Diffuse Alveolar Hemorrhage as a Life-Threatening Complication of Systemic Lupus Erythematosus: A Case Report

Abstract

Background. Diffuse alveolar hemorrhage (DAH) or pulmonary hemorrhage (PH) may occur in 2-5% of Systemic Lupus Erythematosus (SLE) patients and is associated with a high mortality rate (%79-90). In 1985, Abud-Mendoza et al. published criteria for diagnosis of PH: 1) dense alveolar infiltrates involving at least 3/4 of the lung tissue in chest X-ray, 2) acute respiratory failure, 3) a descent in hemoglobin level (>3 g/dl). Hemoptysis can occur in 58% of cases. In patients without the classical triad, computed tomography (CT) images can confirm the diagnosis. Bronchoscopy is another valuable test which can confirm PH and show the origin of bleeding. Among rheumatologic diseases DAH can most frequently occur in systemic vasculitis and SLE. Active lupus nephritis with hypoalbuminemia is a major risk factor for DAH. Clinically and radiologically DAH can mimic acute respiratory distress syndrome (ARDS) and severe pneumonia. DAH treatment includes high dose corticosteroids, cyclophosphamide and plasmapheresis. Here we would like to present diffuse alveolar hemorrhage in a patient with active SLE and antiphospholipid syndrome. A 39 years old female patient was admitted to emergency department with rapidly progressive dyspnea, hemoptysis and chest pain. She had history of recurrent pregnancy loss, deep vein thrombosis and had been hospitalized for pulmonary embolism one year ago. Her laboratory tests revealed anticardiolipin IgG positivity, low complements and elevated anti-double-stranded DNA titer. Chest X-ray revealed bilateral pulmonary infiltrates (Figure: a). Thorax CT revealed diffuse alveolar opacities, crazy paving pattern and areas of consolidation (Figure: b). In emergency department her dyspnea and oxygen desaturation worsened. She was transferred to intensive care unit and was intubated. DAH due to SLE was diagnosed and intravenous methylprednisolone 1000 mg was given for 3 days. 1000 mg cyclophosphamide was administered. 1 mg/kg methylprednisolone was given for maintenance. Her clinic improved, she was weaned off ventilatory support. No further hemoptysis episodes occurred. Chest X ray revealed resolution of the opacities (Figure: c). Conclusions. Diffuse alveolar hemorrhage is a rare but life threatening complication of SLE and can be successfully treated with pulse corticosteroids and cyclophosphamide. (Figure Presented).