Revue des maladies respiratoires
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Pulmonary fibrosis is a frequent and serious complication of scleroderma whose pathophysiology remains poorly understood. The alveolar structures are infiltrated by activated chronic inflammatory cells, alveolar macrophages and polymorphonuclear neutrophils in particular and these could play a determining role. We have studied the state of activation of alveolar macrophages and monocytes circulating in these patients who presented with scleroderma and interstitial pulmonary involvement and also in healthy subjects. ⋯ The neutrophil alveolitis is accompanied by a breakdown in the equilibrium of elastase-antielastase which could participate in the development of alveolar lesions leading to fibrosis. In addition to the activation of macrophages, there is an activation of monocytes marked by the increase in secretion of interleukin-6 and interleukin-8 in vitro during the progression of the disease of scleroderma. Thus, alveolar inflammation is integrated with the overall systemic inflammation whose causes remain unknown.
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Primary pulmonary arterial hypertension (PPH) is a rare disorder with a predilection for young subjects (most commonly of 20-40 years) and of the female sex. The prognosis is very poor because the average life expectancy is of the order of two to three years from the time of diagnosis. ⋯ A very recent case-control study has shown that any use of anorexic drugs (mainly fenfluramine derivatives) was associated with an increased risk of PPH (odds ratio: 6/1), and particularly when the duration of treatment exceeds 3 months. Outside the group of appetite suppressants few medications are capable of favouring the development of PPH, the cases are sporadic and the relationship between cause and effect is hardly established.