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Pulmonary hypertension in systemic lupus

University of Toronto Lupus Clinic, Centre for Prognostic Studies in the Rheumatic Diseases, Division of Rheumatology, University of Toronto, Toronto, Canada

D D Gladman

University of Toronto Lupus Clinic, Centre for Prognostic Studies in the Rheumatic Diseases, Division of Rheumatology, University of Toronto, Toronto, Canada

M B Urowitz

University of Toronto Lupus Clinic, Centre for Prognostic Studies in the Rheumatic Diseases, Division of Rheumatology, University of Toronto, Toronto, Canada

D Ibañez

University of Toronto Lupus Clinic, Centre for Prognostic Studies in the Rheumatic Diseases, Division of Rheumatology, University of Toronto, Toronto, Canada

J T Granton

University Health Network Pulmonary Hypertension Program, Division of Respirology, University of Toronto, Toronto, Canada

Pulmonary arterial hypertension (PAH) has devastating consequences in the rheumatic diseases; however, the prevalence in lupus is not well delineated. We searched the University of Toronto lupus database to ascertain the first echocardiogram ordered at their physician’s discretion between 1995 and 2002. We reviewed the echocardiogram reports for right ventricular systolic pressure (RVSP), valvular disease, and atrial and ventricular function. The PAH was defined as RVSP 40 mmHg. Patients were divided into three groups: RVSP 40 mmHg, RVSP ¹/4 30-39 mmHg and RVSP, 30 mmHg. We analysed potential associations between presence of PAH and lupus including disease activity, organ involvement and anticardiolipin antibodies, both at the time of and any time prior to echocardiography. In total, 129 patients underwent echocardiography. Nine patients’ echocardiogramswere not obtainable, and three patients were excluded from analysis, as their visit was more than six months from the date of echocardiography. Sixteen patients (14%) had RVSP 40 mmHg, 43 (37%) patients had RVSP of 30-39, and 60 (51%) patients had RVSP, 30 mmHg. There was no statistical difference in disease activity, organ involvement or serology among all three groups. In conclusion, the prevalence of PAH (RVSP 40 mmHg) on first echocardiogram ordered at physician discretion in our cohort was 14%. An RVSP of 30-39 mmHg was found in 37% of patients. Although abnormal, the clinical significance of this finding is unknown. Disease activity, organ involvement and anti-cardiolipin antibodies were not associated with PAH. Further research is needed to identify the mechanism, response to immunosuppression and impact on quality of life in these patients.

Key Words: Bosentan • immunosuppression • pulmonary hypertension • Raynaud’s • systemic lupus erythematosus

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