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Marked increase in serum KL-6 and surfactant protein D levels during the first 4 weeks after treatment predicts poor prognosis in patients with active interstitial pneumonia associated with polymyositis/dermatomyositis

  • Original Article
  • Published:
Modern Rheumatology

Abstract

Objective

The aim of this study is to determine whether serum KL-6 and surfactant protein D (SP-D) levels predict the prognosis of patients with interstitial pneumonia (IP) in cases of polymyositis (PM) and dermatomyositis (DM).

Patients and methods

Fifty consecutive patients with PM (n = 17) or DM (n = 33) and active IP, 6 of whom died of respiratory failure, were enrolled in this study. Serum KL-6 and SP-D levels were measured every 2–4 weeks. Medical records were reviewed retrospectively. Univariate analyses and multivariate analyses with a logistic regression model were conducted.

Results

Serum KL-6 and SP-D levels were elevated in patients with active IP. At the time of diagnosis of active IP, the serum KL-6 level was within the normal range in 28 % of patients and the SP-D level was within the normal range in 46 % of patients. Serum KL-6 level increased up to 3 months after starting treatment and then decreased gradually to baseline, whereas SP-D level peaked within the first 4 weeks after treatment and decreased rapidly to normal levels. Patients with poor prognosis showed increases in KL-6 and SP-D levels during the first 4 weeks after treatment, which was confirmed by uni- and multivariate analyses. Comparing the marker levels at 2–4 weeks after treatment with those at 0 weeks, an increase in the ratio over 1.70 for KL-6 and over 1.75 for SP-D, and an increase in KL-6 over 850 U/ml during the first 4 weeks after treatment, were poor prognostic factors.

Conclusions

Increases in serum KL-6 and SP-D levels during the first 4 weeks after starting therapy, but not their levels at any one time point, predict poor prognosis in patients with PM/DM. When marked increases of KL-6 and SP-D levels during the first 4 weeks are found or are predicted by serial measurement of the markers, patients have risks of poor prognosis and additional therapy should be considered.

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Authors and Affiliations

  1. Clinical Immunology, Dokkyo Medical University, 880 Kita-Kobayashi, Mibu, Tochigi, 321-0293, Japan

    Satoko Arai, Kazuhiro Kurasawa, Reika Maezawa, Takayoshi Owada, Harutsugu Okada & Takeshi Fukuda

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  1. Satoko Arai
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  2. Kazuhiro Kurasawa
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  3. Reika Maezawa
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  4. Takayoshi Owada
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  5. Harutsugu Okada
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  6. Takeshi Fukuda
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Correspondence to Kazuhiro Kurasawa.

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Arai, S., Kurasawa, K., Maezawa, R. et al. Marked increase in serum KL-6 and surfactant protein D levels during the first 4 weeks after treatment predicts poor prognosis in patients with active interstitial pneumonia associated with polymyositis/dermatomyositis. Mod Rheumatol 23, 872–883 (2013). https://doi.org/10.1007/s10165-012-0756-0

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  • DOI: https://doi.org/10.1007/s10165-012-0756-0

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