title: A confusing case report of pulmonary langerhans cell histiocytosis and literature review.
abstract: Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease. From the insidious onset and nonspecific manifestations, it is difficult to diagnose PLCH. To help improve the diagnosis and therapy options of adult PLCH, we present this case report and literature review about a confusing case of PLCH. In this report, we present a 37-year-old male PLCH case that was negative for CD1a and S100 expression. Smoking cessation and use of prescribed Spiriva appeared to improve the patient's symptoms. To the best of our knowledge, this is the first reported case of PLCH in which improved symptoms were seen with the use of Spiriva alone.The mechanism is not clear, but potentially has some relationship with dilating the airway, decreasing the mucous hypersecretion and promoting anti-inflammatory pathways. From this patient's case, we may be able to find more cases to then find other first line therapies for PLCH patients.
authors.affiliation: Department of Respiratory Medicine, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
authors.forename: Yang
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Respiratory Medicine, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
authors.forename: Wang
authors.initials: W
authors.lastname: Zhen
authors.affiliation: Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Costable
authors.affiliation: Department of Respiratory Medicine, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
authors.forename: Xu
authors.initials: X
authors.lastname: Jun
authors.affiliation: Department of Thoracic Surgery, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
authors.forename: Ren
authors.initials: R
authors.lastname: Zhe
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
authors.forename: Mao
authors.initials: M
authors.lastname: YuPing
keywords: Interstitial lung disease; Pulmonary Langerhans cell histiocytosis; SR-ILD; Smoking-Related Interstial Lung Disease; Spiriva; Tiotropium