Relapsing Polychondritis

What is Relapsing Polychondritis?

Relapsing polychondritis is an autoimmune disorder that presents with many non specific symptoms involving neck, nose, head, throat and ears.

It is characterized by recurring inflammation of cartilage on different body tissues. The tissues that have cartilage and are affected by relapsing polychondritis are the nose, ears, windpipe, spine and joints.

The eyes, blood vessels and even the heart have the biochemical makeup that is comparable to that of the cartilage and are also at the risk of being affected. The condition is associated with the autoimmune disorders and presence of some antibodies to collagen type II.

This factor led to the suggestion the condition can be medicated immunological way. It has been found that 25 to 35 % of   those who suffer from (RP) relapsing polychondritis also suffer from other types of autoimmune diseases. Relapsing polychondritis is  a term that was  used  for the first time in 1960 for this  rare condition that makes the  cartilaginous  structures  and  similar tissues to be affected by recurrent  inflammation.

Relapsing Polychondritis Causes

The real causes of relapsing polychondritis are yet to be established. The doctors suspect that the inflammation is a result of a disorder in the immune system (autoimmunity) which makes the immunity system –which   fights off the infections that invade the body- to be misguided.

Epidemiology

• The annual  incidence is  about  3.5  per one million
• The disease might occur at any age but the peak age of those who are mostly affected is 40 and 50 years.
• The females are mostly affected by other autoimmune diseases but relapsing polychondritis affects both genders equally.
• The disease can occur to people from all races but it is more prevalent in the Caucasians.

Relapsing Polychondritis Symptoms

Relapsing polychondritis presents itself in various ways including:

• Loss of weight
• Pain on the external ear which occurs in majority of the cases.
• Dizziness
• Vestibular  origin ataxia
• Hearing impairment.  46 percent of patients in later stages suffer from the hearing problem.
• Arthralgia with 50 to 85 percent of the cases affecting the synovial joints.
• Chondritis and nasal pain.
• Hoarse voice and difficulty when speaking
• Laryngeal, glottic and subglottic inflammation.

Other relapsing polychondritis symptoms include:

• Oedema
• Dyspnoea, cough, chocking and wheezing.
• Costochondritis which may cause flailing of the chest and joint dissolution.
• Recurrent episcleritis and scleritis.
• Cardiovascular effects including problems of the valve, myocarditis, pericarditis, aneurysms and myocardial infarction.
• Glomerulosclerosis and renal involvement.
• Cranial  lesions on the nerves  and  aneurysms

Diagnosis

Relapsing polychondritis is a rare disorder and it has no exact diagnostic test. Various possible symptoms and diagnostic criteria that are used to establish the presence of the disease have been invented. The most common criterion is known as McAdam. It involves seeking the following features:

• Recurrent chondritis on both  auricles
• Chondritis of respiratory  tract  including  tracheal  cartilage  and  laryngeal
• Inflammatory but non- erosive  polyarthritis
• Inflammation on the ocular  structures
• Vestibular  or cochlear  damage

In addition a cartilage biopsy can be taken to confirm whether the histological picture is compatible. Biopsy cartilage cannot be taken lightly. A cartilage does not have its own supply of blood and this makes it to heal poorly. In some cases, it does not heal.

There are three grounds for confirming a relapsing polychondritis diagnosis. These are:

• At least three criteria where histological confirmation is not required.
• One or even more features including the histological confirmation.
• Chondritis  in  two or even more  different  anatomical  locations  with the response to  the steroids  or  dapsone.

There is an alternative criterion that has been suggested. The proposal has 2 routes that can be used for diagnosis. These are:

• Proven inflammation on two of the three cartilages. These are the nasal, laryngotracheal or auricular cartilages.
• Proven inflammation in one of the three cartilages (laryngotracheal, auricular or nasal) and 2 more signs including the ocular inflammation, seronegative arthritis, hearing loss and vestibular dysfunction.

Differential Diagnosis

There are some conditions with symptoms that are similar to that of relapsing polychondritis. These conditions are known to occur together with relapsing polychondritis.  Lupus erythematosus and Wegener’s granulomatosis are conditions that resemble RP and have to be eliminated before carrying out a diagnosis for the condition. The other conditions that have similarities with the clinical presentation of RP are allergic reactions, tumor and trauma.

Relapsing Polychondritis Treatment

Treatment of relapsing polychondritis can be done using different systems. It is however important to come up with a suitable team approach. There should be good communication with the specialist who will be assisting the patient to manage the disease. The management can be done by using   both drug and non drug methods.

Non Drug Management

Since relapsing polychondritis affects the nasal and auricular cartilage, there is a need to provide   the patient with counseling and any other kind of support that is necessary at that moment. The facial appearance of the patient is affected and it might be necessary for the patient to seek specialist advice on cosmetic in order to determine the right way of addressing the problem.

Medications for Relapsing Polychondritis

Corticosteroids are the most common therapeutic measure. It brings relief from symptoms and reduces severity and duration of the relapses. However they do not alter the progression of the disease.

The other agents that are used to manage relapsing polychondritis are methotrexate, colchicines, azathioprine, cyclophosphamide and dapsone. These involve small numbers. Controlled trials using these agents have never been performed. Anti TNF- alpha drug has been used in recent times.

Surgery

Tracheostomy is performed if the upper airways have collapsed.  Male patients are more likely to get heart valve ailments and aortic valve replacements might be necessary.

Relapsing Polychondritis Complications

There are complications that may arise as a result of having relapsing polychondritis. This is because the condition makes the cartilage to collapse and this ends up causing the face to disfigure. It can also cause the airways to be obstructed. The structures in the eyes and ears of those who are affected might also collapse and results of such occurrences are quite devastating. The other common complications that might be caused by RP are unrelated malignancy and vasculitis.

Relapsing Polychondritis Prognosis

Relapsing polychondritis follows a degenerating and remitting course as it continues to progress steadily. The prognosis for any person will vary significantly depending on the systems that are involved.  It also depends with the way that those who are suffering from the condition respond to therapy.

With early diagnosis of the condition and provision of optimal therapy, there is an improvement of prognosis. There was a study whose results indicated that the survival rate can be as high as 94 percent with the average follow up being eight years. Up to 50 percent of the deaths are going to be as a result of pneumonia as the cartilage that is in respiratory system is affected.

The other common cause of the death among those who are affected by relapsing polychondritis is cardiovascular involvement.   A study by another group concluded that only 10 percent of all deaths are as a result of respiratory disease. The group that carried out the study   found that the most deaths are as a result of by malignancy, systemic vasculitis and infection associated with relapsing polychondritis.