Abstract
A 43-year-old Japanese woman was referred to our hospital in 1997 because of Raynaud’s phenomenon. Systemic lupus erythematosus was diagnosed on the basis of the presence of antinuclear antibody (1:1,280), anti-DNA antibody (1:640), anti-Sm antibody, antiphospholipid antibody, lymphopenia, and proteinuria. She developed pulmonary fibrosis in 1999 and pulmonary hypertension in 2001. In October 2002, a 24-hr continuous infusion of epoprostenol was started. Dyspnea, Raynaud’s phenomenon, and pulmonary hypertension improved with low-dose epoprostenol (3.0 to 4.0 ng kg−1 min−1). The patient could not tolerate larger doses of epoprostenol so 4.0 ng kg−1 min−1 was selected as the maintenance dose. The clinical course was uneventful at this dosage. It appears that pulmonary hypertension can be controlled with low-dose epoprostenol such as 3.0 to 4.0 ng kg−1 min−1 in some rheumatic patients.
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References
Sulica R, Poon M (2004) Current medical treatment of pulmonary arterial hypertension. Mt Sinai J Med 71:103–114
Robbins IM, Gaine SP, Schilz R et al (2000) Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest 117:14–18
Horn EM, Barst RJ Poon M (2000) Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest 118:1229–1230
Kongo KO, Badsha H, Thumboo J et al (2003) Intermittent epoprostenol infusions in systemic lupus erythematosus associated pulmonary hypertension—a series of three cases. Ann Acad Med Singapore 32:118–121
Ooiwa H, Miyazawa T, Yamanishi Y et al (2000) Successful treatment of systemic lupus erythematosus and pulmonary hypertension with intravenous prostaglandin I2 followed by its oral analogue. Intern Med 39:320–323
Scorza R, Caronni M, Mascagni B et al (2001) Effects of long-term cyclic iloprost therapy in systemic sclerosis with Raynaud’s phenomenon. A randomized, controlled study. Clin Exp Rheumatol 19:503–508
Matsukawa Y, Saito O, Aoki M et al (2002) Long-term administration of beraprost, an oral prostacyclin analogue, improves pulmonary diffusion capacity in patients with systemic sclerosis. Prostaglandins Leukot Essent Fatty Acids 67:45–49
Kyotani S (2004) Problems and prospects of continuous intravenous prostacyclin therapy for pulmonary hypertension due to collagen disease. Rheumatology (Tokyo) 31:535–541 (In Japanese)
Yokoo N, Waki K, Aragaki Y et al (2004) Intravenous administration of Frolan. Progress in Medicine 24:286–289 (In Japanese)
Melian EB, Goa KL (2002) Beraprost: a review of its pharmacology and therapeutic efficacy in the treatment of peripheral arterial disease and pulmonary arterial hypertension. Drugs 62:107–133
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Matsukawa, Y., Igei, K., Nozaki, T. et al. Low-dose epoprostenol improved pulmonary hypertension in a patient with systemic lupus erythematosus. Clin Rheumatol 26, 582–583 (2007). https://doi.org/10.1007/s10067-005-0170-5
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DOI: https://doi.org/10.1007/s10067-005-0170-5