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Alveolar Hemorrhage in Systemic Lupus Erythematosus^*

Presentation and Management

^* From the Department of Medicine (Dr. Santos-Ocampo), Makati Medical Center, Makati City, Philippines; the Department of Rheumatic and Immunologic Disease (Dr. Mandell), Cleveland Clinic Foundation, Cleveland, OH; and the Division of Rheumatology (Dr. Fessler), University of Alabama at Birmingham, Birmingham, AL.

Correspondence to: Brian F. Mandell, MD, PhD, Department of Rheumatic and Immunologic Disease, A50, Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH 44195; e-mail: mandelb{at}ccf.org

Aim: To describe our experience with alveolar hemorrhage (AH) in systemic lupus erythematosus (SLE).

Methods: Review of medical records and pertinent medical literature using MEDLINE and reference lists from retrieved publications.

Patients: Seven patients with SLE admitted with episodes of AH (n = 11).

Results: Six patients were female, and one was male. Mean age at the time of AH was 31.1 years. Mean duration of SLE was 4.5 years. AH occurred within 3 weeks of SLE onset in two patients. Recurrent AH was observed in four patients. Six patients were already receiving treatment for SLE at the time of AH. All patients presented with dyspnea and new pulmonary infiltrates. Hemoptysis occurred in only 54%. All patients had BAL within 48 h of presentation. Temperature 39°C (102.2°F) accompanied 82% of episodes. Glomerulonephritis was the most common nonpulmonary SLE manifestation (74%). Treatment with empiric IV antibiotics was initiated in 10 episodes. Initial treatment included high-dose corticosteroids (prednisone, 1 to 3 mg/kg/d [n = 2]; or IV methylprednisolone, 1 g/d [n = 9], with or without oral cyclophosphamide, 2 to 3 mg/kg/d [n = 7]). Plasmapheresis (three to four sessions) was added in five episodes for persistent AH. All patients survived.

Conclusions: AH may mimic pneumonia. Hemoptysis may not be evident. Infection must be aggressively excluded, especially since many patients with AH are already receiving immunosuppressive therapy. AH frequently recurs despite ongoing immunosuppression. Although high mortality rates have been reported with AH in SLE, we observed 100% survival.

Key Words: alveolar hemorrhage • pulmonary hemorrhage • systemic lupus erythematosus

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