pulmonary disorders
Pulmonary Langerhans Cell Histiocytosis
Prognosis for PLCH
Spontaneous resolution of symptoms occurs in some patients with minimally symptomatic pulmonary Langerhans cell histiocytosis. Overall, the 10-year survival is >90% ( Prognosis reference ). The clinical markers for progressive disease include Continued smoking Age extremes Multiorgan involvement Persistent constitutional symptoms Numerous cysts on chest x-ray Reduced DLCO Low forced expiratory volume in 1 second (FEV1)/ forced vital capacity (FVC) ratio (<66%) High residual volume (RV)/total lung capacity (TLC) ratio (> 33%) Need for prolonged corticosteroid use Cause of death is respiratory insufficiency or cancer. Lung Carcinoma risk is increased because of cigarette smoking. 1.Benattia A, Bugnet E, Walter-Petrich A, et al. Long-term outcomes of adult pulmonary Langerhans cell histiocytosis: a prospective cohort.Eur Respir J2022;59(5):2101017. Published 2022 May 26. doi:10.1183/13993003.01017-2021 