Pneumomediastinum in a Patient with Systemic Lupus Erythematosus with Pulmonary Renal Syndrome: Pneumomediastinum in a Patient with Systemic Lupus Erythematosus with Anca-P+ Pulmonary Syndrome

Abstract

This case report describes pneumomediastinum in a patient with systemic lupus erythematosus (SLE) and lung-kidney (pulmonary-renal) syndrome with positive P-ANCA. The authors explain that lung-kidney syndrome combines diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis and is usually associated with rheumatologic diseases, while pneumomediastinum has been described in connective tissue diseases, mainly polymyositis and dermatomyositis, and the evidence relating it to SLE is insufficient. The patient, a 27-year-old woman with a family history of SLE, presented with diffuse alopecia, madarosis and pallor, followed by progressive dyspnea. Investigations showed anemia, proteinuria with active sediment, renal failure, hyperkalemia and metabolic acidosis, with positive P-ANCA and antinuclear antibodies. Chest X-ray showed diffuse alveolar infiltrates, and a diagnosis of SLE (SLEDAI 24 points) with lung-kidney syndrome was made. She was admitted to intensive care and treated with transfusions, oxygen support, methylprednisolone pulses, cyclophosphamide and hemodialysis. Tomography showed findings compatible with diffuse alveolar hemorrhage and pneumomediastinum at the right paratracheal level. After initial cardiorespiratory improvement, she developed fever, renewed respiratory deterioration and massive hemoptysis that caused cardiac arrest, and she died. The authors note that there is no established consensus on the treatment of pneumomediastinum, which in most reported cases is managed conservatively as a self-limited condition.

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