Surfactant abnormalities in idiopathic pulmonary fibrosis, hypersensitivity pneumonitis and sarcoidosis.
Bronchoalveolar lavage fluids (BALF) from patients with idiopathic pulmonary fibrosis (IPF; n=36), hypersensitivity pneumonitis (HP; n=32) and sarcoidosis (n=44) were investigated for their surfactant properties and compared to healthy control subjects (n=29). The phospholipid (PL) and protein concentration, the PL:protein ratio, PL subclasses, and the surfactant apoproteins (SP)A and SP-B were quantified in BALF. Large surfactant aggregates (LSA) were measured by means of ultracentrifugation and assayed for surface activity using the pulsating bubble surfactometer. As compared to controls, SP-A concentrations, LSA content and PL:protein ratios were significantly decreased in all groups, whereas PL and SP-B concentrations remained unchanged. Changes in the phospholipid profile, with reduced percentages of phosphatidylcholine (not significant) and phosphatidylglycerol and increased fractions of phosphatidylinositol and sphingomyelin (p<0.05), occurred more in IPF than in HP, and not in sarcoidosis. Surface activity was found to be severely impaired in IPF (minimum surface tension (gamma min) approximately 15-20 mN x m(-1)), but only modestly affected in HP and sarcoidosis (gamma min approximately 5 mN x m(-1)) compared to controls (gamma min approximately 0 mN x m(-1)). Reconstitution of pelleted surfactant material with soluble BALF proteins further increased gamma min values. In conclusion, moderate changes in biochemical and physical surfactant properties are encountered in hypersensitivity pneumonitis and sarcoidosis, but pronounced disturbances occur in idiopathic pulmonary fibrosis.
Günther
A
A
Dept of Internal Medicine, Justus-Liebig-University, Giessen, FRG.
Schmidt
R
R
Nix
F
F
Yabut-Perez
M
M
Guth
C
C
Rosseau
S
S
Siebert
C
C
Grimminger
F
F
Morr
H
H
Velcovsky
H G
HG
Seeger
W
W
D000293:Adolescent; D000328:Adult; D000368:Aged; D000542:Alveolitis, Extrinsic Allergic / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D001059:Apoproteins / Q000378:metabolism*; D015415:Biomarkers; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry / Q000166:cytology; D002851:Chromatography, High Pressure Liquid; D002908:Chronic Disease; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006023:Glycoproteins / Q000378:metabolism*; D006801:Humans; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D010713:Phosphatidylcholines / Q000378:metabolism; D010715:Phosphatidylglycerols / Q000378:metabolism; D010716:Phosphatidylinositols / Q000378:metabolism; D011510:Proteolipids / Q000378:metabolism*; D011658:Pulmonary Fibrosis / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D037662:Pulmonary Surfactant-Associated Protein A; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants / Q000378:metabolism*; D012129:Respiratory Function Tests; D017565:Sarcoidosis, Pulmonary / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D013109:Sphingomyelins / Q000378:metabolism
D001059:Apoproteins; D015415:Biomarkers; D006023:Glycoproteins; D010713:Phosphatidylcholines; D010715:Phosphatidylglycerols; D010716:Phosphatidylinositols; D011510:Proteolipids; D037662:Pulmonary Surfactant-Associated Protein A; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants; D013109:Sphingomyelins; C046616:pulmonary surfactant apoprotein