%0 English Abstract
%T [Tracheobronchial involvement in relapsing polychondritis and differential diagnoses].
%A Grandière L
%A Gille T
%A Brillet PY
%A Didier M
%A Freynet O
%A Vicaire H
%A Clero D
%A Martinod E
%A Mathian A
%A Uzunhan Y
%J Rev Mal Respir
%V 41
%N 6
%D 2024 Jun 17
%M 38762394
%F 0.714
%R 10.1016/j.rmr.2024.03.009
%X Relapsing polychondritis is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the cartilages involved. In addition to characteristic damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are particularly severe, and can seriously alter the prognosis. Tracheobronchial lesions are assessed by means of a multimodal approach, including dynamic thoracic imaging, measurement of pulmonary function (with recent emphasis on pulse oscillometry), and mapping of tracheal lesions through flexible bronchoscopy. Diagnosis can be difficult in the absence of specific diagnostic tools, especially as there may exist a large number of differential diagnoses, particularly as regards inflammatory diseases. The prognosis has improved, due largely to upgraded interventional bronchoscopy techniques and the development of immunosuppressant drugs and targeted therapies, offering patients a number of treatment options.