Prevalence

US Cases: 1,100 [Kent 2004], Male & Females are equally affected.

30% of people with Relapsing Polychondritis have other medical problems such as hematological disease, myelodysplastic syndrome, or gastrointestinal disorders.

Typical Age of onset

Usually a sudden onset in people between 40-60 years of age.

Symptoms

The signs and symptoms vary from person to person depending on which parts of the body are affected.

General symptoms:

• Flaring (intermittent) inflammation of cartilage (e.g., ears, nose)

• Fatigue or malaise

• Fever

Ears:

• Pain

• Swelling

• Redness

• Limpness of the ear (the most common symptom)

• Middle ear inflammation can cause obstruction and may produce symptoms of hearing impairment and dizziness

Joints:

• Rib pain

• Joint pain

• Swelling

Nose:

• Inflammation on the bridge of the nose

• Nasal congestion

• Nose bleeds (epistaxis)

• “Saddle nose” deformity caused by collapse of the cartilage at the bridge of the nose

• Nasal stuffiness or “fullness”

Airways:

Airway involvement may affect the cartilage of the larynx and bronchial tubes.

• Wheezing

• Shortness of breath

• Cough

• Hoarseness

• High-pitched sound during breathing (stridor)

• Speech difficulties

Airway problems can become life-threatening if not properly managed.

Less common:

Eye discoloration

Resources

Link to Clinical Trials

Link to Scientific Publications
 

References

Genetic and Rare Disease Information Center (GARD)
Medscape
Merck Manual
Orphanet