{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T02:04:13Z","timestamp":1793930653317,"version":"4.8.0"},"reference-count":36,"publisher":"The Journal of Rheumatology","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>To identify predictors of poor prognosis in patients with systemic sclerosis (SSc) associated with interstitial lung disease (ILD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>Fifty patients with early-stage SSc-ILD who had never received disease-modifying drugs and were either observed for \u2265 10 years or died from ILD-related causes were enrolled. The baseline variables of patients who developed endstage lung disease (ESLD) were compared with those of patients who remained ESLD-free, and the Cox proportional hazard model was used to identify initial factors that correlated with ESLD development.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>Sixteen patients (32%) developed ESLD during 173.5 \u00b1 64.7 months of followup. Elevated serum Krebs von den Lungen-6 (KL-6) at initial assessment was highly correlated with ESLD development (p = 0.0002). Receiver-operating characteristic curve analysis revealed that a KL-6 value of 1273 U\/ml effectively discriminated patients who developed ESLD from those who did not. Patients with KL-6 &gt; 1273 U\/ml were less likely to remain ESLD-free compared with those with lower KL-6 levels (p &lt; 0.0001). Multivariate analysis showed that KL-6 &gt; 1273 U\/ml was the most reliable predictor of ESLD development (OR 51.2, 95% CI 7.6\u2013343, p &lt; 0.0001). Finally, the initial KL-6 level correlated with the forced vital capacity (FVC) decline rate (r = 0.58, p &lt; 0.0001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.160339","type":"journal-article","created":{"date-parts":[[2016,8,2]],"date-time":"2016-08-02T01:58:20Z","timestamp":1470103100000},"page":"1825-1831","source":"Crossref","is-referenced-by-count":100,"title":["Elevated Serum Krebs von den Lungen-6 in Early Disease Predicts Subsequent Deterioration of Pulmonary Function in Patients with Systemic Sclerosis and Interstitial Lung Disease"],"prefix":"10.3899","volume":"43","author":[{"given":"Masataka","family":"Kuwana","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichiro","family":"Shirai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsutomu","family":"Takeuchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2016,8,1]]},"reference":[{"key":"2018112909361653000_43.10.1825.1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0034-1371541"},{"key":"2018112909361653000_43.10.1825.2","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2006.066068"},{"key":"2018112909361653000_43.10.1825.3","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00001813"},{"key":"2018112909361653000_43.10.1825.4","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.149.6.8004317"},{"key":"2018112909361653000_43.10.1825.5","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.100675"},{"key":"2018112909361653000_43.10.1825.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2010.12.009"},{"key":"2018112909361653000_43.10.1825.7","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200706-877OC"},{"key":"2018112909361653000_43.10.1825.8","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780370903"},{"key":"2018112909361653000_43.10.1825.9","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2106012"},{"key":"2018112909361653000_43.10.1825.10","doi-asserted-by":"publisher","DOI":"10.1111\/j.1752-699X.2010.00228.x"},{"key":"2018112909361653000_43.10.1825.11","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-2626"},{"key":"2018112909361653000_43.10.1825.12","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230510"},{"key":"2018112909361653000_43.10.1825.13","unstructured":"Medsger TA Jr. Systemic sclerosis (scleroderma), localized scleroderma, eosinophilic fasciitis and calcinosis. 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