What is Recurrent Polychondritis?
Recurrent polychondritis (panchondritis, systemic chondromalacia, chronic atrophic polychondritis, etc.) is a generalized progressive inflammatory disease of the cartilage tissue involving the sensory organs (eyes, ears, vestibular apparatus), leading to structural changes in the cartilage until it disappears completely.
Recurrent polychondritis is a rare disease. Some authors usually describe single observations. Recurrent polychondritis most often affects people aged 30-40 years, regardless of gender. This process also occurs in children, adolescents and the elderly.
Pathogenesis during Recurrent Polychondritis
The pathogenesis of recurrent polychondritis is under study.
Histological examination of the affected cartilage reveals areas of metachromasia, chondrocyte degeneration, the introduction of active fibroblasts into the cartilage thickness, dissolution, lysis, and sequestration of the cartilage matrix. Focal or diffuse infiltration of lymphoid and plasma cells is detected in perichondral tissues. In some sections (sclera, aortic arch), there are changes in elastin fibers (degeneration, necrosis), collapse of collagen fibrils, subsequent fibrosing changes, inflammatory process in the walls of blood vessels.
When examined under an electron microscope, some authors found in the cartilaginous tissue of patients with recurrent polychondritis a large number of so-called matrix granules, much higher than their content in healthy cartilage and similar to lysosomes. It is suggested that these lysosomes are apparently released from damaged chondrocytes. The presence of a significant amount of lysosomes and their enzymes in the cartilage is likely to cause degradation of the protein polysaccharide complex of the main substance of the cartilage and cellular elements, followed by destruction and fibrosis of the cartilage tissue.
The release of lysosomes and the activation of their enzymes may be due to the action of a variety of infectious and non-infectious factors. Autoimmune mechanisms, in particular the formation of antibodies to cartilage, may play a role in the pathogenesis of the disease. There are signs of the inclusion of cellular immune reactions in the process: peripheral blood lymphocytes of patients with recurrent polychondritis, unlike healthy individuals, react actively (according to the absorption reactions of labeled thymidine and blast transformation) to healthy cartilage extract.
Symptoms of Recurrent Polychondritis
The most typical signs of recurrent polychondritis are as follows:
- inflammatory changes in the auricles, which are observed in 58% of patients;
- in classical cases, the auricles look reddened, significantly swollen, they are sharply painful when touched, hearing in this period can be significantly reduced due to inflammatory edema of the external auditory canal; in the future, due to the development of fibrotic changes, the auricles are deformed, in 48% of patients obstruction of the auditory passages occurs and hearing is steadily reduced;
- involvement in the pathological process of the cartilaginous septum of the nose (observed in 82% of patients), which is manifested by its saddle deformation; this symptom may appear already at the first attack of the disease, but more often with exacerbations of the process; pain syndrome does not always accompany the development of deformity of the nose;
- fever, which is more often observed during the first attacks of polychondritis (in 81% of patients);
- articular syndrome (in 71-78% of patients) in the form of arthralgia, transient polyarthritis or polyarthritis resembling rheumatoid, with erosive changes in radiographs in case of chronic joint inflammation;
- damage to the cartilage of the larynx and trachea (in 70% of patients) is manifested by their pain on palpation; in severe cases, due to edema in this area, difficulty breathing may occur, which sometimes requires a tracheotomy; can be involved in the process and bronchi; collapse of small bronchi develops, which leads to activation of an infection of the respiratory tract, and sometimes to asphyxiation;
- eye damage (in 60% of patients) – episcleritis, conjunctivitis, iritis.
Less commonly affected are costal cartilages, the vestibular apparatus, the aortic arch and valves, with the development of the aortic insufficiency clinic.
Laboratory changes are nonspecific: usually an increase in ESR, slight anemia, a decrease in the level of albumin, an increase in the content of a and yglobulins are usually detected.
The course and forecast
The disease usually begins acutely, can take a chronic course with periodic exacerbations lasting from several days to months. With each exacerbation, more and more cartilaginous structures are involved in the process and changes in the sensory organs are revealed. The prognosis for recovery is unfavorable. The duration of the disease varies from several months to 20 years. Death occurs from respiratory or cardiovascular failure.
Treatment for Recurrent Polychondritis
Usually, prednisone is used at a dose of 20-60 mg / day, followed by a decrease in the dose to the maintenance dose (5-10 mg / day), on which the patient is on a long time to prevent recurrent severe exacerbations. GCS treatment can be combined with non-hormonal anti-inflammatory drugs and analgesics (indomethacin, voltaren, ram gin, etc.).
The question of the advisability of using immunosuppressants for polychondritis is still unclear.