[Airway involvement in relapsing polychondritis].
Relapsing Polychondritis (RP) is an uncommon, chronic, and potentially life-threatening multisystem disorder characterized by recurrent inflammatory episodes affecting the cartilaginous tissues of the external ears, nose, peripheral joints, larynx and tracheobronchial tree, sometimes leading to their destruction. RP can also inflame other proteoglycan-rich structures, such as the eye, heart, blood vessels and inner ears. Systemic symptoms are common, and vasculitis affecting skin or internal organs may occur. 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. Although several reports have demonstrated the clinicopathologic manifestations and radiologic findings of RP, there are no specific features of RP. Therefore, it is difficult to show the diagnosis of RP. Airway involvements are major causes of morbidity and mortality, and they have accounted for most of the deaths due to RP. To suppress the inflammation of airway mucosa and cartilage is extremely important in the successful treatment for RP. Above all, earlier diagnosis would lead to better outcomes.
Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Japan.
Naoto
N
Azuma
D000293:Adolescent; D000368:Aged; D000369:Aged, 80 and over; D058109:Airway Management; D001323:Autoantibodies; D064236:Cartilage Oligomeric Matrix Protein; D003094:Collagen / Q000276:immunology; D016326:Extracellular Matrix Proteins / Q000276:immunology; D005260:Female; D006023:Glycoproteins / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan / Q000453:epidemiology; D008297:Male; D064235:Matrilin Proteins; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D011239:Prednisolone / Q000627:therapeutic use; D012140:Respiratory Tract Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D015607:Stents; D014057:Tomography, X-Ray Computed; D014140:Tracheotomy; D055815:Young Adult
D001323:Autoantibodies; D064236:Cartilage Oligomeric Matrix Protein; D016326:Extracellular Matrix Proteins; D006023:Glycoproteins; D007166:Immunosuppressive Agents; D064235:Matrilin Proteins; C577553:TSP5 protein, human; D003094:Collagen; D011239:Prednisolone