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Relapsing Polychondritis

Evidence: Antibody

Names

Atrophic polychondritis
Chronic atrophic polychondritis
Meyenburg-Altherr-Uehlinger syndrome
Polychondropathia
Relapsing perichondritis
Recurrent polychondritis
Systemic chondromalocia
Von Meyenburg disease

Description

Relapsing polychondritis (RP) occurs when the immune system attacks cartilage and other related tissues in the body, causing inflammation and tissue damage. Though the disease is frequently characterized through ears, nose, and joint symptoms, it is a multi-systemic disease. Episodes may last several days to weeks and typically resolve without treatment.

In cases where the cartilage is not visible, RP can cause heart valve abnormalities, kidney disease, neurological problems, gastrointestinal problems.

Bloggers

None found.

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