Recurrent polychondritis: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Recurrent polychondritis is an episodic inflammatory and destructive disease primarily affecting the cartilage of the ear and nose, but also capable of damaging the eyes, tracheobronchial tree, heart valves, kidneys, joints, skin and blood vessels.
Diagnosis is established clinically. Treatment of recurrent polychondritis is carried out by prednisolone, in some cases by immunosuppressants.
Recurrent polychondritis occurs with equal frequency in both men and women; The greatest frequency of the disease is among middle-aged people. Association with RA, systemic vasculitis, SLE and other connective tissue diseases presupposes an autoimmune etiology of the disease.
[Symptoms of recurrent polychondritis
The most common acute pain, erythema and swelling of the cartilage of the auricle. The defeat of nasal cartilage is less often noted, and even more rarely arthritis, ranging from arthralgia to symmetrical and asymmetric non-deforming arthritis, involving large and small joints, with a predominant lesion of bone chondral joints. Next, in order of decreasing frequency, follow: eye damage (conjunctivitis, scleritis, iritis, keratitis, choreoretinitis), cartilage of the larynx, trachea and bronchi (hoarseness, coughing), inner ear, cardiovascular system (aortic regurgitation, pericarditis, myocarditis, aneurysm aorta, aortitis), kidneys, skin. Attacks of acute inflammation take place from several weeks to several months; a few years later, repeated attacks are noted.
Progression of the disease can lead to the destruction of supporting cartilages with the development of sagging ears, saddle-shaped deformation of the nose, funnel-shaped deformation of the chest, visual, auditory and vestibular disorders, stenosis of the trachea. In rare cases, it is possible to develop systemic vasculitis (leukocytoclastic vasculitis or subacute polyarteritis), myelodysplastic syndrome, malignant tumors.
Diagnosis of recurrent polychondritis
Diagnosis is established if the patient has at least three of the following symptoms: bilateral outer ear chondritis, inflammatory polyarthritis, nasal cartilage chondritis, eye inflammation, airway chondrite, auditory or vestibular dysfunction. With the complexity of the diagnosis it is useful to conduct a biopsy involved in the pathological process of cartilage.
Conducting laboratory studies is not necessary, but it can be useful to exclude other diseases. In the synovial fluid, there may be signs of mild inflammation. In the blood, there may be normocyte and normochromic anemia, leukocytosis, an increase in ESR or a concentration of gamma-globulins, sometimes rheumatoid factor, antinuclear antibodies (AHA), in 25% of patients - antineutrophil cytoplasmic antibodies. Impaired renal function may indicate the adherence of vasculitis. The detection of antineutrophilous cytoplasmic antibodies, mainly binding to proteinase-3, presupposes the presence of Wegener's granulomatosis in the patient, which has a similar clinical picture.
Patients, especially with tracheal injury, need constant monitoring to assess the extent of its narrowing with CT.
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Treatment of recurrent polychondritis
The five-year mortality rate for this disease is 30%, the main causes are stenosis of the larynx and trachea, as well as cardiovascular complications (aneurysms of large vessels, damage to the heart valves, systemic vasculitis).
With a mild disease, NSAIDs can be prescribed. Nevertheless, most patients showed oral administration of prednisolone in a dose of 30 to 60 mg once daily, followed by a decrease in dose immediately after clinical improvement. In some cases, it may be necessary to administer long-term glucocorticoid therapy. In such patients, a reduction in the dose of glucocorticoids is possible when combined with methotrexate at a dose of 7.5 to 20 mg orally for a week. Severe cases may also require the use of other immunosuppressants, in particular cyclosporine, cyclophosphamide, azathioprine. However, none of these treatments has been studied in controlled clinical trials and demonstrated no reduction in mortality. With the development of stenosis of the trachea, complicated by a narrow breathing, tracheotomy and stent placement may be required.