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Therapy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematosus

Department of Internal Medicine-Rheumatology, Hospital General Regional 110-Instituto Mexicano del Seguro Social (IMSS), Guadalajara, México, lauragl{at}mail.udg.mx

E G Cardona-Muñoz

Department of Cardiology, HGR 45-IMSS Guadalajara, México

A Celis

Clinical Epidemiology Research Unit and Department of Cardiology, del Hospital de Especialidades Centro Médico Nacional de Occidente IMSS Guadalajara, México

I García-de la Torre

Department of Immunology and Rheumatology HGO-Secretaria de Salud Zapopan, México

G Orozco-Barocio

Department of Immunology and Rheumatology HGO-Secretaria de Salud Zapopan, México

M Salazar-Paramo

Clinical Epidemiology Research Unit and Department of Cardiology, del Hospital de Especialidades Centro Médico Nacional de Occidente IMSS Guadalajara, México

C Garcia-Gonzalez

Clinical Epidemiology Research Unit and Department of Cardiology, del Hospital de Especialidades Centro Médico Nacional de Occidente IMSS Guadalajara, México

A Garcia-Gonzalez

Clinical Epidemiology Research Unit and Department of Cardiology, del Hospital de Especialidades Centro Médico Nacional de Occidente IMSS Guadalajara, México

A Sanchez-Ortiz

Department of Internal Medicine-Rheumatology, Hospital General Regional 110-Instituto Mexicano del Seguro Social (IMSS), Guadalajara, México

B Trujillo-Hernandez

University Center for Biomedical Research at the University of Colima, Colima, México

J I Gamez-Nava

Clinical Epidemiology Research Unit and Department of Cardiology, del Hospital de Especialidades Centro Médico Nacional de Occidente IMSS Guadalajara, México

The aim of this study was to compare the efficacy of intravenouscyclophosphamide(IVCYC) versus oral enalapril in mild or moderate pulmonary hypertension (PH) in systemic lupus erythematosus (SLE). Thirty-four patients with SLE who had systolic pulmonary artery pressure (SPAP) > 30mmHg by Doppler echocardiography were randomized to receive IVCYC (0.5g/mt² body surface area, monthly), or oral enalapril (10mg/day) for six months. The primary outcome was the significant decrease in SPAP. An additional outcome measure included the improvement in the heart functional class (NYHA). Sixteen patients received cyclophosphamide and 18 enalapril. IVCYC decreased the median values of SPAP from 41 to 28mmHg (P < 0.001), and enalapril from 35 to 27mmHg (P 0.02). IVCYC reduced more than twice as much SPAP than enalapril (P 0.04). In those patients with SPAP 35mmHg, cyclophosphamidedecreased from 43 to 27mmHg (P 0.003), but enalapril was not effective (P 0.14). The NYHA functional class improved only in those with cyclophosphamide (P 0.021). Also IVCYC had a higher frequency of side effects including infections (RR 1.6; 95% CI, 1.001-2.47), and gastrointestinal side effects (RR 14.6; 95% CI,2.15-99.68). We concluded that IVCYC was effective in mild and moderate PH associated with SLE. Further research is needed to evaluate its long-term efficacy.

Key Words: cyclophosphamide • pulmonary hypertension • SLE

Lupus, Vol. 13, No. 2, 105-112 (2004)
DOI: 10.1191/0961203304lu509oa


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