Abstract
Relapsing polychondritis is a rare inflammatory disease involving essentially cartilaginous structures. Other systemic manifestations can be encountered as the eye and ear disturbance. Aortic aneurysms affect few cases. If affection of airway cartilages occurs, prognosis may be worsened by eventual stenotic lesions. We report a 22-year-old woman with Relapsing polychondritis. When she was referred to our hospital 4 weeks after the onset of respiratory symptoms, she was having severe breathing difficulty. Immediate tracheostomy followed by steroid therapy improved her respiratory condition, although the treatment was complicated because of her diabetes. While airway involvement of Relapsing polychondritis can be life threatening, it is curable with steroid therapy. Clinicians should keep in mind that airway obstruction could be caused by this disease.
Highlights
Relapsing polychondritis (RP) is a disease of unknown etiology characterized by recurrent non-infectious inflammation of cartilaginous and connective tissues
The aim of this study is to show that RP diagnosis should be considered in case of airway narrowing
RP is a relapsing degenerative disease of cartilaginous tissues characterized by the involvement of multiple organs and vessels, including the nose, ears, throat, trachea, eyes, joints and cardiac valves; the etiology of RP is still not fully understood [1]-[3]
Summary
Relapsing polychondritis (RP) is a disease of unknown etiology characterized by recurrent non-infectious inflammation of cartilaginous and connective tissues. It is an uncommon, chronic, and potentially life-threatening multisystem disorder in case of laryngeal and tracheobronchial disturbance. The etiology of RP is still unknown, but the pathogenetic role of the autoimmunity is suggested by frequent overlaps with various autoimmune diseases, and by the presence of autoantibody against cartilage in the serum of patients with RP [1] [2]. Clinical presentations of RP vary considerably from patient to patient, and the involvement of multiple organs has been reported. Several reports have demonstrated the clinical signs, pathologic manifestations and. International Journal of Clinical Medicine, 6, 646-651. It is difficult to show the diagnosis of RP [1] [2]
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