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INTERSTITIAL PNEUMONIAE WITH AUTOIMMUNE MANIFESTATIONS. JOINT CONSENSUS OF THE ERS/ATS

Abstract

Some patients with idiopathic interstitial pneumonias (IIPs) have extrathoracic signs and symptoms suggestive for coexisting autoimmune disease that, however, do not meet criteria for any connective tissue disease (CTD). Currently, there are no unique terms and criteria for these cases. The European Respiratory Society and the American Thoracic Society created the Task Force Group on Undifferentiated Forms of CTD Associated with Interstitial Lung Disease in order to develop consensus on terminology and diagnostic criteria for patients with IIP and clinical features of an autoimmune disorder. The Task Force created the term «interstitial pneumonia with autoimmune features (IPAF)» and diagnostic criteria based on three groups of signs: clinical signs including specific clinical extrathoracic features; serological features including specific autoantibodies and morphological features including imaging, histology and respiratory physiology. Experts supposed that the term IPAF should facilitate identification patients with IIP and specific autoimmune signs but without a definitive CTD. This term should also provide involvement a uniform cohort of patients in clinical studies.

About the Author

статья Редакционная

Russian Federation


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  INTERSTITIAL PNEUMONIAE WITH AUTOIMMUNE MANIFESTATIONS. JOINT CONSENSUS OF THE ERS/ATS. PULMONOLOGIYA. 2016;26(5):529-538. (In Russ.)

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