Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

Adam May; Garvan Kane; Eunhee Yi; Robert Frantz; Robert Vassallo

doi:10.1016/j.rmcr.2014.11.005

Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

Respir Med Case Rep. 2014 Nov 22:14:13-5. doi: 10.1016/j.rmcr.2014.11.005. eCollection 2015.

Authors

Adam May¹, Garvan Kane², Eunhee Yi³, Robert Frantz², Robert Vassallo⁴

Affiliations

¹ Department of Medicine, Mayo Clinic, Rochester, MN, 55905, USA.
² Division of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, 55905, USA.
³ Department of Laboratory Medicine and Anatomic Pathology, Mayo Clinic, Rochester, MN, 55905, USA.
⁴ Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, 55905, USA.

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.

Keywords: Interstitial lung disease; Pulmonary Langerhans cell histiocytosis; Pulmonary hypertension; Vasodilator therapy.

Publication types

Case Reports