title: Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis.
abstract: Searching for early predictive markers of the therapeutic effects of high-dose corticosteroids ("pulse therapy") on patients with rapidly progressing idiopathic pulmonary fibrosis (IPF), we evaluated 14 such patients, who had received weekly pulse therapy for at least 3 wk. Eight patients responded to the treatment and survived. However, six patients failed to respond, and all of them died within 3 mo after treatment. Serum levels of KL-6 (MUC1 mucin), neutrophil elastase (NE), and lactate dehydrogenase (LDH) were measured before, and at 1 wk and 3 wk after treatment. Levels of KL-6 decreased significantly in patients who lived, whereas KL-6 levels tended to increase in patients who died. The values of NE did not change significantly. LDH levels decreased significantly at 1 wk, and tended to decrease at 3 wk in patients who lived. However, in patients who died, they did not significantly change. At the first cycle of treatment when clinical effects may not be evident, the decrease in KL-6 but not LDH levels was significantly related to a favorable outcome, whereas their increase was related to a poor outcome. Results suggest that monitoring with KL-6 may contribute to early clinical decisions for alternative therapy in the management of rapidly progressing IPF.
authors.affiliation: Second Department of Internal Medicine, Ehime University School of Medicine, Ehime, Department of Internal Medicine, National Kinki-Chuo Hospital for Chest Diseases, Nagasone, Sakai, Osaka, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Yokoyama
authors.forename: N
authors.initials: N
authors.lastname: Kohno
authors.forename: H
authors.initials: H
authors.lastname: Hamada
authors.forename: M
authors.initials: M
authors.lastname: Sakatani
authors.forename: E
authors.initials: E
authors.lastname: Ueda
authors.forename: K
authors.initials: K
authors.lastname: Kondo
authors.forename: Y
authors.initials: Y
authors.lastname: Hirasawa
authors.forename: K
authors.initials: K
authors.lastname: Hiwada
mesh_terms: D000368:Aged; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000941:Antigens / Q000097:blood*; D000951:Antigens, Neoplasm; D015415:Biomarkers / Q000097:blood; D018450:Disease Progression; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006023:Glycoproteins / Q000097:blood*; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D019272:Leukocyte Elastase / Q000097:blood; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D018396:Mucin-1; D009077:Mucins / Q000097:blood*; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000188:drug therapy*; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D000941:Antigens; D000951:Antigens, Neoplasm; D015415:Biomarkers; D005938:Glucocorticoids; D006023:Glycoproteins; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins; D011239:Prednisolone; D007770:L-Lactate Dehydrogenase; D019272:Leukocyte Elastase; D008775:Methylprednisolone