Primary and secondary pulmonary hypertension in systemic lupus erythematosus.
OBJECTIVE
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.


CONCLUSIONS
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.
Pan
T L
TL
The Department of Rheumatology and Immunology, Tan Tock Seng Hospital, Singapore.
Thumboo
J
J
Boey
M L
ML
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012846:Singapore / Q000453:epidemiology