Relapsing polychondritis
Relapsing polychondritis (RP) is an uncommon and severe episodic inflammatory condition
involving cartilaginous structures, predominantly those of the ear, nose, and
laryngotracheobronchial tree. Other affected structures may include the eye,
cardiovascular system, peripheral joints, middle ear, and inner ear. In 1923,
Jaksch-Wartenhorst described a patient who experienced an 18-month course of progressive
degeneration of the peripheral joints, external ears, nasal septum, external auditory
canals, inner ear, and epiglottis. He termed this condition polychondropathia.
In 1960, Pearson, Kline, and Newcomer reviewed 12 cases and expanded the clinical
spectrum of RP to include nonconcurrent inflammation of the auricles, nasal septum,
peripheral joints, and larynx, with occasional involvement of the middle and inner ears,
the eyes, costal cartilages, spine, trachea, bronchi, and epiglottis. They noted that
after a few episodes of inflammation, the cartilage was replaced by fibrous connective
tissue. The term relapsing polychondritis was introduced in that review.
Symptoms of Relapsing polychondritis
Typically, relapsing polychondritis causes sudden pain in the inflamed tissue at the
onset of the disease. Common symptoms are pain, redness, swelling, and tenderness in one
or both ears, the nose, throat, joints and/or eyes. Fever, fatigue, and weight loss often
develop.
Inflammation of the ears and nose can cause deformity (saddle nose deformity and floppy
ears) from weakened cartilage. Impaired hearing, balance, and nausea can be caused by
inner ear inflammation.
Inflammation of the windpipe, or trachea, can lead to throat pain, hoarseness, and
breathing difficulty. This is a potentially dangerous area of inflammation in patients
with relapsing polychondritis which can require assisted breathing methods when severe.
Joint inflammation (arthritis) can cause pain, swelling, and stiffness of the joints,
including of the hands, knees, ankles, wrists, and feet.
Eye inflammation can be mild or severe and can damage vision. Cataracts can be caused
by the inflammation or from the cortisone used to treat relapsing polychondritis (see
below).
Other tissues that can develop inflammation include the aorta (which can lead to
aneurysm or aortic valve weakness), tissues in or around the heart (myocarditis and
pericarditis), the skin (vasculitis), and the nerves from the brain (cranial nerve
palsies).
Diagnosis of Relapsing polychondritis
A characteristic array of symptoms and physical findings will yield a diagnosis of
relapsing polychondritis. Laboratory tests are sometime helpful. Biopsies of the affected
cartilage may confirm the diagnosis. Further diagnostic test are done to confirm other
associated conditions such as rheumatoid arthritis. It is important to evaluate the
airway, although only 10% of patients will die from airway complications.
Treatment of Relapsing polychondritis
Mild inflammations can be treated with aspirin or nonsteroidal anti-inflammatory drugs
(NSAIDs) such as ibuprofen. Corticosteroids (most often prednisone) are usually prescribed
for more advanced conditions and do improve the disease. They may have to be continued
over long periods of time, in which case their usage must be closely watched to avoid
complications. Immune suppression with cyclophosphamide, azathioprine, cyclosporine, or
dapsone is reserved for more aggressive cases. A collapsed chest or airway may require
surgical support, and a heart valve or aorta may need repair or replacing.
Prognosis of Relapsing polychondritis
There is no known cure for relapsing polychondritis. It can only be combated with each
onset of inflammation and deterioration of cartilaginous tissue. As the disease progresses
over a period of years, the mortality rate increases. At five years duration, relapsing
polychondritis has a 30% mortality rate |
