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Primary and secondary pulmonary hypertension in systemic lupus erythematosus

The Department of Rheumatology and Immunology, Tan Tock Seng Hospital, Singapore

J Thumboo

Department of Rheumatology and Immunology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore Tel: (+ 65) 357 7822; Fax: (+ 65) 357 7837 Julian_Thumboo{at}notes.ttsh.gov.sg

M-L Boey

The Department of Rheumatology and Immunology, Tan Tock Seng Hospital, Singapore

Objectives: To describe the aetiology and clinical profile of primary and secondary pulmonary hypertension (PHT) in SLE patients.

Methods: A retrospective study of SLE patients with PHT identified from a cohort of 786 SLE patients seen at Tan Tock Seng Hospital, Singapore.

Results: 22 patients had primary and 24 patients had secondary PHT, with similar clinical features at presentation and a similar degree of pulmonary pressure elevation. Secondary PHT was due to valvular heart disease (50%), pulmonary embolism (13%), interstitial lung disease (8%) or a combination of these factors (29%). Primary PHT tended to present after a shorter duration of lupus than secondary PHT (8.8 vs 43.2 months, P = 0.118). At presentation, Raynaud's phenomenon was present in 34.8% of subjects with primary or secondary PHT. Among those with secondary PHT, the presence of Raynaud's phenomenon was associated with a trend towards higher pulmonary artery systolic pressures (51.0 vs 40.5 mmHg, P = 0.101). 17% of patients with PHT died, but from causes unrelated to PHT.

Conclusion: Primary and secondary PHT are equally common in SLE patients. Secondary PHT is often multi-factorial, and Raynaud's phenomenon may be a marker for the severity of PHT in this group of patients.

Key Words: hypertension • pulmonary • Lupus erythematosus, systemic • Raynaud's phenomenon • Singapore

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