Relapsing Polychondritis

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Relapsing Polychondritis
 
 
 Relapsing polychondritis is an episodic, inflammatory, and destructive disorder involving primarily cartilage of the ear and nose but also potentially affecting the eyes, tracheobronchial tree, heart valves, kidneys, joints, skin, and blood vessels. Diagnosis is clinical. Treatment requires prednisone and sometimes immunosuppressants.

Relapsing polychondritis affects men and women equally; onset typically is in middle age. An association with RA, systemic vasculitis, SLE, and other connective tissue diseases suggests an autoimmune etiology.

~Symptoms and Signs

Acute pain, erythema, and swelling most commonly affect the pinna cartilage. Nasal cartilage inflammation is the next most common, followed by arthritis that varies from arthralgias to symmetric or asymmetric nondeforming arthritis involving large and small joints, with a predilection for the costochondral joints. The next most common manifestations, in decreasing order of frequency, are inflammation of the eye (conjunctivitis, scleritis, iritis, keratitis, chorioretinitis); cartilaginous tissue of the larynx, trachea, or bronchi (causing hoarseness, cough, and tenderness); internal ear; cardiovascular system (aortic regurgitation, pericarditis, myocarditis, aortic aneurysms, aortitis); kidney; and skin. Bouts of acute inflammation heal over weeks to months, with recurrences over several years.

Advanced disease can lead to destruction of supporting cartilage, producing floppy ears, saddle nose, pectus excavatum, and visual, auditory, and vestibular abnormalities. Tracheal narrowing can lead to pneumonia or even tracheal collapse. Systemic vasculitis (leukocytoclastic vasculitis or polyarteritis nodosa), myelodysplastic syndrome, or malignancy occasionally develops.

~Diagnosis

Diagnosis is established if the patient develops at least 3 of the following: bilateral chondritis of the external ears, inflammatory polyarthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis, or auditory or vestibular dysfunction. Biopsy of involved cartilage is helpful if clinical diagnosis is not clear-cut.

Laboratory tests are not necessary but may help decrease the likelihood of other disorders. Synovial fluid is usually mildly inflammatory, if at all. Blood tests may show normocytic-normochromic anemia, leukocytosis, elevated ESR or γ-globulin levels, and occasionally positive rheumatoid factor, antinuclear antibodies (ANA), or in up to 25%, antineutrophil cytoplasmic antibodies (ANCA).

Abnormal renal function may indicate an associated vasculitis. A positive c-ANCA test (ANCA that are reactive mainly to proteinase-3) suggests Wegener's granulomatosis, which can cause similar findings (see Vasculitis: Wegener's Granulomatosis).

Patients need close monitoring, usually with CT, for evidence of tracheal narrowing.

~Prognosis and Treatment

Mortality after 5 yr is 30%, from collapse of laryngeal and tracheal structures or from cardiovascular complications such as large-vessel aneurysm, cardiac valvular insufficiency, or systemic vasculitis.

Mild disease may respond to NSAIDs in anti-inflammatory doses. However, most patients are treated with prednisone 30 to 60 mg po once/day, with tapering of the dose as soon as there is a clinical response. Some patients require chronic use.
In such patients, methotrexate 7.5 to 20 mg po once/wk can reduce the requirement for corticosteroids. Very severe cases may require other immunosuppressants, such as cyclosporine, cyclophosphamide, or azathioprine .
 None of these therapies have been tested in controlled trials or been shown to decrease mortality. If tracheal narrowing causes stridor, a tracheostomy or stent may be needed.

 
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