To determine the clinical features of our systemic lupus erythematosus (SLE) patients presenting with pulmonary hemorrhage (PH).
We reviewed the records of all SLE patients who had PH between 1994 and 2001, a total of 22.
All patients had radiographic infiltrates. The mean drop in hemoglobin was 3.2 +/-1.1 g/dL, hemoptysis occurred in 50%, the Dlco was increased in 10 of 11 patients (91%), and 11 of 14 patients who underwent bronchoscopy had positive findings. All received high-dose prednisolone and most also were given pulse methylprednisolone (MEP) and cyclophosphamide. All patients required care in the intensive/monitored care unit, 14 were intubated, 11 had plasmapheresis, and 8 died as a result of PH (mortality rate, 36%). SLEDAI and SLAM were able to indicate active disease in SLE patients with PH (median SLEDAI, 19 [interquartile range, 10 to 24] and mean SLAM, 16.1 +/- 5.8). There was a statistically significant increase in the SLEDAI from 1 month prior to PH to the time of PH (P =.014), indicating that the patients were having a significant disease flare. The median SLEDAI and SLAM scores of patients who died were slightly higher than that of survivors.
PH in SLE patients occurred in those with severe, multiorgan involvement, with high SLEDAI scores. A high degree of suspicion should be maintained in lupus patients with active disease and unexplained infiltrates on chest radiographs and dropping red cell indices, even in the absence of hemoptysis. Early aggressive management with high-dose steroids and intravenous pulses of cyclophosphamide is advocated and may explain recent trends of improved survival. Plasmapheresis may be useful for the acutely ill patient who does not respond to the above measures but does not clearly lead to improved survival.