The favourable effect of cyclophosphamide pulse therapy in the treatment of massive pulmonary haemorrhage in systemic lupus erythematosus.

Pulmonary haemorrhage (PH) is a rare but very serious complication of systemic lupus erythematosus (SLE) and the treatment is still controversial. Some authors showed the effectiveness of methylprednisolone pulse therapy for PH, although its effect was often transient. A 12-year-old Japanese girl with lupus nephritis and recurrent massive PH in SLE was treated with methylprednisolone pulse therapy. The effect on PH was transient and she needed three cycles within a month and side-effects developed. Pulse therapy with cyclophosphamide, synchronized with plasmapheresis, was tried. Thereafter she did not experience PH for 7 months, whereas lupus nephritis did not improve. Pulse cyclophosphamide would be effective for life threatening massive PH in SLE patients.