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Original Research: Pulmonary Vascular Disease |

Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary HypertensionPulmonary Langerhans Cell Histiocytosis: Clinical Characteristics and Impact of Pulmonary Arterial Hypertension Therapies

Jérôme Le Pavec, MD, PhD; Gwenaël Lorillon, MD; Xavier Jaïs, MD; Colas Tcherakian, MD; Séverine Feuillet, MD; Peter Dorfmüller, MD, PhD; Gérald Simonneau, MD; Marc Humbert, MD, PhD; Abdellatif Tazi, MD, PhD
Author and Funding Information

From the Centre National de Référence de l’Histiocytose Langerhansienne (Drs LePavec, Lorillon, Feuillet, and Tazi), Univ Paris Diderot, Sorbonne Cité, Assistance Publique Hôpitaux de Paris, Service de Pneumologie, Hôpital Saint Louis, Paris; Université Paris-Sud (Drs Jaïs, Dorfmüller, Simonneau, and Humbert), Faculté de Médecine, Kremlin-Bicêtre; Assistance Publique Hôpitaux de Paris (Drs Jaïs, Simonneau, and Humbert), Centre National de Référence de l’Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine, Béclère, Clamart; INSERM U999 (Drs Jaïs, Dorfmüller, Simonneau, and Humbert), Clamart; Service de Pneumologie (Dr Tcherakian), Hôpital Foch, Suresnes; and the Service d’Anatomie et Cytologie Pathologiques (Dr Dorfmüller), Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France.

Correspondence to: Abdellatif Tazi, MD, PhD, Service de Pneumologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75475, Paris cedex 10, France; e-mail: abdellatif.tazi@sls.aphp.fr


For editorial comment see page 1087

Funding/Support: The authors have reported to CHEST that no funding was received for this study.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2012;142(5):1150-1157. doi:10.1378/chest.11-2490
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Background:  Precapillary pulmonary hypertension (PH) is a complication of pulmonary Langerhans cell histiocytosis (PLCH) associated with increased mortality. However, outcomes and efficacy of pulmonary arterial hypertension (PAH) therapies in patients with PH complicating PLCH (PLCH-PH) remain unknown.

Methods:  Consecutive patients with PLCH with PH confirmed by right-sided heart catheterization were included in the study. Characteristics at baseline and during follow-up as well as survival were analyzed.

Results:  Twenty-nine patients were studied. Baseline characteristics of patients with PLCH-PH were as follows: 83% of patients in World Health Organization (WHO) functional class III to IV, mean 6-min walk distance of 355 ± 95 m, mean pulmonary arterial pressure (mPAP) of 45 ± 14 mm Hg, cardiac index of 3.2 ± 0.9 L/min/m2, and pulmonary vascular resistance (PVR) of 555 ± 253 dyne/s/cm5. Use of PAH therapy in 12 patients was followed by an improvement in mPAP (56 ± 14 mm Hg and 45 ± 12 mm Hg, P = .03) and PVR (701 ± 239 dyne/s/cm5 and 469 ± 210 dyne/s/cm5, P = .01) between baseline and follow-up evaluations. No significant oxygen worsening was observed in the treated group. The 1-, 3-, and 5-year survival estimates of the 29 patients were 96%, 92%, and 73%, respectively. Except a trend toward a better survival rate associated with the use of PAH therapy, WHO functional class was the only variable significantly associated with death.

Conclusions:  In this group of patients, PAH therapies improved hemodynamics without oxygen worsening or pulmonary edema. WHO functional class was the only prognostic factor identified. Prospective clinical trials focusing on this population of patients are warranted.

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