Published in Presse medicale (Paris, France : 1983)
Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is the least frequent antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). Major advances of our knowledge on its pathophysiology have revealed features of both AAV and eosinophilic disorders. The development of targeted biotherapies for both dise...
Published in Presse medicale (Paris, France : 1983)
Lung involvement is one of the most common clinical features in ANCA-associated vasculitides (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In this review, we detail the five main presentations of pulmonary involvement in AAV: necrotizing granulomato...
Published in Presse medicale (Paris, France : 1983)
Treatment of vasculitides has benefited from the results of several prospective clinical trials focusing on the evaluation of new drugs, therapeutic strategies and adjuvant treatments. In the field of autoimmunity, vasculitides are the group of diseases for which the most important medical progress has been made, combining advances in understanding...
Published in Presse medicale (Paris, France : 1983)
Idiopathic pulmonary fibrosis (IPF) is a chronic and devastating disease of unknown etiology, characterized by irreversible morphological changes, ultimately leading to lung fibrosis and death. In recent years, significant progress has been achieved in understanding the pathogenesis of IPF. Moreover, we assisted to the conceptual change of the path...
Published in Presse medicale (Paris, France : 1983)
Idiopathic pulmonary fibrosis (IPF) is characterized by relentlessly progressive lung function impairment that is consistently fatal in the absence of lung transplantation, as no curative pharmacological treatment exists. The pace of progression varies across patients, and acute life-threatening exacerbations occur unpredictably, causing further sh...
Published in Presse medicale (Paris, France : 1983)
At least 10% of patients with pulmonary fibrosis, whether idiopathic or secondary, present heritable pulmonary fibrosis suspected on familial aggregation of pulmonary fibrosis, specific syndromes or early age of diagnosis. Approximately 30% of those patients have an identified mutation mostly in telomere related genes (TRG) more rarely in surfactan...
Published in Presse medicale (Paris, France : 1983)
Published in Presse medicale (Paris, France : 1983)
Interstitial lung diseases encompass a broad range of numerous individual conditions, some of them characterized histologically by fibrosis, especially idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, chronic hypersensitivity pneumonia, interstitial lung disease associated with connective tissue diseases, and unclassifiable inters...
Published in Presse medicale (Paris, France : 1983)
Published in Presse medicale (Paris, France : 1983)
