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  • Print publication year: 2011
  • Online publication date: October 2011

Case 3 - Relapsing polychondritis

Summary

Imaging description

Relapsing polychondritis is a multi-system disorder that can involve the cartilage of the external ear, nose, larynx, trachea, and central bronchi. This disorder is characterized by recurrent inflammation and destruction of the cartilage in these areas. Although uncommon at presentation, respiratory tract involvement occurs in up to 50% of patients at some point during the course of the illness [1–4]. Involved airways are typically thickened along the cartilaginous portions of the wall with sparing of the posterior membranous portion of the wall (Figures 3.1 and 3.2). The wall thickening is typically smooth and diffuse [1–4] and often has increased attenuation ranging from subtly increased to obviously calcified [1] (Figure 3.3). In one-third of patients the airways are narrowed. When narrowing is present it may be either diffuse or focal (Figure 3.4). When expiratory imaging is obtained, tracheomalacia and/or bronchomalacia can be seen in over 50% of patients [1, 2] (Figure 3.5).

References
Behar, JVChoi, Y-WHartman, TERelapsing polychondritis affecting the lower respiratory tractAJR Am J Roentgenol 2002 178 173
Tillie-LeBlond, IWallaert, BLeBlond, DRespiratory involvement in relapsing polychondritis: clinical, functional, endoscopic, and radiographic evaluationsMedicine 1998 77 168
Marom, EMGoodman, PCMcAdams, HPDiffuse abnormalities of trachea and main bronchiAJR Am J Roentgenol 2001 176 713
Ernst, ARafeq, SBoiselle, PRelapsing polychondritis and airway involvementChest 2009 135 1024