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

Bandoh, Shuji; Fujita, Jiro; Ohtsuki, Yuji; Ueda, Yutaka; Hojo, Satoko; Tokuda, Michiaki; Dobashi, Hiroaki; Kurata, Noriyuki; Yoshinouchi, Takeo; Kohno, Nobuoki; Takahara, Jiro

doi:10.1136/ard.59.4.257

Cited by 96 publications

(50 citation statements)

References 11 publications

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“…SP-D serum levels have been found to be higher in patients with PM/DM and ILD compared with patients without lung involvement and healthy controls [146]. Similarly, serum levels of KL-6, a glycoprotein expressed on type-II alveolar pneumocytes and bronchial epithelial cells, have been found to be increased in PM/DM patients with interstitial lung involvement when compared with patients without parenchymal disease and healthy controls [147]. Finally, SP-D is suggested to be a useful marker in patients with PM/DM [146].…”

Section: Serum Biomarkersmentioning

confidence: 78%

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement: Table 1—

Antoniou¹,

Margaritopoulos²,

Economidou³

et al. 2009

Eur Respir J

151

122

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The connective tissue disorders (CTDs), also called collagen vascular diseases (CVDs), represent a heterogeneous group of immunologically mediated inflammatory disorders with a large variety of affected organs. Individuals with a CTD (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren's syndrome, polymyositis/dermatomyositis and mixed connective tissue disease) are susceptible to respiratory involvement. When the lungs are affected, an increasing mortality and morbidity in CVDs occurs. Interstitial lung disease (ILD) is established as a clinical corollary across the spectrum of CTDs, with an overall incidence estimated at 15%.Therefore, pivotal clinical dilemmas remain in the evaluation and management of ILD involvement in CVDs. Critical questions are the presence of fibrosis and whether the disease is clinically significant. Moreover, the clinician has to decide if treatment is warranted and which is the best therapeutic approach. The use of additional tests, such as pulmonary function tests, high-resolution computed tomography scan, bronchoalveolar lavage fluid and surgical lung biopsy, deserves better discussion. The present review focuses on establishing the diagnosis of ILD in CTD, and on evaluating disease activity and prognosis. This will provide the basis for therapeutic decisions that will be discussed, including an overview of recent advances.

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Section: Serum Biomarkersmentioning

confidence: 78%

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement: Table 1—

Antoniou¹,

Margaritopoulos²,

Economidou³

et al. 2009

Eur Respir J

151

122

View full text Add to dashboard Cite

show abstract

“…24 Bandoh et al found that the prognoses of six ILD-patients associated with PM/DM corresponded to the levels of KL-6. 25 In this study, KL-6 levels decreased in all of the patients who showed improved or unchanged CT fi ndings (Fig. 2).…”

Section: Discussionmentioning

confidence: 79%

Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases

et al. 2007

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The availability of intravenous cyclophosphamide (CYC) pulse therapy for collagen vascular diseases (CVD)-associated interstitial lung disease (ILD) has been indicated. However, the standard protocol concerning the dosage and the interval of CYC infusion has not yet been established. The aim of this study is to elucidate the efficacy and the safety of our "divided administration" protocol of CYC for the treatment of CVD-ILD. The treatment protocol consists of two steps: step 1, CYC 400-500 mg at 10-day intervals for at least 30 days, and step 2, CYC 500 mg at 14-day intervals for at least 4 weeks. The ILD activities were monitored by respiratory symptoms, serum levels of KL-6 (a serological marker of IP), chest computed tomography (CT), and pulmonary function tests. Seventeen patients [nonspecific interstitial pneumonia (NSIP), 12 patients; usual interstitial pneumonia (UIP), 4; lymphocytic interstitial pneumonia (LIP), 1] accomplished the study protocol. The sessions of CYC infusion ranged from 5 to 20 (mean, 8.3). In all patients, respiratory symptoms were improved and the serum levels of KL-6 were decreased (from 1572 +/- 904 to 978 +/- 392 U/ml; P < 0.01). Chest CT findings were improved in 4 patients (23.5%): they were all classified as NSIP; not deteriorated, 13 patients (76.5%). An improvement in the vital capacity percentage (%VC) was recognized in 10 patients (78.6%) and in diffusing capacity of carbon monoxide (%DLco) in 8 patients (61.5%). Nevertheless, mean %VC and mean %DLco did not change significantly. No major adverse event(s) occurred. The efficacy and safety of our "divided administration" protocol of CYC for CVD-ILD was demonstrated.

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“…56,57 Upon epithelial damage, KL-6 leaks into the circulation where it can be measured. It acts as a chemotactic factor, promoting the migration, proliferation and survival of lung fibroblasts.…”

Section: Krebs Von Den Lungen-6mentioning

confidence: 99%

Molecular classification of idiopathic pulmonary fibrosis: Personalized medicine, genetics and biomarkers

2015

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Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic lung disease associated with high morbidity and poor survival. Characterized by substantial disease heterogeneity, the diagnostic considerations, clinical course and treatment response in individual patients can be variable. In the past decade, with the advent of high-throughput proteomic and genomic technologies, our understanding of the pathogenesis of IPF has greatly improved and has led to the recognition of novel treatment targets and numerous putative biomarkers. Molecular biomarkers with mechanistic plausibility are highly desired in IPF, where they have the potential to accelerate drug development, facilitate early detection in susceptible individuals, improve prognostic accuracy and inform treatment recommendations. Although the search for candidate biomarkers remains in its infancy, attractive targets such as MUC5B and MPP7 have already been validated in large cohorts and have demonstrated their potential to improve clinical predictors beyond that of routine clinical practices. The discovery and implementation of future biomarkers will face many challenges, but with strong collaborative efforts among scientists, clinicians and the industry the ultimate goal of personalized medicine may be realized.

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

Cited by 96 publications

References 11 publications

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement: Table 1—

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement: Table 1—

Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases

Molecular classification of idiopathic pulmonary fibrosis: Personalized medicine, genetics and biomarkers

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