Pulmonary hemorrhage as an unusual initial manifestation of systemic lupus erythematosus

Pulmonary hemorrhage as the initial manifestation of systemic lupus erythematosus (SLE) has been rarely reported in children. We present the case of a 10-year-old girl who was admitted to Kangbuk Samsung Hospital with hemoptysis. She had a 5-day history of cough with dyspnea. On physical exam, breath sound was significantly decreased combined with rales on both lung fields. Blood tests revealed pancytopenia, decreased complement levels (C3, 21.28 mg/dL; C4, 3.10 mg/dL), positive antinuclear antibody (> 1:640) and anti-double-stranded DNA antibody (262.5 IU/mL). Chest computed tomography revealed patchy ground glass opacity on both lung fields. She had proteinuria and diffuse lupus nephritis (International Society of Nephrology/Renal Pathology Society class IV-G(A)) confirmed by renal biopsy. High-dose methylprednisolone pulse therapy (30 mg/kg/day) was given for 3 days and then switched to a maintenance dose (1 mg/kg/day). Initially hemoptysis resolved after administration of methylprednisolone, but recurred on the 14th day of treatment. She was then treated with cyclophosphamide pulse therapy and hemoptysis subsided without recurrence. She was discharged on the 31st day of admission. She continued to receive monthly cyclophosphamide pulse therapy until the occurrence of leukopenia and then her regimen was switched to mycophenolate and hydroxychloroquine. SLE continues to be well controlled after 18 months of treatment. Recognition of pulmonary hemorrhage as a possible initial manifestation of SLE is crucial for early diagnosis. SLE was successfully treated with good outcome.