Am. J. Respir. Crit. Care Med., Volume 161, Number 1, January 2000, 216-223

Severe Pulmonary Hypertension in Histiocytosis X

MURIEL FARTOUKH, MARC HUMBERT, FRÉDÉRIQUE CAPRON, SOPHIE MAÎTRE, FLORENCE PARENT, CATHERINE LE GALL, OLIVIER SITBON, PHILIPPE HERVÉ, PIERRE DUROUX, and GÉRALD SIMONNEAU

UPRES EA 2705 (Maladies Vasculaires Pulmonaires), Service de Pneumologie et Réanimation Respiratoire, Service d'Anatomie Pathologique, et Service de Radiologie, Hôpital Antoine Béclère, Assistance Publique des Hôpitaux de Paris, Clamart, France

Diminished exercise capacity in advanced pulmonary histiocytosis X does not appear to be related to ventilatory limitation but may be related to pulmonary vascular dysfunction. Pulmonary hemodynamics and respiratory function were studied in 21 consecutive patients with advanced pulmonary histiocytosis X, and compared with parameters of patients with other severe chronic lung diseases (29 patients with chronic obstructive pulmonary disease and 14 patients with idiopathic pulmonary fibrosis). All patients with pulmonary histiocytosis X displayed severe pulmonary hypertension: mean pulmonary arterial pressure, 59 ± 4 mm Hg; cardiac index, 2.6 ± 0.8 L/min/m²; and total vascular pulmonary resistance, 25 ± 3 IU/m² (p < 0.05, as compared with patients with other chronic lung diseases). Pa_O2 was similar in the three groups, whereas FEV₁ was lower in patients with other chronic lung diseases (p < 0.05). In contrast to other chronic lung diseases, the degree of pulmonary hypertension was not related to variables of pulmonary function in pulmonary histiocytosis X. Histopathology was available for 12 patients with pulmonary histiocytosis X and revealed proliferative vasculopathy involving muscular arteries and veins, with prominent venular involvement. Two consecutive lung samples (taken before and after the occurrence of pulmonary hypertension) were available for six patients with pulmonary histiocytosis X, and showed that pulmonary vasculopathy worsened, whereas parenchymal and bronchiolar lesions remained relatively unchanged. These results indicate that pulmonary hypertension in pulmonary histiocytosis X might be related to an intrinsic pulmonary vascular disease, in which the pulmonary circulation is involved independent of small airway and lung parenchyma injury. Fartoukh M, Humbert M, Capron F, Maître S, Parent F, Le Gall C, Sitbon O, Hervé P, Duroux P, Simonneau G. Severe pulmonary hypertension in histiocytosis X.

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