What is Relapsing Polychondritis?

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Relapsing Polychondritis (RP) is a rare, progressive condition characterised by recurrent episodes of inflammation of cartilaginous structures.  These include the ears, nose, upper respiratory tract, chest wall and joints.  Other organs and tissues may also become involved such as the eye, inner ear, heart, blood vessels and kidneys.   

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The exact cause of Relapsing Polychondritis (RP) is not known.  However, it is believed to be an autoimmune disorder where the body's immune system starts attacking its own healthy cells believing them to be foreign.  

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RP has an episodic pattern with periods of intense inflammation and periods of remission and dependant on the severity of the inflammatory process, often requiring the use of immune suppressant medications.

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RP can be life threatening, debilitating and difficult to diagnose and prognosis is variable, depending on organ involvement and treatment complications.   Where the disease is mild or responds quickly to treatment, there is a good prognosis.

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Relapsing Polychondritis is also known by other names such as Meyenburg Altherr Uehlinger syndrome, chronic atrophic polychondritis and Von Meyenburg syndrome.

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Symptoms

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Patients may present with any or all of the following common signs of Relapsing Polychondritis:

 

• swelling and pain of the nose, 

• painful inflammation and redness of the ears 

• joint pain, swelling and stiffness.

• if airways are involved, breathlessness, stridor, throat pain and hoarseness in voice.

• fever, fatigue and weight loss

• eye scleritis, redness and inflammation 

 

Diagnosis

 

Diagnosis is difficult as the various symptoms may be similar to and mistaken for other, more common diseases.  There are no blood tests which can confirm RP and diagnosis is usually made on clinical symptoms alone. Due to the rarity of the condition, clinicians are often unfamiliar with the symptoms and patients occasionally wait years before getting a diagnosis.  

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Doctors will diagnose Relapsing Polychondritis on observing at least three of the following:

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• inflammation of cartilage in the ears (excluding the soft lower earlobe)

• pain and swelling in several joints

• inflammation of the nose cartilage

• eye inflammation

• inflammation of the cartilage in the windpipe

• hearing loss/tinnitus/vertigo

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Treatment

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Relapsing Polychondritis does not have a cure but it can often be well controlled with medication.  

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Disease-modifying anti rheumatic drugs like Methotrexate and Prednisolone (Corticosteroids) are often given to suppress the immune system.  For milder cases of the condition, non-steroidal, anti-inflammatory drugs and painkillers may be prescribed.  

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Where the airways are involved, surgery may be required to repair the windpipe (trachea) or stenting to keep the airway open.  

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