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Recurrent polychondritis: causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
Relapsing polychondritis is an episodic inflammatory and destructive disease that primarily affects the cartilage of the ear and nose, but can also affect the eyes, tracheobronchial tree, heart valves, kidneys, joints, skin, and blood vessels.
Diagnosis is made clinically. Treatment of relapsing polychondritis is carried out with prednisolone, in some cases - immunosuppressants.
Relapsing polychondritis occurs equally in men and women; the highest incidence is in middle-aged individuals. The association with RA, systemic vasculitis, SLE, and other connective tissue diseases suggests an autoimmune etiology of the disease.
[ Symptoms of relapsing polychondritis
The most common manifestations are acute pain, erythema, and swelling of the cartilages of the auricle. Less common are lesions of the nasal cartilages, and even less common are arthritis, ranging from arthralgia to symmetrical and asymmetrical non-deforming arthritis, involving large and small joints, with predominant lesions of the costochondral articulations. Further, in decreasing order of frequency, come: lesions of the eyes (conjunctivitis, scleritis, iritis, keratitis, chorioretinitis), cartilages of the larynx, trachea, and bronchi (hoarseness, cough), inner ear, cardiovascular system (aortic regurgitation, pericarditis, myocarditis, aortic aneurysm, aortitis), kidneys, and skin. Attacks of acute inflammation last from several weeks to several months; repeated attacks are observed after several years.
Progression of the disease can lead to destruction of supporting cartilages with the development of drooping ears, saddle nose deformity, funnel chest deformity, visual, auditory and vestibular disorders, tracheal stenosis. In rare cases, systemic vasculitis (leukocytoclastic vasculitis or subacute polyarteritis), myelodysplastic syndrome, malignant tumors may develop.
Diagnosis of relapsing polychondritis
The diagnosis is established if the patient has at least three of the following symptoms: bilateral chondritis of the outer ear, inflammatory polyarthritis, chondritis of the nasal cartilages, inflammation of the eyes, chondritis of the respiratory tract, auditory or vestibular dysfunction. If the diagnosis is difficult, it is useful to perform a biopsy of the cartilages involved in the pathological process.
Laboratory tests are not necessary but may be useful to exclude other diseases. Signs of moderate inflammation may be found in the synovial fluid. Blood may show normocytic and normochromic anemia, leukocytosis, increased ESR or gamma globulin concentrations, sometimes rheumatoid factor, antinuclear antibodies (AHA), and in 25% of patients, antineutrophil cytoplasmic antibodies. Impaired renal function may indicate the addition of vasculitis. Detection of antineutrophil cytoplasmic antibodies, primarily binding to proteinase-3, suggests the presence of Wegener's granulomatosis in the patient, which has a similar clinical picture.
Patients, especially those with tracheal involvement, require constant monitoring to assess the degree of tracheal narrowing using CT.
What do need to examine?
Treatment of relapsing polychondritis
The five-year mortality rate for this disease is 30%, the main causes being stenosis of the larynx and trachea, as well as cardiovascular complications (aneurysms of large vessels, damage to heart valves, systemic vasculitis).
In mild cases, NSAIDs may be prescribed. However, most patients are prescribed oral prednisolone at a dose of 30 to 60 mg once daily, with subsequent dose reduction immediately after clinical improvement. In some cases, long-term glucocorticoid therapy may be required. In such patients, glucocorticoid dose reduction is possible in combination with methotrexate at a dose of 7.5 to 20 mg orally for a week. Severe cases of the disease may also require the use of other immunosuppressants, in particular, cyclosporine, cyclophosphamide, azathioprine. However, none of these treatments have been studied in controlled clinical trials and have not demonstrated a decrease in mortality. In the development of tracheal stenosis complicated by stridor breathing, tracheotomy and stent placement may be required.