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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.

AbstractOBJECTIVE:
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.
AuthorsSatoko Arai, Kazuhiro Kurasawa, Reika Maezawa, Takayoshi Owada, Harutsugu Okada, Takeshi Fukuda
JournalModern rheumatology (Mod Rheumatol) Vol. 23 Issue 5 Pg. 872-83 (Sep 2013) ISSN: 1439-7609 [Electronic] England
PMID22983659 (Publication Type: Journal Article)
Chemical References
  • MUC1 protein, human
  • Mucin-1
  • Pulmonary Surfactant-Associated Protein D
Topics
  • Adult
  • Aged
  • Dermatomyositis (blood, complications, drug therapy)
  • Female
  • Humans
  • Lung Diseases, Interstitial (blood, complications, drug therapy)
  • Male
  • Middle Aged
  • Mucin-1 (blood)
  • Prognosis
  • Pulmonary Surfactant-Associated Protein D (blood)
  • Retrospective Studies
  • Treatment Outcome

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