ESSENTIALS OF DIAGNOSIS
Auricular chondritis (spares the earlobe).
Inflammation in other cartilaginous areas (eg, the nose, joints, trachea, ribcage, and airways) and in tissues rich in proteoglycans, such as the eyes and heart valves.
Frequently associated with an underlying disorder such as systemic vasculitis, connective tissue disease, or myelodysplastic syndrome.
Relapsing polychondritis (RP) is an immune-mediated condition associated with inflammation in cartilaginous structures such as the ears, nose, joints, larynx, and trachea. Noncartilaginous connective tissues throughout the body, such as the eyes, heart, aorta, inner ear, and skin, can also be affected. The prevalence of RP is estimated to be approximately 4.5 cases per million people. The disease onset is generally between ages 30 and 60, with an average of 50, though pediatric cases as well as older onset have been described as well. RP affects men and women relatively equally. Thirty percent of RP cases occur in association with another disease, usually some form of systemic vasculitis (particularly granulomatosis with polyangiitis), connective tissue disorder (eg, rheumatoid arthritis or systemic lupus erythematosus), or a myelodysplastic syndrome. RP is often assumed to be autoimmune in nature, but the evidence for a true autoimmune pathogenesis is relatively weak. Some patients have been reported to have antibodies to type 2 collagen, but these assays are not widely available and their poor sensitivities and specificities make them inappropriate for general clinical use. In general, a cartilage biopsy is not required to make the diagnosis. Rather, the identification of cartilaginous inflammation in typical areas (auricular cartilage, nasal bridge, costochondral joints) and the exclusion of other possible causes suffice.
RP is associated with a broad range of clinical courses. One end of the disease spectrum includes intermittent bouts of auricular cartilage inflammation that respond quickly to treatment. The other end is characterized by widespread, aggressive cartilaginous inflammation that leads to serious end-organ complications. The greatest clinical challenge is identifying the presence of cartilaginous inflammation and instituting effective therapy before irreparable damage occurs in the involved organs.
Table 47–1 lists the major clinical manifestations of RP.
Table 47–1.Major clinical manifestations of relapsing polychondritis. ||Download (.pdf) Table 47–1. Major clinical manifestations of relapsing polychondritis.
|Feature Data ||Data |
|Mean age at diagnosis ||47 years |
|Auricular chondritis ||90% |
|Reduced hearing ||37% |
|Nasal chondritis ||60% |
|Saddle-nose deformities ||25% |
|Laryngotracheal involvement ||52% |
|Ocular inflammation ||54% |
|Arthritis ||69% |
|Skin involvement ||25% |
|Aortic or mitral regurgitation ||8% |
|Vasculitis ||12% |
Unilateral or bilateral auricular chondritis is often the first disease symptom (Figure 47–1). The onset of auricular inflammation is usually quite abrupt and not subtle. The ...