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Bosentan use in systemic lupus erythematosus patients with pulmonary arterial hypertension

Department of Medicine, Queen Mary Hospital, Pokfulam Road, Hong Kong, mymok{at}netvigator.com

P.L. Tsang

Department of Physiotherapy, Queen Mary Hospital, Pokfulam Road, Hong Kong

Y.M. Lam

Department of Medicine, Queen Mary Hospital, Pokfulam Road, Hong Kong

Y. Lo

Department of Medicine, Queen Mary Hospital, Pokfulam Road, Hong Kong

W.S. Wong

Department of Medicine, Queen Mary Hospital, Pokfulam Road, Hong Kong

C.S. Lau

Department of Medicine, Queen Mary Hospital, Pokfulam Road, Hong Kong

Pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE) is uncommon but is associated with poor survival. This study aimed to examine the long-term effects of bosentan, a dual endothelin-1 receptor antagonist, on symptomatology, haemodynamics and quality of life measures in SLE patients with symptomatic PAH. Four local patients had been followed up prospectively with pre-defined protocol during 12-months of bosentan treatment. Six minute walk distance (6MWD), NYHA functional class, Borg Dyspnoea Index (BDI) and SF-36 were measured at 0, 3, 6, 9 and 12 months. Systolic pulmonary arterial pressure (PAP) was measured by transthoracic echocardiography at zero, six and 12 months. Clinical parameters were analysed, pooling data from other SLE patients reported in the literature (n = 4). Bosentan was found to result in significant improvement in 6MWD compared to baseline [+24.8 m, +26.2 m, +54 m and +62.7 m at three (P = 0.001), six (P = 0.001), nine (P = 0.24) and 12 (P = 0.01) months respectively]. A differential effect was found with greater response in patients with lower exercise capacity. This was accompanied by decrease in NYHA functional class, BDI, transient or sustained drop in systolic PAP and mild improvement in SF-36 domains including mental health, vitality, social function and general health. Significantly deranged liver function was found in one patient. Lupus (2007) 16, 279—285.

Key Words: echocardiography • endothelin-1 • pulmonary hypertension • survival

Lupus, Vol. 16, No. 4, 279-285 (2007)
DOI: 10.1177/0961203307076509


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