Relapsing Polychondritis

Relapsing polychondritis is a chronic autoimmune disease of the joints that affects more than 1 in every 1000 people. It can cause pain and discomfort and can be hard to diagnose. Fortunately, there are treatment options available that will reduce symptoms. Learn more about the condition, diagnosis, and treatment options here.

Autoimmunity

Relapsing polychondritis (RP) is an autoimmune condition that causes chronic inflammation of the cartilage in various parts of the body. It is most commonly seen in the ears and nose. The cartilage is a structure that covers the ends of bones in the joints and gives the body shape. It also supports the other organs and body parts. The main goal of treatment is to preserve the structures that have been affected.

The disease is most common in people with white ancestry. It is thought to be triggered by an immune-mediated attack on the proteins in cartilage. The severity of RP can range from mild to severe, with the latter causing a number of complications.

Patients with relapsing polychondritis have recurrent episodes of inflammation, pain, and a decrease in their ability to breathe. They may also suffer from neurological complications. In the past, researchers have not been able to pinpoint a cause for this disease. However, researchers have discovered that the symptoms of RP are similar to other rheumatic diseases.

A blood screen can help determine if a patient has relapsing polychondritis. The blood will contain antibodies that are thought to trigger the disease. In some cases, the disease can be misdiagnosed, or missed, because it is often accompanied by nonspecific symptoms. Fortunately, recent research has shown that improved diagnosis and treatments are improving outcomes for RP patients.

Polychondritis is a rare connective tissue disorder, but recent research has suggested that it is an autoimmune disease. It is believed that the body’s immune system mistakenly attacks the cartilage in the ears and nose.

Relapsing polychondritis can cause the cartilage in the ears to deteriorate and break down. This can lead to painful inflammation and anatomical deformation, including the development of a saddle nose. The disease can also affect other body parts, such as the heart valves, skin, and kidneys.

The disease is considered to be an X-linked autoimmune syndrome. The symptoms of relapsing polychondritis can mimic those of other autoimmune diseases, such as rheumatic diseases and meningoencephalitis.

Relapsing polychondritis is a complicated disorder that requires close collaboration with a doctor. It is important to be proactive and to make regular appointments to ensure that the condition is under control.

Complications

Relapsing polychondritis (RP) is a relatively uncommon disease that is characterized by recurrent episodes of inflammation and destruction of cartilaginous structures. It is an autoimmune disease that is mainly associated with systemic rheumatic diseases. It can also affect other tissues, such as the heart and eyes. The symptoms of RP include ear pain, pulmonary capillary wedge pressure, and abnormal blood examination findings. It can be a life-threatening condition if left untreated.

The most common symptom of RP is auricular chondritis. It can be disfiguring, painful, and verrucous. It can occur as a unilateral or bilateral condition. Patients often have nasal chondritis as well. Occasionally, it can be accompanied by angle closure glaucoma.

Other organs may be involved as well, although this is rarely seen. The mainstay of treatment for relapsing polychondritis is medical therapy. Corticosteroids are a good option for patients with this disease. Surgical interventions, such as phacoemulsification, are sometimes used to alleviate late ocular complications.

Relapsing polychondritis is a very debilitating disease. It can cause severe pain, loss of hearing, and sensorineural deafness. It is very important to identify RP as soon as possible. A delay in diagnosis can result in therapeutic delays.

Relapsing polychondritis affects the respiratory tract and is a risk factor for tracheobronchial collapse. It can also cause secondary open glaucoma. The ophthalmic manifestations of RP are usually refractory to non-surgical interventions. Surgical intervention, such as phacoemulsification, is very important to avoid late ocular complications. Surgical treatment is also used to treat systemic autoimmune diseases.

The patient in this case had a history of recurrent bilateral auricular chondritis. He also suffered from bilateral hearing loss and had a complicated cataract. He was treated with cyclophosphamide and prednisolone. He is now on maintenance doses of prednisolone.

The most common clinical manifestations of RP are auricular and nasal chondritis. Other anatomical areas can be affected as well, depending on the degree of inflammation. The respiratory system is thought to be involved in 50% of all RP cases. Despite its rarity, relapsing polychondritis can be a deadly disease. However, the survival rate is much higher than previously estimated.

Diagnosis

Relapsing polychondritis is a rare and immune-mediated systemic disease. It involves recurrent inflammation of the cartilage of various body parts, leading to progressive anatomical deformation. It is an autoimmune disorder that affects both genders and ages. Relapsing polychondritis can be fatal at times. It is a multisystem disorder and can present with symptoms such as skin rash, chest pain, weight loss, and ear pain. In addition, relapsing polychondritis may be associated with systemic vasculitis or other inflammatory or haematological diseases.

Diagnosis of relapsing polychondritis is made on the basis of clinical presentation, supportive laboratory data, and biopsy of affected cartilage. The most common clinical features include auricular and nasal chondritis and polyarthritis.

A recent study by Pearson et al. described four cases of relapsing polychondritis. In the case of auricular chondritis, one side of the patient was successfully operated on. It was discovered that the cartilage had been replaced with fibrous connective tissue. It was also observed that the chondritis had progressed from a remitting phase to an acute one.

A dynamic exploratory CT scan is recommended for patients with relapsing polychondritis. The dynamic CT scan is particularly helpful for evaluating functional airway abnormalities such as air trapping. It can also be useful in diagnosing subglottic stenosis.

A patient with relapsing polychondritis who is treated with corticosteroids appears to be unable to achieve remission. However, there are reports of successful treatment with high-dose prednisolone.

Relapsing polychondritis should be differentiated from other causes of small-sized vessel vasculitis. It has been shown that anti-type II collagen antibodies are increased in RP. It has also been suggested that cellular immunity plays an important role in the pathogenesis of this disease.

Relapsing polychondritis usually presents with asymmetrical and progressive anatomical deformation. The patient’s clinical picture can also include symptoms such as ocular involvement, systemic vasculitis, and atypical chest pain. In addition, the patient is at high risk for cardiovascular complications. In most cases, relapsing polychondritis leads to hydrolysis, the breakdown of the cartilage. This can lead to facial and eye abnormalities, as well as conductive deafness.

Treatment options

Relapsing polychondritis is an immune-mediated systemic disease that affects cartilage and other proteoglycan-rich structures throughout the body. This autoimmune disorder can lead to anatomical deformities and functional impairment of the involved structures. Approximately 3.5 out of every 1,000 people are diagnosed with relapsing polychondritis each year.

Treatment options for relapsing polychondritis vary with the stage of the disease and may be affected by the presence of associated comorbid conditions. However, the main goals of treatment are to control symptoms and preserve cartilage. Some patients may require immunosuppression to help manage moderate to severe symptoms.

There are a variety of medications used to treat relapsing polychondritis. Corticosteroids can be beneficial. Nebulized racemic ephedrine is used to control respiratory manifestations of the disease. Fluticasone propionate is also used. It is a glucocorticoid that is given in a bolus of one gram per day for three consecutive days. It is administered as an adjunct to analgesic supplementation.

Cyclophosphamide is a biologic that is given to some relapsing polychondritis patients. Infliximab is another agent that is used to treat the disease. It is an IL-1 receptor antagonist. Anakinra is also prescribed as an IL-1 receptor antagonist. Infliximab is the most widely used biologic.

A pneumococcal vaccine can be given to patients with relapsing polychondritis and significant laryngotracheobronchitis disease. The disease is considered a risk factor for pneumonia and other infectious diseases, so a pneumococcal vaccination can be given.

Surgery is sometimes required to correct the anatomical changes caused by relapsing polychondritis. In some cases, a saddle nose deformity is apparent. Nasal chondritis can also develop. The nose is often the most affected part of the body. Nasal chondritis can be very painful.

Relapsing polychondritis can be a life-threatening condition, especially if the patient has cardiopulmonary disease. The condition can lead to a variety of complications, including heart valve disorders. It can cause other problems such as vertigo, hoarseness, and balance issues.

The disease continues to intrigue the scientific community. It is believed to be an autoimmune disorder, with the body’s immune system mistaking healthy tissues for those containing diseased ones.

Relapsing Polychondritis – NORD (National Organization for Rare Disorders) (rarediseases.org)
Relapsing Polychondritis (RP) – Vasculitis UK