Altered fatty acid composition of lung surfactant phospholipids in interstitial lung disease

2002 ◽  
Vol 283 (5) ◽  
pp. L1079-L1085 ◽  
Author(s):  
R. Schmidt ◽  
U. Meier ◽  
P. Markart ◽  
F. Grimminger ◽  
H. G. Velcovsky ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Interstitial Lung Disease ◽  
Palmitic Acid ◽  
Lung Disease ◽  
Pulmonary Surfactant ◽  
Hypersensitivity Pneumonitis ◽  
Lung Surfactant ◽  
Relative Content ◽  
Surfactant Aggregates ◽  
Surfactant Phospholipids

Deterioration of pulmonary surfactant function has been reported in interstitial lung disease; however, the molecular basis is presently unclear. We analyzed fatty acid (FA) profiles of several surfactant phospholipid classes isolated from large-surfactant aggregates of patients with idiopathic pulmonary fibrosis (IPF; n = 12), hypersensitivity pneumonitis ( n = 5), and sarcoidosis ( n = 12). Eight healthy individuals served as controls. The relative content of palmitic acid in phosphatidylcholine was significantly reduced in IPF (66.8 ± 2.5%; means ± SE; P < 0.01) but not in hypersensitivity pneumonitis (78.5 ± 1.8%) and sarcoidosis (78.2 ± 3.1%; control 80.1 ± 0.7%). In addition, the phosphatidylglycerol FA profile was significantly altered in the IPF patients, with a lower relative content of its major FA, oleic acid, at the expense of saturated FA. In the phosphatidylcholine class, a significant correlation between the impairment of biophysical surfactant function and decreased percentages of palmitic acid was noted. We conclude that significant alterations in the FA profile of pulmonary surfactant phospholipids occur predominantly in IPF and may contribute to the disturbances of alveolar surface activity in this disease.

Abnormal Lung Surfactant Related to Essential Fatty Acid Deficiency in a Neonate

PEDIATRICS ◽  
1979 ◽  
Vol 63 (6) ◽  
pp. 855-859
Author(s):  
Zvi Friedman ◽  
Abraham Rosenberg
Keyword(s):  
Fatty Acid Composition ◽  
Fatty Acid ◽  
Essential Fatty Acid ◽  
Acid Composition ◽  
Pulmonary Surfactant ◽  
Lung Surfactant ◽  
Fatty Acid Pattern ◽  
Hyaline Membrane Disease ◽  
Lavage Fluid ◽  
Surfactant Phospholipids

A low-birth-weight infant, suffering from chronic bronchopulmonary dysplasia following hyaline membrane disease and recurrent episodes of necrotizing enterocolitis, developed biochemical evidence of essential fatty acid (EFA) deficiency in the plasma. Fatty acid composition of phosphatidylcholine and phosphatidylglycerol in the lung lavage fluid was abnormal. Plasma changes includcd a decrease in the level of linoleic acid and an increased level of palmitic, palmitoleic, oleic, and 5,8, 11-eicosatrienoic acids, the ratio of 5,8,11-eicosatrienoic acid to arachidonic acid being &gt; 0.4:1. A lower than normal level of palmitic acid and an increased level of palmitoleic and oleic acids were seen in pulmonary surfactant phospholipid components. Upon treatment and recovery from EFA deficiency, the fatty acid pattern both in plasma and surfactant phospholipids returned to normal along with clinical improvement. An association between EFA deficiency and altered fatty acid composition of pulmonary surfactant phospholipids is suggested.

Fibrinolysis-inhibitory capacity of clot-embedded surfactant is enhanced by SP-B and SP-C

2003 ◽  
Vol 284 (1) ◽  
pp. L69-L76 ◽  
Author(s):  
Philipp Markart ◽  
Clemens Ruppert ◽  
Friedrich Grimminger ◽  
Werner Seeger ◽  
Andreas Günther
Keyword(s):  
Pulmonary Surfactant ◽  
Lung Surfactant ◽  
Inhibitory Effect ◽  
Fibrin Clot ◽  
Organic Extract ◽  
Inhibitory Capacity ◽  
Surfactant Aggregates ◽  
Fibrin Plate ◽  
Surfactant Phospholipids ◽  
Clot Material

Incorporation of pulmonary surfactant into fibrin inhibits its plasmic degradation. In the present study we investigated the influence of surfactant proteins (SP)-A, SP-B, and SP-C on the fibrinolysis-inhibitory capacity of surfactant phospholipids. Plasmin-induced fibrinolysis was quantified by means of a 125I-fibrin plate assay, and surfactant incorporation into polymerizing fibrin was analyzed by measuring the incorporation of3H-labeled L-α-dipalmitoylphosphatidylcholine into the insoluble clot material. Incorporation of a calf lung surfactant extract (Alveofact) and an organic extract of natural rabbit large surfactant aggregates (LSA) into a fibrin clot revealed a stronger inhibitory effect on plasmic cleavage of this clot than a synthetic phospholipid mixture (PLX) and unprocessed LSA. Reconstitution of PLX with SP-B and SP-C increased, whereas reconstitution with SP-A decreased, the fibrinolysis-inhibitory capacity of the phospholipids. The SP-B effect was paralleled by an increased incorporation of phospholipids into fibrin. We conclude that the inhibitory effect of surfactant incorporation into polymerizing fibrin on its susceptibility to plasmic cleavage is enhanced by SP-B and SP-C but reduced by SP-A. In the case of SP-B, increased phospholipid incorporation may underlie this finding.

scholarly journals ‘When you hear hooves, think zebras, not horses’; two challenging cases of interstitial lung disease (ILD)

2019 ◽  
Vol 12 (2) ◽  
pp. bcr-2018-224507
Author(s):  
Marissa O’Callaghan ◽  
Aurelie Fabre ◽  
Michael Keane ◽  
Timothy J McDonnell
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Fibrosis ◽  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Usual Interstitial Pneumonia ◽  
Case Series ◽  
Imaging Features ◽  
Exertional Dyspnoea ◽  
Multidisciplinary Team Meeting

Our case series describes two siblings with complex fibrosing lung diseases. The first patient was initially given a diagnosis of sarcoidosis based on imaging and exclusion of alternative diagnoses. A number of years after diagnosis, he had rapid deterioration of his disease and following surgical lung biopsy, his lung fibrosis was re-classified as chronic hypersensitivity pneumonitis (cHP) with a usual interstitial pneumonia pattern. He subsequently underwent successful lung transplantation. The second patient presented with rapidly progressing exertional dyspnoea. His bloods, imaging, bronchoalveolar lavage and histology were discussed at our multidisciplinary team meeting. His histology was most in keeping with subacute on cHP with overlapping imaging features between the two siblings. He was treated accordingly but unfortunately succumbed to his illness shortly after diagnosis. These cases highlight the difficulties differentiating between the various interstitial lung disease (ILD) subtypes and the challenges in management while also increasing awareness of familial ILD.

Transbronchial Biopsy and Cryobiopsy in the Diagnosis of Hypersensitivity Pneumonitis among Patients with Interstitial Lung Disease

2021 ◽  
Vol 18 (1) ◽  
pp. 148-161 ◽  
Author(s):  
Hassan A. Chami ◽  
Javier Diaz-Mendoza ◽  
Abigail Chua ◽  
Abhijit Duggal ◽  
Alex R. Jenkins ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Hypersensitivity Pneumonitis ◽  
Transbronchial Biopsy

scholarly journals Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

2020 ◽  
Vol 144 (12) ◽  
pp. 1509-1516
Author(s):  
Andrew Churg
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Fibrosis ◽  
Usual Interstitial Pneumonia ◽  
Diffuse Fibrosis ◽  
Chronic Hypersensitivity Pneumonitis

Context.— Various pulmonary diseases can produce centrilobular (peribronchiolar) fibrosis, which may be isolated or associated with other patterns of more diffuse fibrosis. The major forms of interstitial lung disease in which centrilobular fibrosis is found are fibrotic (chronic) hypersensitivity pneumonitis, connective tissue disease–associated interstitial lung disease, and (a disputed issue) usual interstitial pneumonia/idiopathic interstitial fibrosis. Objective.— To review recent literature that addresses separation of these entities. Data Sources.— Data comprised recent publications. Conclusions.— In a specially constructed multidisciplinary discussion exercise, it was found that peribronchiolar metaplasia affecting more than half the bronchioles or more than 2 foci of peribronchiolar metaplasia per square centimeter of biopsy area was strongly associated with a confident diagnosis of fibrotic hypersensitivity pneumonitis. Giant cells or granulomas were only found in cases with a greater than 50% diagnostic confidence in hypersensitivity pneumonitis. Conversely, greater numbers of fibroblast foci per square centimeter and increasing measured amounts of subpleural fibrosis favored a diagnosis of usual interstitial pneumonia. Recent data also suggest that centrilobular fibrosis can be found in usual interstitial pneumonia, although the presence of centrilobular fibrosis statistically favors an alternate diagnosis. Connective tissue disease is a major confounder because many patterns are very similar to fibrotic hypersensitivity pneumonitis or usual interstitial pneumonia. Genetic abnormalities, such as the MUC5B minor allele overlap, in these conditions and at this point cannot be used for discrimination. Thus, the separation of fibrotic hypersensitivity pneumonitis and usual interstitial pneumonia remains a difficult problem. Accurate biopsy diagnosis of all of these diseases requires correlation with imaging and clinical findings, and is crucial for treatment.

LUNG TRANSPLANTATION IN AN ADOLESCENT WITH PULMONARY FIBROSIS AS A RESULT OF INTERSTITIAL LUNG DISEASE (HYPERSENSITIVITY PNEUMONITIS)

2020 ◽  
Vol 99 (6) ◽  
pp. 287-293
Author(s):  
N.S. Lev ◽  
◽  
Y.L. Mizernitskiy ◽  
P.A. Shatokha ◽  
O.S. Groznova ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Children And Adolescents ◽  
Hypersensitivity Pneumonitis ◽  
Clinical Observation ◽  
Clinical Importance ◽  
Large Group ◽  
Chronic Course

Interstitial lung disease (ILD) is a large group of diseases with a severe course and, as a rule, an unfavorable prognosis, since irreversible pulmonary fibrosis forms in the outcome of most ILD. Among ILD in children and adolescents, hypersensitivity pneumonitis (HP) is of significant clinical importance. The clinical observation presented in the article demonstrates the first successful experience in our country (2018) of transplanting both lungs to an adolescent with pulmonary fibrosis resulting from the HP chronic course. Lung transplantation has become the only way to prolong the patient's life.

Micronodular pattern organising pneumonia mimicking miliary lung disease: a rare radiological presentation

2021 ◽  
Vol 14 (2) ◽  
pp. e239856
Author(s):  
David Ng ◽  
Garun Hamilton ◽  
Eric Hu ◽  
Kenneth Lau
Keyword(s):  
Differential Diagnosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Hypersensitivity Pneumonitis ◽  
Imaging Findings ◽  
Review Of The Literature ◽  
Radiological Findings ◽  
Rare Presentation

Organising pneumonia (OP) is an interstitial lung disease characterised by granulation tissues in alveoli and alveolar ducts. Typical imaging findings are migratory airspace opacities with peripheral or peribronchovascular distribution. Diffuse micronodular OP (MNOP) is a rare imaging manifestation, which has imaging differential diagnosis of endobronchial infection such as tuberculosis, hypersensitivity pneumonitis and respiratory bronchiolitis. Although clinical and ancillary radiological findings may aid in refining the differential diagnosis, histopathological assessment is frequently required for this rare presentation due to implications of treatment and prognosis. We report a case of MNOP and performed a review of the literature.

Cyclophosphamide in steroid-refractory hypersensitivity pneumonitis and non-classifiable interstitial lung disease

2017 ◽  
Author(s):  
Ivo A. Wiertz ◽  
Coline H.M. van Moorsel ◽  
Coline H.M. Van Moorsel ◽  
Marian J. Quanjel ◽  
Jan C. Grutters ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Hypersensitivity Pneumonitis ◽  
Steroid Refractory
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