Relapsing Polychondritis Clinical Review
Relapsing polychondritis is an uncommon, multisystem disease that can be life-threatening, debilitating, and difficult to diagnose. It is characterized by recurrent, potentially severe, and frightening episodes of inflammation of cartilaginous tissues. All types of cartilage may be involved, including the elastic cartilage of the ears and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites, and the cartilage in the tracheobronchial tree. Relapsing polychondritis can also inflame other proteoglycan-rich structures, such as the eye, heart, blood vessels, and inner ear. Systemic symptoms (for example, fever, lethargy, and weight loss) are common, and vasculitis affecting skin or internal organs may occur. Patients can present with a wide array of painful symptoms that often pose major diagnostic dilemmas. Because no specific tests for it are available, relapsing polychondritis must be diagnosed on clinical grounds. Pathogenetically, a linkage to HLADR4 and, occasionally, other autoimmune disease suggests that antiself reactions may be operative.
Ann Intern Med 1998; 129:114-122.
David E. Trentham, MD, and Christine H. Le, MBBS, FRACP
Type: Reference Material
Author/Contact: David Trentham
Institution: Not Available
Submitted by: admin
Added: Sat Mar 17 2007