Relapsing polychondritis

Abstract

Relapsing polychondritis is a unique, rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction but the immune damage can spread to involve noncartilaginous tissues like the kidney, blood vessels, and so forth. The manifestations of the disease can take many different forms and the pathogenesis is still unclear. It may occur in a primary form or it may be associated with other disease states. This article summarizes important aspects of the disease with a focus on recent information regarding clinical manifestations, disease associations, pathogenesis, and advances in therapeutics.

Relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms

“…5,7 Currently, the diagnosis is made on the basis of demonstration of either chondritis in two of three sites (auricular, nasal, laryngotracheal); or one of these sites and two additional features, including ocular inflammation, audiovestibular damage, or sero-negative inflammatory arthritis. 1,9 There are no specific laboratory findings. 5,7 Neurological symptoms occur in a minority of cases (3%) and may range from compromise of cranial nerves to a more overt presentation with cerebellar compromise, seizures or other focal findings suggestive of cortical compromise.…”

“…1,2 We report the case of a patient with relapsing polychondritis with several neurological manifestations.…”

Polychondritis Associated with Chronic Hepatitis C Virus Infection: Figure 1.

“…Relapsing polychondritis is a rare disease of unknown etiology and pathogenesis 4 . The pathogenesis is considered to be autoimmune, based on increased frequency of human leukocyte antigen (HLA)-DR4 in these patients, and detection of antibodies to type II collagen.…”

A Patient with Relapsing Polychondritis who Had Been Diagnosed as Intractable Bronchial Asthma

“…This is a rare disorder with an estimated incidence of 3.5/ 1,000,000 persons/year (2), and the treatment has not been established. When tracheal/bronchial cartilages are affected, respiratory symptoms such as dyspnea and stridor may appear (3), which can be misleading, prompting an improper diagnosis as bronchial asthma (4).…”