

prostaglandin prostacyclin connective tissue disease secondary pulmonary hypertension

Get full access to this article

View all access options for this article.

References

Winslow TM , Ossipov MA , Fazio GP et al. Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus . Am Heart J 1995; 129: 510–515 .

Hughes JD , Rubin LJ . Primary pulmonary hypertension: an analysis of 28 cases and review of literature . Medicine (Baltimore) 1976; 65: 56–72 .

D'Alonzo GE , Barst RJ , Ayres SM et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry . Ann Intern Med 1991; 115: 343–349 .

Perez HD , Kramer N . Pulmonary hypertension is systemic lupus erythematosus: Report of four cases and review of the literature . Semin Arthritis Rheum 1981; 11: 177–181 .

Groen H , Bootsma H , Postma DS , Kallenberg CGM . Primary pulmonary hypertension in a patient with systemic lupus erythematosus: partial improvement with cyclophosphamide . J Rheumatol 1993; 20: 1055–1057 .

Jose de la Mata , Gomez-Sanchez MA , Aranzana M , Gomez-Reino JJ . Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases . Arthritis Rheum 1994; 37: 1528–1533 .

Dauod FS , Reeves JT , Kelly DB . Isoproterenol as a potential pulmonary vasodilator in primary pulmonary hypertension . Am J Cardiol 1978; 42: 817-817 .

Rubin LJ , Peter RH . Oral hydralazine therapy for primary pulmonary hypertension . N Engl J Med 1980; 302: 69-69 .

Klinke WP , Gilbert JAL . Diazoxide in primary pulmonary hypertension . N Engl J Med 1980; 302: 91-91 .

10.

Rich S , Kauffmann E , Levy PS . The effect of high doses of calciumchannel blockers on survival in primary pulmonary hypertension . N Engl J Med 1992: 327: 76–81 .

11.

Person B , Proctor RJ . Primary pulmonary hypertension: response to indomethacin, terbutaline and isoproterenol . Chest 1979; 76: 601–603 .

12.

Watkins WD , Peterson MB , Shannon DC , Levine L . Prostacyclin and prostaglandin E1, for severe idiopathic pulmonary artery hypertension . Lancet 1980; 17: 1083-1083 .

13.

Higenbottam TW , Butt AY , Dinh-Xaun AT et al. Treatment of pulmonary hypertension with the continuous infusion of a prostacyclin analogue, iloprost . Heart 1998; 79: 175–179 .

14.

Barst RJ , Rubin LJ , Long WA et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group . N Engl J Med 1996; 334: 296–301 .

15.

Olschewski H , Walmrath D , Schermuly R et al. Aerosolized prostacyclin and iloprost in severe pulmonary hypertension . Ann Int Med 1996; 124: 820–824 .

16.

Wagenvoort CA , Wagenvoort N . Primary pulmonary hypertension: a pathologic study of lung vessels in 156 clinically diagnosed cases . Circulation 1970; 42: 1163–1184 .

17.

Alpert MA , Pressly TA , Mukerji V et al. Acute and long-term effects of nifedipine on pulmonary and systemic hemodynamics in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease . Am J Cardiol 1991; 68: 1687–1691 .

18.

Menon N , McAlpine L , Peacock AJ , Madhok R . The acute effects of prostacyclin on pulmonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis . Arthritis Rheum 1998; 41: 466–469 .

19.

Gladman DD , Sternberg L . Pulmonary hypertension in systemic lupus erythematosus . J Rheumatol 1985; 12: 365–367 .

20.

Mahowald ML , Weir EK , Ridley DJ , Messner RP . Pulmonary hypertension in systemic lupus erythematosus: effect of vasodilators on pulmonary hemodynamics . J Rheumatol 1985; 12: 773–777 .

21.

Morelli S , Giordano M , De Marzio P et al. Pulmonary arterial hypertension responsive to immunosuppressive therapy in systemic lupus erythematosus . Lupus 1993; 2: 367–369 .

22.

Ignaszewski AP , Percy JS , Humen DP . Successful treatment of pulmonary hypertension . J Rheumatol 1993; 20: 595–596 .

23.

Luchi ME , Asherson RA , Lahita RG . Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies Arthritis Rheum. 1992; 35: 700–704 .

24.

Currie PJ , Seward JB , Chan KL et al. Continuous-wave Doppler determination of right ventricular pressure: a simultaneous Dopplercatheterizationstudy in 127 patients . J Am Coll Cardiol 1985;6: 750–756 .