Jérôme Le Pavec1, Gwenaël Lorillon1, Xavier Jaïs2, Colas Tcherakian3, Séverine Feuillet1, Peter Dorfmüller4, Gérald Simonneau2, Marc Humbert2, Abdellatif Tazi5. 1. Centre National de Référence de l'Histiocytose Langerhansienne, Univ Paris Diderot, Sorbonne Cité, Assistance Publique Hôpitaux de Paris, Service de Pneumologie, Hôpital Saint Louis, Paris. 2. Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre; Assistance Publique Hôpitaux de Paris, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine, Béclère, Clamart; INSERM U999, Clamart. 3. Service de Pneumologie, Hôpital Foch, Suresnes. 4. Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre; INSERM U999, Clamart; Service d'Anatomie et Cytologie Pathologiques, Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France. 5. Centre National de Référence de l'Histiocytose Langerhansienne, Univ Paris Diderot, Sorbonne Cité, Assistance Publique Hôpitaux de Paris, Service de Pneumologie, Hôpital Saint Louis, Paris. Electronic address: abdellatif.tazi@sls.aphp.fr.
Abstract
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 wereas 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/m 2 , and pulmonary vascular resistance (PVR) of 555 ±253 dyne/s/cm 5. Use of PAH therapy in 12 patients was followed by an improvement in mPAP (56±14 mm Hg and 45±12 mm Hg, P 5 .03) and PVR (701±239 dyne/s/cm 5 and 469±210 dyne/s/cm 5 , 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
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 wereas 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/m 2 , and pulmonary vascular resistance (PVR) of 555 ±253 dyne/s/cm 5. Use of PAH therapy in 12 patients was followed by an improvement in mPAP (56±14 mm Hg and 45±12 mm Hg, P 5 .03) and PVR (701±239 dyne/s/cm 5 and 469±210 dyne/s/cm 5 , 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
Authors: Nishant Gupta; Robert Vassallo; Kathryn A Wikenheiser-Brokamp; Francis X McCormack Journal: Am J Respir Crit Care Med Date: 2015-06-15 Impact factor: 21.405
Authors: Michael Girschikofsky; Maurizio Arico; Diego Castillo; Anthony Chu; Claus Doberauer; Joachim Fichter; Julien Haroche; Gregory A Kaltsas; Polyzois Makras; Angelo V Marzano; Mathilde de Menthon; Oliver Micke; Emanuela Passoni; Heinrich M Seegenschmiedt; Abdellatif Tazi; Kenneth L McClain Journal: Orphanet J Rare Dis Date: 2013-05-14 Impact factor: 4.123