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

Research output: Contribution to journalArticle

8 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)13-15
Number of pages3
JournalRespiratory Medicine Case Reports
Volume14
DOIs
StatePublished - Jan 1 2015

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Langerhans Cell Histiocytosis
Vasodilator Agents
Pulmonary Hypertension
Lung
Therapeutics
Langerhans Cells
Lung Diseases

Keywords

  • Interstitial lung disease
  • Pulmonary hypertension
  • Pulmonary Langerhans cell histiocytosis
  • Vasodilator therapy

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

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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.",
keywords = "Interstitial lung disease, Pulmonary hypertension, Pulmonary Langerhans cell histiocytosis, Vasodilator therapy",
author = "Adam May and Kane, {Garvan M} and Eunhee Yi and Robert Frantz and Robert Vassallo",
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T1 - Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

AU - May, Adam

AU - Kane, Garvan M

AU - Yi, Eunhee

AU - Frantz, Robert

AU - Vassallo, Robert

PY - 2015/1/1

Y1 - 2015/1/1

N2 - 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.

AB - 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.

KW - Interstitial lung disease

KW - Pulmonary hypertension

KW - Pulmonary Langerhans cell histiocytosis

KW - Vasodilator therapy

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