Dramatic efficacy of cyclophosphamide pulsed therapy combined with double–filtration plasmapheresis for treating severe massive pulmonary hemorrhage associated with systemic lupus erythematosus

Abstract

Pulmonary hemorrhage (PH) is a rare but fatal complication of systemic lupus erythematosus (SLE). We report a patient with SLE and a massive PH who was treated with double-filtration plasmapheresis synchronized with cyclophosphamide pulsed therapy. The patient showed dramatic improvement immediately and was followed for 3 years without recurrence. Prompt treatment during the acute phase of PH with this short-term intensive combination therapy may offer the best chance of success. There are few reports of long-term followup, especially in Japan.

Key words::

• Cyclophosphamide pulsed therapy
• Double-filtration plasmapheresis (DFPP)
• Plasma exchange
• Pulmonary hemorrhage (PH)
• Systemic lupus erythematosus (SLE)