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Interstitial lung disease in connective tissue disorders
Authors:Mouthon Luc  Berezné Alice  Brauner Michel  Valeyre Dominique  Guillevin Loïc
Affiliation:1. Service de Médecine Interne, Hôpital Cochin, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75679 Paris Cedex 14, et Université Paris V;2. Service de Radiologie, Hôpital Avicenne, AP-HP, Bobigny, et Université Paris XIII;3. Service de Pneumologie, Hôpital Avicenne, AP-HP, Bobigny, et Université Paris XIII;1. Hospices Civils de Lyon, Groupement Hospitalier Est, Service de pneumologie, Centre de référence des maladies pulmonaires rares de l’adulte, 69677 Lyon cedex, France;2. Université Claude Bernard Lyon 1, 69100 Villeurbanne, France;1. Université Paris – Sud, AP-HP, Centre de Référence de l’Hypertension Pulmonaire, Service de Pneumologie, Hôpital de Bicêtre, INSERM UMR_S999, 94270 Le Kremlin Bicêtre, France;1. Service de rhumatologie, hôpital Bichat, DHU FIRE, AP–HP, 46, rue Henri-Huchard, 75018 Paris, France;2. Université Paris-Diderot, 75018 Paris, France;3. Inserm UMR 1152, 75018 Paris, France;1. Service de médecine interne et immunologie clinique, centre de référence des maladies neuromusculaires rares, hôpital Pitié-Salpêtrière, AP–HP, 47-83, boulevard de l’Hôpital, 75615 Paris cedex 13, France;2. Inserm U-1135, CIMI Paris, Sorbonne universités, UPMC, 75013 Paris, France;3. Service de pneumologie, centre constitutif du centre de référence maladies pulmonaires rares, hôpital Bichat, AP–HP, 75018 Paris, France;4. Inserm U-1152, 75018 Paris, France;5. Department of medicine, University of Colorado School of Medecine, Aurora, 80045 Colorado, États-Unis
Abstract:Interstitial lung diseases (ILD) associated with connective tissue disorders differ from idiopathic ILD in several aspects, although most of them are comparable. In most patients, ILD occurs during the course, or at the time of diagnosis of connective tissue disease. Opportunistic pulmonary infections, together with adverse effects of treatment should always be discussed. The prevalence of ILD varies among the different connective tissue disorders. Thus, ILD is frequently encountered in patients with systemic sclerosis and to a lesser degree in patients with myositis. As compared to idiopathic ILD, histopathological aspects of ILD associated with connective tissue diseases are more frequently those of non-specific interstitial pneumonia, whereas usual interstitial pneumonia is rare. Other ILD, such as organized pneumonia, interstitial lymphoid pneumonia, diffuse alveolar damage and alveolar hemorrhage are occasionally encountered. ILD must be detected early in the course of collagen disorders by performing computed tomodensitometry and pulmonary function tests. The prognosis of connective tissue associated ILD is better than that of idiopathic ILD. The treatment requires corticosteroids and/or immunosuppressants, depending on the nature of the associated connective tissue disease and ILD progression.
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