Role of endothelial damage in the pathogenesis of interstitial pneumonitis in patients with polymyositis and dermatomyositis.
OBJECTIVE
Polymyositis and dermatomyositis (PM/DM) are often complicated by interstitial pneumonitis (IP), which is an important cause of death. It has been reported that blood concentration of transforming growth factor-beta (TGF-beta), which is produced by a wide range of cells including endothelial cells and enhances the fibrotic changes in various tissues, is increased in PM/DM with IP. Endothelial damage is likely to exist in PM/DM. We studied the relationship between endothelial damage and IP in PM/DM.


METHODS
Blood levels of sialylated carbohydrate antigen KL-6, TGF-beta, endothelin-1 (ET-1), thrombomodulin (TM), and plasminogen activator inhibitor-1 (PAI-1) were determined in 43 patients with PM or DM with or without IP, and the relationship between these measures was analyzed.


RESULTS
Blood levels of KL-6 and TGF-beta were higher in the patients with IP than those without, and these measures were well correlated with each other. Levels of ET-1, TM, and PAI-1, all known to reflect the extent of endothelial damage, were also increased in patients with IP, and these measures correlated well with TGF-beta.


CONCLUSIONS
Our data suggest that endothelial damage might play an important role through the production of fibrosis-enhancing factors such as TGF-beta or ET-1 in PM/DM.
Funauchi
Masanori
M
Department of Neurology and Rheumatology, Kinki University School of Medicine, Osaka, Japan. funauchi@med.kindai.ac.jp
Shimadsu
Hideki
H
Tamaki
Chise
C
Yamagata
Toshiaki
T
Nozaki
Yuji
Y
Sugiyama
Masafumi
M
Ikoma
Shinya
S
Kinoshita
Koji
K
D000328:Adult; D000368:Aged; D000951:Antigens, Neoplasm / Q000097:blood / Q000502:physiology; D001772:Blood Cell Count; D003882:Dermatomyositis / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D019332:Endothelin-1 / Q000097:blood / Q000502:physiology; D004730:Endothelium, Vascular / Q000378:metabolism / Q000503:physiopathology*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000209:etiology* / Q000503:physiopathology; D008875:Middle Aged; D018396:Mucin-1; D009077:Mucins / Q000097:blood / Q000502:physiology; D017395:Plasminogen Activator Inhibitor 1 / Q000097:blood / Q000502:physiology; D017285:Polymyositis / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D018180:Thrombomodulin / Q000097:blood / Q000502:physiology; D016212:Transforming Growth Factor beta / Q000097:blood / Q000502:physiology
D000951:Antigens, Neoplasm; D019332:Endothelin-1; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins; D017395:Plasminogen Activator Inhibitor 1; D018180:Thrombomodulin; D016212:Transforming Growth Factor beta