Serum KL-6 levels in Pulmonary Langerhans' Cell Histiocytosis

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 is 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).