relapsing polychondritis


Also found in: Wikipedia.

Relapsing Polychondritis

 

Definition

Relapsing polychondritis is a disease characterized by autoimmune-like episodic or progressive inflammation of cartilage and other connective tissue, such as the nose, ears, throat, joints, kidneys, and heart.

Description

Cartilage is a tough, flexible tissue that turns into bone in many places in the body. Bones all start out as cartilage in the fetus. Consequently, children have more cartilage than adults. Cartilage persists in adults in the linings of joints, the ears, the nose, the airway and the ribs near the breast bone. All these sites are attacked by relapsing polychondritis, which usually occurs equally in middle-aged males and females. It is frequently diagnosed along with rheumatoid arthritis, systemic lupus erythematosus, and other connective tissue diseases.

Causes and symptoms

The most common first symptom of relapsing polychondritis is pain and swelling of the external ear. Usually, both ears turn red or purple and are tender to the touch. The swelling can extend into the ear canal and beyond, causing ear infections, hearing loss, balance disturbances with vertigo and vomiting, and eventually a droopy ear. The nose is often afflicted as well and can deteriorate into a flattened nose bridge called saddle nose. Inflammation of the eye occurs less frequently, but can lead to blindness.
As relapsing polychondritis advances, it causes more dangerous symptoms such as deterioration of the cartilage that holds the windpipe open. Progressive disease can destroy the integrity of the airway and compromise breathing. Destruction of the rib cartilage can collapse the chest, again hindering breathing. Joints everywhere are involved in episodes of arthritis, with pain and swelling. Other tissues besides cartilage are also involved, leading to a variety of problems with the skin and other tissues. Occasionally, the aorta or heart valves are damaged.
The disease may occur in episodes with complete remission between, or it may smolder along for years, causing progressive destruction.

Diagnosis

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 tests 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

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.

Key terms

Aorta — The biggest artery in the body, receiving blood directly from the heart.
Connective tissue — Several types of tissue that hold the body's parts together-tendons, ligaments, fascia, and cartilage.
Inflammation — The body's immune reaction to presumed foreign substances like germs. Inflammation is characterized by increased blood supply and activation of defense mechanisms. It produces redness, swelling, heat, and pain.

Prognosis

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.

Resources

Books

Gilliland, Bruce C. "Relapsing Polychondritis and Other Arthritides." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.

polychondritis

 [pol″e-kon-dri´tis]
inflammation of many cartilages of the body.
chronic atrophic polychondritis (polychondritis chro´nica atro´phicans) (relapsing polychondritis) an acquired disease of unknown origin, chiefly involving various cartilages and showing both chronicity and a tendency to recurrence; it is marked by inflammatory and degenerative lesions of various cartilaginous structures.

re·lap·sing pol·y·chon·dri·tis

a degenerative and inflammatory disease of cartilage producing arthritis, collapse of the pinna, the cartilaginous portion of the nose, and the tracheobronchial tree; death may occur from chronic infection or suffocation because of loss of stability in the tracheobronchial tree; of autosomal genetic transmission.

relapsing polychondritis

a rare disease of unknown cause resulting in inflammation and destruction of cartilage with replacement by fibrous tissue. Autoimmunity may be involved in this condition. Most commonly the ears and noses of middle-aged people are affected with episodes of tender swelling, often accompanied by fever, arthralgias, and episcleritis. Consequences include floppy ears, collapsed nose, hearing loss, or hoarseness and airway obstruction because of laryngeal and tracheal cartilage involvement. Corticosteroids suppress the activity of the disease.

relapsing polychondritis

A rare condition characterised by inflammation and cartilaginous degeneration, beginning about age 40.

Clinical findings
Fever, vasculitis, arthropathy.
 
Diagnosis
3+ of following symptoms, in descending frequency: auricular chondritis with external ear drooping, non-erosive arthritis, nasal chondritis with saddle nose deformity, upper respiratory obstruction, audiovestibular symptoms, cardiovascular disease—e.g., aortic valve insufficiency.
 
Prognosis
74% 5-year, 55% 10-year survival.

relapsing polychondritis

Dermatology An uncommon condition characterized by inflammation and cartilaginous degeneration, beginning about age 40 Clinical Fever, vasculitis, arthropathy Diagnosis 3+ of following Sx–in descending frequency: auricular chondritis with ear drooping, non-erosive arthritis, nasal chondritis with saddle nose deformity, upper respiratory obstruction, audiovestibular Sx, cardiovascular disease–eg, aortic valve insufficiency Prognosis 74% 5–yr, 55% 10–yr survival

re·lap·sing pol·y·chon·dri·tis

(rē-lap'sing pol'ē-kon-drī'tis)
A hereditary degenerative disease of cartilage producing a bizarre form of arthritis, with collapse of the ears, the cartilaginous portion of the nose, and the tracheobronchial tree; death may occur from chronic infection or suffocation because of loss of stability in the tracheobronchial tree.
Synonym(s): Meyenburg disease.

Meyenburg,

H. von, Swiss pathologist, 1877–.
Meyenburg complex - clusters of small bile ducts occurring in polycystic livers, separate from the portal areas.
Meyenburg disease - a degenerative disease of cartilage. Synonym(s): Meyenburg-Altherr-Uehlinger syndrome; relapsing polychondritis; von Meyenburg disease
Meyenburg-Altherr-Uehlinger syndrome - Synonym(s): Meyenburg disease
von Meyenburg disease - Synonym(s): Meyenburg disease

polychondritis

inflammation of many cartilages of the body.

relapsing polychondritis
affected cats have a history of swollen, painful ears which eventually become deformed; most cats are infected with feline leukemia or feline immunodeficiency viruses.
References in periodicals archive ?
There are more than 100 known autoimmune diseases, including psoriasis, Graves' disease, Sjogren's syndrome, Type 1 diabetes, relapsing polychondritis, multiple sclerosis, rheumatoid arthritis, Crohn's disease and lupus.
Relapsing polychondritis (RPC) is a rare autoimmune disorder that results in inflammation and destruction of cartilaginous structures throughout the body, including the ears, nose, joints and laryngotracheobronchial tree.
Saddle-nose deformity can occur as a result of trauma to the nose, but it has also been well described in the setting of infections such as leprosy and syphilis and idiopathic inflammatory conditions such as granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) and relapsing polychondritis.
Relapsing polychondritis (RP) is a disease which progresses with inflammatory attacks on articular and non-articular cartilaginous tissue and is most commonly encountered as auricular chondritis.
Relapsing polychondritis and malignant lymphoma: is polychondritis paraneoplastic?
Relapsing polychondritis with multifocal neurological abnormalities.
We present the case of a patient with KFD and systemic lupus erythematosus, as well as relapsing polychondritis.
We describe the anaesthetic management of a patient with relapsing polychondritis who underwent laparoscopic cholecystectomy.
June 4, 2015 /PRNewswire/ -- The Relapsing Polychondritis Awareness and Support Foundation is proud to release its first documentary, " RP The Ride of My Life," chronicling one woman's journey to live life with purpose while suffering from Relapsing Polychondritis (RP), a rare autoimmune disease.
Total hearing loss and blindness caused by relapsing polychondritis and rnyelodysplastic syndrome.
Coalition member Tom Christie of Anoka, Minnesota, is the co-founder of a nonprofit organization for people with relapsing polychondritis, a painful and potentially fatal condition.
Nasal (notably mucosal) involvement has been reported in various vasculitic and connective tissue diseases, such as Wegener granulomatosis, (14) Churg-Strausssyndrome, (15) systemic lupus erythematosus, (16,17) Sjogren syndrome, (18) systemic sclerosis, (19) and relapsing polychondritis.