RT Book, Section
A1 Yazdany, Jinoos
A1 Manno, Rebecca
A1 Hellmann, David B.
A1 Imboden Jr., John B.
A2 Papadakis, Maxine A.
A2 McPhee, Stephen J.
A2 Rabow, Michael W.
SR Print(0)
ID 1175803636
T1 Relapsing Polychondritis
T2 Current Medical Diagnosis & Treatment 2021
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260469868
LK accessmedicine.mhmedical.com/content.aspx?aid=1175803636
RD 2021/02/28
AB This  disease  is  characterized  by  inflammatory  destructive  lesions  of  cartilaginous  structures,  principally  the  ears,  nose,  trachea,  and  larynx.  Nearly  40%  of  cases  are  associated  with  another  disease,  especially  either  other  immunologic  disorders  (such  as  SLE,  rheumatoid  arthritis,  or  Hashimoto  thyroiditis)  or  cancers  (such  as  plasma  cell  myeloma)  or  hematologic  disorders  (such  as  myelodysplastic  syndrome).  The  disease,  which  is  usually  episodic,  affects  males  and  females  equally.  The  cartilage  is  painful,  swollen,  and  tender  during  an  attack  and  subsequently  becomes  atrophic,  resulting  in  permanent  deformity.  Biopsy  of  the  involved  cartilage  shows  inflammation  and  chondrolysis.  Laryngotracheal  and  bronchial  chondritis  can  lead  to  life-threatening  airway  narrowing  and  collapse.  Noncartilaginous  manifestations  of  the  disease  include  fever,  episcleritis,  uveitis,  deafness,  aortic  regurgitation,  and  rarely  glomerulonephritis.  In  85%  of  patients,  a  migratory,  asymmetric,  and  seronegative  arthropathy  occurs,  affecting  both  large  and  small  joints  and  the  costochondral  junctions.  Diagnosing  this  uncommon  disease  is  especially  difficult  since  the  signs  of  cartilage  inflammation  (such  as  red  ears  or  nasal  pain)  may  be  more  subtle  than  the  fever,  arthritis,  rash,  or  other  systemic  manifestations.