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An episodic, inflammatory, and destructive disorder involving cartilaginous and other connective tissues including the ear, joints, nose, larynx, trachea, eye, heart valves, kidney, and blood vessels. An autoimmune etiology is suggested by the frequent association with RA, systemic vasculitis, SLE, and other connective tissue diseases. RP occurs with equal frequency in men and women; onset typically is in middle age.
Symptoms, Signs, and Course
The most common presentation is acute pain, erythema, and swelling of the cartilaginous portions of both external ears, with an associated arthritis. The arthropathy varies from arthralgias to symmetric arthritis involving both large and small joints, with a predilection for the costochondral joints. Inflammation of the nasal cartilage is the next most common manifestation, followed in decreasing order of frequency by inflammation of the eye (conjunctivitis, scleritis, iritis, or chorioretinitis); cartilaginous tissues of the larynx, trachea, or bronchi; the internal ear; the cardiovascular system; the kidney; and the skin. The course is characterized by bouts of acute inflammation, healing over a few weeks, with recurrences over several years. Destruction of supporting cartilaginous tissues becomes prominent in the later stages of the illness and is manifested as floppy ears, saddle nose, and visual, auditory, and vestibular abnormalities. Mortality after a 5-yr illness approaches 30% and is usually due to collapse of laryngeal and tracheal cartilaginous supporting structures, or to cardiovascular involvement in the form of large vessel aneurysm, cardiac valvular insufficiency, or systemic vasculitis.
Diagnosis
Diagnosis is established clinically if over a period of time the patient develops > 3 of the following: (1) bilateral chondritis of the external ears, (2) inflammatory polyarthritis, (3) nasal chondritis, (4) ocular inflammation, (5) respiratory tract chondritis, or (6) auditory or vestibular dysfunction. Biopsy of involved cartilaginous tissue may confirm the diagnosis or help rule out alternative diagnoses such as Wegener's granulomatosis. Laboratory abnormalities are those that accompany chronic inflammation; eg, anemia, leukocytosis, and elevated ESR. Abnormal renal function or CSF pleocytosis may indicate an associated vasculitis.
Treatment
Mild cases may respond to symptomatic treatment with aspirin, indomethacin, or other NSAIDs. More severe cases are usually treated with daily doses of prednisone 30 to 60 mg, with rapid tapering of the dose as soon as there is a clinical response. Very severe cases may require the addition of immunosuppressive agents such as cyclophosphamide (see also RHEUMATOID ARTHRITIS, above). None of the above-mentioned therapies has been tested in controlled trials or appears to alter RP's ultimate course.
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Fluid & Electrolyte
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