A Case of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Treatment with Plasmapheresis.
- Author:
Jay Hyun KOH
1
;
Seo Young SONG
;
Chang Keun LEE
;
Gi Hyeon SEO
;
Hong Joon AHN
;
Hoon Suk CHA
;
Jinseok KIM
;
Eun Mi KOH
;
Jae Hoon SONG
Author Information
1. Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Systemic lupus erythematosus;
Diffuse alveolar hemorrhage;
Plasmapheresis
- MeSH:
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
- From:The Journal of the Korean Rheumatism Association
1999;6(2):185-191
- CountryRepublic of Korea
- Language:Korean
-
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.
