BACKGROUND: Serum Krebs von den Lungen-6 (sKL-6) is an high-molecular-weight (200 kDa) glycoprotein predominantly expressed by damaged alveolar type II cells, and it has been proposed as a potential biomarker of different ILD. This is a prognostic biomarker for chronic hypersensitivity pneumonitis (cHP) and idiopathic pulmonary fibrosis (IPF), two diseases that share several clinical and radiological features. Little data are available on the potential role of KL-6 in granulomatous and cystic interstitial lung diseases, including the orphan disease known as pulmonary Langerhans cell histiocytosis (PLCH). METHODS: For the first time, sKL-6 concentrations were assayed and compared in 96 patients (17 PLCH, 22 IPF, 34 cHP) and 22 healthy controls. RESULTS: Serum KL-6 concentrations were significantly higher in PLCH (599 ± 594 U/mL), IPF (1645 ± 846 U/mL) and cHP patients (1691 ± 1643 U/mL) than in healthy controls (268 U/mL) (P = .037). Area-under-the-curve values of sKL-6 were 73.4% between PLCH and healthy controls, 84.5% between IPF and PLCH and 78% between cHP and PLCH. An indirect correlation between sKL-6 concentrations and peripheral CD1a-positive cells was demonstrated (r = -0.82; P = .034). CONCLUSION: Serum KL-6 concentrations were higher in PLCH patients than in controls, reflecting the alveolar damage typical of this rare interstitial lung disease.
BACKGROUND: Serum Krebs von den Lungen-6 (sKL-6) is an high-molecular-weight (200 kDa) glycoprotein predominantly expressed by damaged alveolar type II cells, and it has been proposed as a potential biomarker of different ILD. This is a prognostic biomarker for chronic hypersensitivity pneumonitis (cHP) and idiopathic pulmonary fibrosis (IPF), two diseases that share several clinical and radiological features. Little data are available on the potential role of KL-6 in granulomatous and cystic interstitial lung diseases, including the orphan disease known as pulmonary Langerhans cell histiocytosis (PLCH). METHODS: For the first time, sKL-6 concentrations were assayed and compared in 96 patients (17 PLCH, 22 IPF, 34 cHP) and 22 healthy controls. RESULTS: Serum KL-6 concentrations were significantly higher in PLCH (599 ± 594 U/mL), IPF (1645 ± 846 U/mL) and cHP patients (1691 ± 1643 U/mL) than in healthy controls (268 U/mL) (P = .037). Area-under-the-curve values of sKL-6 were 73.4% between PLCH and healthy controls, 84.5% between IPF and PLCH and 78% between cHP and PLCH. An indirect correlation between sKL-6 concentrations and peripheral CD1a-positive cells was demonstrated (r = -0.82; P = .034). CONCLUSION: Serum KL-6 concentrations were higher in PLCHpatients than in controls, reflecting the alveolar damage typical of this rare interstitial lung disease.
Authors: Elena Bargagli; Francesca Bellisai; Maria Antonietta Mazzei; Edoardo Conticini; Lorenzo Alderighi; Paolo Cameli; Giovanni Biasi; Laura Bergantini; Susanna Guerrini; Miriana d'Alessandro; Bruno Frediani Journal: Intern Emerg Med Date: 2020-07-29 Impact factor: 3.397
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Authors: Maria Alimova; Eriene-Heidi Sidhom; Abhigyan Satyam; Moran Dvela-Levitt; Michelle Melanson; Brian T Chamberlain; Seth L Alper; Jean Santos; Juan Gutierrez; Ayshwarya Subramanian; Elizabeth Grinkevich; Estefania Reyes Bricio; Choah Kim; Abbe Clark; Andrew Watts; Rebecca Thompson; Jamie Marshall; Juan Lorenzo Pablo; Juliana Coraor; Julie Roignot; Katherine A Vernon; Keith Keller; Alissa Campbell; Maheswarareddy Emani; Matthew Racette; Silvana Bazua-Valenti; Valeria Padovano; Astrid Weins; Stephen P McAdoo; Frederick W K Tam; Lucienne Ronco; Florence Wagner; George C Tsokos; Jillian L Shaw; Anna Greka Journal: bioRxiv Date: 2020-06-30
Authors: Maria Kost-Alimova; Eriene-Heidi Sidhom; Abhigyan Satyam; Brian T Chamberlain; Moran Dvela-Levitt; Michelle Melanson; Seth L Alper; Jean Santos; Juan Gutierrez; Ayshwarya Subramanian; Patrick J Byrne; Elizabeth Grinkevich; Estefanía Reyes-Bricio; Choah Kim; Abbe R Clark; Andrew J B Watts; Rebecca Thompson; Jamie Marshall; Juan Lorenzo Pablo; Juliana Coraor; Julie Roignot; Katherine A Vernon; Keith Keller; Alissa Campbell; Maheswarareddy Emani; Matthew Racette; Silvana Bazua-Valenti; Valeria Padovano; Astrid Weins; Stephen P McAdoo; Frederick W K Tam; Luciene Ronco; Florence Wagner; George C Tsokos; Jillian L Shaw; Anna Greka Journal: Cell Rep Med Date: 2020-10-29
Authors: Miriana d'Alessandro; Laura Bergantini; Paolo Cameli; Maria Pieroni; Rosa Metella Refini; Piersante Sestini; Elena Bargagli Journal: Cancers (Basel) Date: 2021-02-09 Impact factor: 6.639
Authors: Miriana d'Alessandro; Laura Bergantini; Dalila Cavallaro; Sara Gangi; Paolo Cameli; Edoardo Conticini; Bruno Frediani; Francesco Dotta; Elena Bargagli Journal: Int J Environ Res Public Health Date: 2022-02-15 Impact factor: 3.390