J Korean Rheum Assoc.  1999 Jun;6(2):185-191.

A Case of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Treatment with Plasmapheresis

• Koh JH
• Song SY
• Lee CK
• Seo GH
• Ahn HJ
• Cha HS
• Kim J
• Koh EM
• Song JH

Abstract

Pulmonary alveolar hemorrhage (PAH) is a rare and often fetal presenting feature of systemic lupus erythematosus (SLE) and enters the differential diagnosis of diffuse lung disease in patients with SLE. Reported mortality rats are extremely high, between 70 and 90 percents. Because death frequently occurs within the first several days of the hemorrhage, the diagnosis needs to be established promptly and treatment should be initiated immediately. Treatment of alveolar hemorrhage has included various combinations of corticosteroids, cytotoxic agents, and plasmapheresis, but survival rates have been extremely low despite aggressive therapy. We experienced a case of diffuse alveolar hemorrhage in a 29 year-old SLE male patient. PAH was diagnosed by hemoptysis, anemia, infiltration on chest X-ray and hemosiderin-laden macrophages in bronchoalveolar lavage. After high dose intravenous steroid, cyclophosphamide intravenous therapy and plasmapheresis, the condition of patient was markedly improved. He was discharged and received monthly intravenous pulse cyclophosphamide. He has done well since, showing no further pulmonary hemorrhage with steroid tapering.

Keyword

Systemic lupus erythematosus; Diffuse alveolar hemorrhage; Plasmapheresis

MeSH Terms

Adrenal Cortex Hormones
Adult
Anemia
Animals
Bronchoalveolar Lavage
Cyclophosphamide
Cytotoxins
Diagnosis
Diagnosis, Differential
Hemoptysis
Hemorrhage*
Humans
Lung Diseases
Lupus Erythematosus, Systemic*
Macrophages
Male
Mortality
Plasmapheresis*
Rats
Survival Rate
Thorax
Adrenal Cortex Hormones
Cyclophosphamide
Cytotoxins