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ABOUT
RP

Relapsing Polychondritis (RP) is a rare, progressive condition characterised by recurrent episodes of inflammation of cartilaginous structures.

WHAT IS RP?

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, lower 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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RP is very rare with an estimated prevalence of 9/1,000,000 and an incidence of 0.7/1,000,000 year in the UK.  

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The exact cause of 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, depending on the severity of the inflammatory process, often requires the use of immune suppressant medications.

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

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Specialist involved in RP are usually Rheumatology, ENT, Respiratory, Pulmonary, Ophthalmology, Cardiology and others depending on symptoms.

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SYMPTOMS.

Patients may present with any or all of the following common signs of RP - (their reported frequency):

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  • Ear chondritis - painful inflammation, hearing loss and redness (>90%)

  • Nose chondritis - swelling and pain of the nose (>50%)

  • Eye inflammation - scleritis and redness (>50%)

  • Musculoskeletal - joint pain, swelling and stiffness (>50%)

  • Airway - breathlessness, stridor, hoarseness, stenosis (>30%)

  • General fever, fatigue and weight loss (20%)

  • Vessel & Heart valves - (>10%)

  • Skin inflammation (10%)

DIAGNOSIS.

Diagnosis is difficult as the various symptoms may be similar to and mistaken for other, more common diseases. There is no single blood tests or imaging test that can be used to confirm RP.  The  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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Click here to see real patient photographs of RP.

TREATMENT.

RP does not yet have a cure but it can often be well controlled with medication.  There is no established standard therapy and treatment is based upon symptoms and prevention of progression.

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Disease-modifying anti-rheumatic drugs (DMARDs) 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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