[Pulmonary hypertension complicating systemic diseases other than connective tissue diseases].
A COMPLICATION OF CERTAIN SYSTEMIC DISEASES: Pulmonary hypertension (PH) can complicate the progression of certain systemic diseases such as sarcoidosis, histiocytosis X and some vasculites. The mechanisms at the origin of PH are varied and always require rigorous analysis in order to optimise treatment.
PH associated with sarcoidosis is essentially related to specific lung parenchymal fibrosis and is poorly responder to corticosteroids. Other mechanisms may be more rarely incriminated (compressive andenopathies, mediastinal fibrosis, florid sarcoidosis concomitant to a pulmonary occlusive vascular disease...). During histiocytosis X, the ventilatory limitation of these patients does not always correlate with the severity of the respiratory failure, suggesting the existence of a pulmonary vascular disease progressing independently of the pulmonary parenchymal lesions. The pulmonary artery damage during Takayasu's arteritis and other auto-immune pulmonary arteritis may lead to potentially life-threatening complications, notably through stenosis and/or obstruction of the pulmonary arteries. Pulmonary hypertension is exceptional during Wegener's disease or periateritis nodosa.


CONCLUSIONS
PH can complicate the progression of certain systemic diseases. The physiopathological mechanisms responsible are unclear (specific parenchymal fibrosis, isolated vascular involvement...). Globally, available treatments are disappointing.
Sanchez
O
O
Centre des maladies vasculaires pulmonaires (UPRES EA 2705), Service de pneumologie et réanimation respiratoire, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart.
Garcia
G
G
Hamid
A
A
Nunes
H
H
Ioos
V
V
Andriamihoatra
T H Razafy
TH
Parent
F
F
Simonneau
G
G
Humbert
M
M
D006328:Cardiac Catheterization; D018450:Disease Progression; D014890:Granulomatosis with Polyangiitis / Q000150:complications; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D017565:Sarcoidosis, Pulmonary / Q000150:complications*; D013625:Takayasu Arteritis / Q000150:complications*