Diffuse alveolar hemorrhage in a pediatric patient with systemic lupus erythematosus

A 15-year-old female patient was initially transferred due to symptomatic anemia following months of menorrhagia, fatigue, dyspnea, and weight loss. Early during her hospital course, pulmonary complications occurred with the development of respiratory failure secondary to bilateral pleural effusions. She was managed with bi-level pressure support ventilation alternating with a high-flow nasal cannula. An extensive workup resulted in a diagnosis of systemic lupus erythematosus: C3 level was 9 mg/dl (81-157 mg/dl) and her C4 level was 2 mg/dl (12-39 mg/dl); ANA titer was 1:5120 in a homogenous pattern. Scl-70 was qualitatively positive, quantitative anti-dsDNA level was 1044 IU/ml (<150 IU/ml), hemoglobin at presentation was 6.3 g/dl (11.8-15.7 g/dl), and her albumin level was 2.8 g/dl (3.5-5.0 g/dl). The patient was started on prednisone 60 mg daily and then transitioned to cyclophosphamide. Her symptoms improved initially; however, several days into admission she developed acute respiratory decompensation with blood-tinged sputum. Her hemoglobin levels, which had stabilized, began decreasing from 9.5 to 7.4 g/dl. Chest radiography showed new bilateral hazy infiltrates, and computerized tomography of the chest showed bilateral pleural effusions and parenchymal disease. A bronchoscopy confirmed a diagnosis of diffuse alveolar hemorrhage. The patient was treated with plasma exchange therapy and her corticosteroids were restarted. She completed a course of steroids and at initial pulmonology follow-up and showed stable pulmonary status with an improvement of her symptoms.