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. 2000 Apr;59(4):257–262. doi: 10.1136/ard.59.4.257

Sequential changes of KL-6 in sera of patients with interstitial pneumonia associated with polymyositis/dermatomyositis

S Bandoh 1, J Fujita 1, Y Ohtsuki 1, Y Ueda 1, S Hojo 1, M Tokuda 1, H Dobashi 1, N Kurata 1, T Yoshinouchi 1, N Kohno 1, J Takahara 1
PMCID: PMC1753113  PMID: 10733471

Abstract

OBJECTIVE—KL-6 is a mucin-like high molecular weight glycoprotein, which is strongly expressed on type II alveolar pneumocytes and bronchiolar epithelial cells. It has been demonstrated that the KL-6 antigen is a useful marker for estimating the activity of interstitial pneumonia. In this study, it is hypothesised that serum KL-6 is a useful marker to evaluate the activity of interstitial pneumonia associated with polymyositis/dermatomyositis (PM/DM).
METHODS—KL-6 was measured in sera in 16 patients diagnosed with PM/DM. Five had non-specific interstitial pneumonia (NSIP), three had diffuse alveolar damage (DAD), and eight had no pulmonary involvement, and 10 were normal non-smokers as a control group. The correlation was also evaluated between the KL-6 level and each clinical course in patients with pulmonary involvement associated with PM/DM. Immunohistochemical analysis using monoclonal anti-KL-6 antibody was also performed.
RESULTS—KL-6 concentrations in sera of patients with interstitial pneumonia associated with PM/DM were significantly high compared with those of PM/DM without interstitial pneumonia, and normal non-smokers. KL-6 concentrations in sera in patients with DAD significantly increased compared with those of other groups. KL-6 values in sera changed according to the progression or improvement of interstitial pneumonia. Immunohistochemical study using pulmonary tissues obtained from patients with DAD demonstrated that the hyaline membrane, proliferating type II pneumocytes, bronchial epithelial cells and some endothelial cells in pulmonary veins were stained by antihuman KL-6 antibody.
CONCLUSION—These data demonstrate that measurement of serum KL-6 was a useful marker to evaluate the activity of acute interstitial pneumonia associated with PM/DM.



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Figure 1  .

Figure 1  

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Serum KL-6 concentrations in normal non-smokers, patients with PM/DM without interstitial pneumonia and patients with PM/DM with interstitial pneumonia (non-specific interstitial pneumonia (NIP, empty circles), and patients with diffuse alveolar damage (DAD, black circles)). Bars represent mean (SEM).

Figure 2  .

Figure 2  

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Changes of serum KL-6 concentrations according to clinical courses in six patients with PM/DM with interstitial pneumonia. Progression or improvement of the disease was determined by PaO2 values, pulmonary function tests, and changes of chest computed tomographic findings one month after treatment. (A) Progressed cases (two patients), (B) improved cases (four patients).

Figure 3  .

Figure 3  

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Changes of serum KL-6, lactate dehydrogenase (LDH), C reactive protein (CRP) and creatine phosphokinase (CPK) values according to clinical courses in a patient (patient number 1 in table 1) with PM/DM complicated with interstitial pneumonia (DAD). This case did not respond to the immunosuppressive treatment at all. The clinical course was reflected in the sequential changes of KL-6.

Figure 4  .

Figure 4  

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The distribution of KL-6 antigen in lung tissue sections from patients with DAD was examined by means of immunoperoxidase staining and counterstained with haematoxylin. (A) Lung section featuring DAD; KL-6 antibody reacted moderately with the hyaline membrane (arrows). (B) Lung section exhibiting fibrotic change; KL-6 antibody reacted strongly with regenerating type II pneumocytes (arrows), especially their apical parts, but did not react with interstitial components. (C) Under high magnification of the hyaline membrane staining (large arrow), was less dense compared with the surrounding type II pneumocytes (arrowheads) and the content in the narrowed airspase (small arrows). (D) Some endothelial cells in pulmonary veins were also stained by this antibody (arrow heads) as well as bronchiolar epithelial cells (small arrows). The labelled streptavidin biotin peroxidase (LSABC) method was used. (A: original magnification ×90; B, C, D: original magnification ×180).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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