Systemic lupus erythematosus-associated alveolar hemorrhage: presentation, treatment, and outcome

Alveolar hemorrhage (AH) is a rare, dramatic, and life-threatening manifestation of systemic lupus erythematosus (SLE), which may occur early or late in disease evolution. Presentation is highly variable, and hemoptysis may be absent. The most reliable clinical signs include a drop in hemoglobin accompanied by new pulmonary infiltrates. Extrapulmonary disease, especially nephritis, is common; however, milder SLE symptoms may be minimal and "masked" in patients receiving immunosuppression for other symptoms of SLE. Predictors of patients at risk for this complication are unclear at this time. An aggressive diagnostic approach to exclude infection is indicated. Use of broad-spectrum antibiotics, coincident with immunosuppressive treatment of the AH, while awaiting initial culture results, is prudent. Treatment regimens lack standardization because of the absence of controlled clinical trials and the rarity of this complication. Timely intensification of immunosuppression is required and effective. The capacity of AH to occur and recur, despite ongoing immunosuppressive therapy, is noted, but long-term AH-free episodes among survivors are attainable.