Pulmonary Hemorrhage in Patients with Systemic Lupus Erythematosus

Pulmonary hemorrhage (PH) is a life-threatening complication in systemic lupus erythematosus (SLE) occurring in 0.5% to 5.7% of lupus patients. PH is more common in women. The mean age at onset is around 30 years. Most have an established diagnosis of SLE but in approximately 20% PH occurs at onset of diagnosis. PH should be suspected in lupus patients presenting new pulmonary infiltrates, a rapid decline in hemoglobin level and any of the following: hemoptysis, dyspnea, hypoxemia, paradoxical increase of carbon monoxide diffusing capacity, or bronchoscopic evidence of hemorrhage. Radiographic abnormalities are non-specific but commonly show alveolar infiltrates. Pulmonary infiltrates are usually bilateral, perihilar or basilar with sparing of apices. PH usually occurs in patients with active lupus presenting extra-pulmonary manifestations, the most common being renal involvement. Hematologic abnormalities, central nervous system involvement, mucocutaneous manifestations, elevated anti-dsDNA antibodies and low C3 and C4 complements are also frequently seen. PH can lead to a prompt death; thus, early diagnosis and aggressive therapy are crucial. Treatment includes high-dose corticosteroids, cyclophosphamide and plasmapheresis. Improved survival rates have been reported lately, which could be related to better management of ventilatory support and the use of broad-spectrum antibiotics, together with timely and aggressive therapy.