Am. J. Respir. Crit. Care Med., Volume 158, Number 5, November 1998, 1680-1684

Circulating KL-6 Predicts the Outcome of Rapidly Progressive Idiopathic Pulmonary Fibrosis

AKIHITO YOKOYAMA, NOBUOKI KOHNO, HIRONOBU HAMADA, MITSUNORI SAKATANI, EINOSUKE UEDA, KEIICHI KONDO, YUTAKA HIRASAWA, and KUNIO HIWADA

Second Department of Internal Medicine, Ehime University School of Medicine, Ehime; and Department of Internal Medicine, National Kinki-Chuo Hospital for Chest Diseases, Nagasone, Sakai, Osaka, Japan

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.

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