Introduction
Relapsing polychondritis is an autoimmune disease characterized by inflammation of the cartilage and connective tissues in many body organs. Jaksh-Warten was the first to describe it in 1923. The term relapsing polychondritis was coined by Pearson and his co-workers in 1960. These identified the condition as inflammation of both cartilaginous and noncartilaginous structures.
What Is Relapsing Polychondritis?
Relapsing polychondritis (RP) is considered a degenerative disorder that exhibits recurrent inflammation of the cartilage in the body. The weakening of cartilage affects all parts consisting of cartilage. The cartilage is a connective tissue present throughout the body. The most affected parts are ear, nose, trachea, and larynx. The aortic heart valve may be affected by this condition.
Other names of relapsing polychondritis include:
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Chronic atrophic polychondritis.
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Generalized or systemic chondromalacia.
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Meyenburg-Altherr-Uehlinger syndrome.
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Relapsing perichondritis.
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Von Meyenburg disease.
Relapsing chondritis can be seen among anyone but usually affects individuals of 40 years to 60 years. It was estimated that 1 in 2,85,000 are affected by this condition. 30 percent of cases are found to be linked with autoimmune or hematologic comorbidity.
What Are the Causes of Relapsing Polychondritis?
The cause of this condition is not known. Some researchers consider it may be an autoimmune disorder (the body’s immune system attacks the healthy tissues). A genetically predisposed person upon exposure to a triggering factor may be affected by this condition.
The triggering factors include trauma, infectious agents, and chemical or toxic exposure. During trauma, the cryptogenic antigen may be released and identified by the immune system. Studies related to genes have found that the HLA-DR4 antigen has shown an association with relapsing polychondritis.
Other researchers have noticed that relapsing polychondritis may be caused by immunologic sensitivity to type II collagen that is present in the skin and connective tissues.
Almost 80 percent of individuals with RP exhibit auricular chondritis, and polyarthritis although many organs are involved. It begins insidiously, with an acute painful inflammatory crisis. This is followed by spontaneous remission which is of variable duration. This makes it difficult to diagnose at an early stage which leads to therapeutic delay and life-threatening sequelae.
RP is found to be linked with other autoimmune disorders like rheumatoid arthritis (RA).
What Are the Symptoms of Relapsing Polychondritis?
Relapsing polychondritis is found to occur in episodes or flares. The episodes were found to occur for a few days or weeks and found to occur repeatedly in a year.
The symptoms of relapsing polychondritis include:
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Experience pain, tenderness, swelling, and redness in one or both ears. The cartilage present in both inside and outside of the ear may be affected but the ear lobe is not affected. The ears may appear floppy.
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Saddle nose deformity may be seen due to the collapse of cartilage at the bridge of the nose.
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Experience joint pains that are similar to arthritis.
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Nasal congestion may occur.
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Experience pain near the breast bone.
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Pain and redness may occur in the eyes.
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Hearing loss, tinnitus, and vertigo may occur if the inner ear is affected.
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Difficulty in breathing may be experienced if the trachea is affected.
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Difficulty in speech may be felt if the larynx is affected.
Sometimes individuals with relapsing polychondritis may affect the heart valve, and experience heart murmur, vision loss, and kidney disease.
How to Diagnose Relapsing Polychondritis?
The diagnosis is based on the combination of clinical features, radiographic findings, and biopsy of the cartilaginous site. Clinically this condition can be diagnosed if at least three symptoms are found over a period.
A CT scan should be done to learn about airway involvement. Pulmonary function test is done to airway trapping, and to know about lung volumes. (Positron emission tomography) PET-CT (computed tomography) is a new technique that aids with old methods and helps target the biopsy site. A biopsy of the ear cartilage and blood tests help diagnose the condition.
How to Treat Relapsing Polychondritis?
There is no cure for relapsing polychondritis. The symptoms of this condition are managed and chances of complications are decreased with the treatment. The treatment includes:
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Corticosteroids: Prednisone is used to treat this condition.
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Dapsone: This drug is used as an antiinfective agent.
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Non-steroidal Anti-inflammatory Drugs: These drugs such as colchicine are used for pain relief.
In severe cases, the drugs given are:
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Cyclophosphamide or Azathioprine: These are given to suppress the immune system.
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Methotrexate: A type of chemotherapy used in the case of arthritis.
Some cases may require surgery. These include:
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Heart valve surgery.
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Insertion of breathing tube (tracheostomy).
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Some procedures like inserting a laryngeal or tracheal stent which helps prop open the airway, or airway dilation. The balloon is used to stretch the airway.
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Surgery is done to reconstruct the larynx and trachea.
There are no ways to prevent relapsing polychondritis as the cause is not known.
What Is the Prognosis of Relapsing Polychondritis?
The life expectancy is considered to be good in the case of RP. It was noticed that almost 95
percent of affected individuals live for eight years after the diagnosis. Life-threatening conditions may be caused and other complications like breathing problems, vision loss, and hearing loss may occur.
If a person is known about RP, regular visits to healthcare providers are a must. If any complications such as breathing problems, vision or hearing loss, immediate medical attention should be sought as early as possible.
What Are the Disorders That Are Similar to Relapsing Polychondritis?
The disorders that are similar to RP include:
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Rheumatoid Arthritis: It is an autoimmune disorder that exhibits symptoms like lack of appetite, tiredness, painful and deformed joints, and early morning stiffness in joints of hands, knees, and feet.
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Osteoarthritis: It is a degenerative disorder affecting cartilage and bony joints.
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Behcet’s Syndrome: It is an inflammatory disorder affecting many organs.
Conclusion
Relapsing polychondritis is an autoimmune disease affecting the cartilaginous tissue and can affect many organs of the body. It is difficult to identify the cause of the condition and treatments are available to reduce the symptoms and complications. It is difficult to detect the disease at an early stage. Hence it becomes important to know about the condition, its cause, symptoms, and management. Knowing about the condition helps identify the condition early. Early diagnosis helps in effective treatment. This in turn helps to lead a good quality of life.
