Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis

Am J Respir Crit Care Med. 1998 Nov;158(5 Pt 1):1680-4. doi: 10.1164/ajrccm.158.5.9803115.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Anti-Inflammatory Agents / administration & dosage
  • Anti-Inflammatory Agents / therapeutic use
  • Antigens / blood*
  • Antigens, Neoplasm
  • Biomarkers / blood
  • Disease Progression
  • Drug Administration Schedule
  • Female
  • Follow-Up Studies
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / therapeutic use
  • Glycoproteins / blood*
  • Humans
  • L-Lactate Dehydrogenase / blood
  • Leukocyte Elastase / blood
  • Male
  • Methylprednisolone / administration & dosage
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Mucin-1
  • Mucins / blood*
  • Prednisolone / administration & dosage
  • Prednisolone / therapeutic use
  • Pulmonary Fibrosis / drug therapy*
  • Survival Rate
  • Treatment Outcome


  • Anti-Inflammatory Agents
  • Antigens
  • Antigens, Neoplasm
  • Biomarkers
  • Glucocorticoids
  • Glycoproteins
  • MUC1 protein, human
  • Mucin-1
  • Mucins
  • Prednisolone
  • L-Lactate Dehydrogenase
  • Leukocyte Elastase
  • Methylprednisolone