title: Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.
abstract: Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.
authors.affiliation: Department of Medicine, Mayo Clinic, Rochester, MN, 55905, USA.
authors.forename: Adam
authors.initials: A
authors.lastname: May
authors.affiliation: Division of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, 55905, USA.
authors.forename: Garvan
authors.initials: G
authors.lastname: Kane
authors.affiliation: Department of Laboratory Medicine and Anatomic Pathology, Mayo Clinic, Rochester, MN, 55905, USA.
authors.forename: Eunhee
authors.initials: E
authors.lastname: Yi
authors.affiliation: Division of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, 55905, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Frantz
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, 55905, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Vassallo
keywords: Interstitial lung disease; Pulmonary Langerhans cell histiocytosis; Pulmonary hypertension; Vasodilator therapy