Relapsing polychondritis is a systemic disease characterized by repeated episodes of inflammation and in some cases deterioration of cartilage. The disease can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. The exact mechanism is poorly understood. The diagnosis is based on the symptoms and supported by investigations such as blood tests and sometimes other investigations. Treatment may involve symptomatic treatment with painkillers or anti-inflammatory medications, and more severe cases may require suppression of the immune system.
Signs and symptoms Though any cartilage in the body may be affected in people with relapsing polychondritis, in many cases, the disease affects several areas while sparing others. The disease may exhibit variable signs and symptoms, resulting in a challenging diagnosis that can lead to delayed recognition for several months, years, or even decades.
Associated diseases Several other overlapping diseases associated with RP should also be considered. Approximately one-third of people with RP might be associated with other autoimmune diseases, vasculitides, and hematologic disorders. Systemic vasculitis is the most common association with RP, followed by rheumatoid arthritis and systemic lupus erythematosus. The following table displays the main diseases associated with RP.
Cartilage inflammation Cartilage inflammation (technically known as chondritis) that is relapsing is very characteristic of the disease. These recurrent episodes of inflammation over the course of the disease may result in breakdown and loss of cartilage. The signs and symptoms of cartilage inflammation in various parts of the body will be described first.
Ear
Inflammation of the cartilage of the ear is a specific symptom of the disease and affects most people. It is present in about 20% of persons with RP at presentation and in 90% at some point. Both ears are often affected, but the inflammation may alternate between either ear during a relapse. It is characteristic for the entire outer part of the ear except the earlobe to be swollen, red, or less often purplish, warm, and painful to light touch. The inflammation of the ear usually lasts a few days or more, rarely a few weeks, and then resolves spontaneously and recurs at various intervals. Because of the loss of cartilage, after several flares cauliflower ear deformity may result. The outer part of the ear may be either floppy or hardened by calcifications of the scar tissue that replaces the cartilage. These cauliflower ear deformities occur in about 10% of persons with RP.
Nose The inflammation of the cartilage of the nose involves the bridge of the nose and is often less marked than the ears. Statistics show that this clinical manifestation is present in 15% of persons with RP and occurs at some point in 65% of persons with RP. Nasal obstruction is not a common feature. Atrophy may eventually develop secondarily during the disease; this appears gradually and may go unnoticed. This can result in collapse of the nasal septum with saddle-nose deformity, which is painless but irreversible.
Respiratory tract Inflammation occurs in the laryngeal, tracheal, and bronchial cartilages. Both of these sites are involved in 10% of persons with RP at presentation and 50% over the course of this autoimmune disease, and are more common among females. The involvement of the laryngotracheobronchial cartilages may be severe and life-threatening; it causes one-third of all deaths among persons with RP. Laryngeal chondritis is manifested as pain above the thyroid gland and, more importantly, as dysphonia with a hoarse voice or transient aphonia. Because this disease is relapsing, recurrent laryngeal inflammation may result in laryngomalacia or permanent laryngeal stenosis with inspiratory dyspnea that may require emergency tracheotomy as a temporary or permanent measure. Tracheobronchial involvement may or may not be accompanied by laryngeal chondritis and is potentially the most severe manifestation of RP. The symptoms include shortness of breathing (dyspnea), wheezing, and a nonproductive cough. Obstructive respiratory failure may develop as the result of either permanent tracheal or bronchial narrowing or chondromalacia with expiratory collapse of the tracheobronchial tree. Endoscopy, intubation, or tracheotomy has been shown to hasten death.
Ribs Involvement of the rib cartilages results in costochondritis. Symptoms include chest wall pain or, less often, swelling of the involved cartilage. The involvement of the ribs is seen in 35% of persons with RP but is rarely the first symptom.
Other manifestations Relapsing polychondritis can affect various organ systems of the body. At first, some people with the disease may have only nonspecific signs and symptoms such as fever, weight loss, and malaise.
Joint The second most common clinical finding of this disease is joint pain with or without arthritis, after chondritis. All synovial joints may be affected. At presentation, around 33% of people have joint symptoms that involve polyarthralgia and/or polyarthritis or oligoarthritis that affects various parts of the body and often appears to be episodic, asymmetric, migratory, and non-deforming. The most common sites of involvement are the metacarpophalangeal joints, proximal interphalangeal joints, and knees. Other affected joints include the ankles, wrists, metatarsophalangeal joints, and the elbows. Involvement of the axial skeleton is very rare. Tests for rheumatoid factor are negative in affected persons with RP, unless there is a co-morbidity with RA. Less often, it has been reported that persons may experience arthralgia, monoarthritis, or chronic polyarthritis that mimics rheumatoid arthritis, leading to a difficult diagnosis for this disease. The appearance of erosions and destruction, however, is exceedingly rare, and this may point instead to rheumatoid arthritis as a cause. Diseases and inflammation of tendons have been reported in small numbers of people with RP. During the course of the disease, around 80% of people develop joint symptoms.
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