[Polychondritis relapsans--a case report].

BACKGROUND

The relapsing polychondritis (RP) rarely occurs and it's classified to connective tissue diseases. Woman from the age of 35 to 45 suffer most often from this types of collagenosis. Typical features of all collagenosis are the connective tissue inflammation and the presence of autoantibodies in the patient's peripheral blood. The RP disease relies on general polychondritis with gradually cicatrization and fibromatosis of different cartilages of the body. Inflammatory process leads to destruction of the collagen type A by autoantibodies. The main symptom of RP is the inflammation of the auricle, nose, nasal septum, laryngeal, tracheal and bronchial cartilages, which causes deformations of these organs. The polyarthritis without a distortion accompanies to cartilages changes. The heart and blood vessels connective tissue changes have been hardly described but they can lead to develop acquired valvular disease and aneurysms in large blood vessels. An increase of the erythrocyte sedimentation rete, the anemia and the leucocytosis have been observed. The auricle ache, edema and hyperemia of the auricle and general polyarthritis are often the beginning of the RP disease. The external and intranasal polychondritis with later nose deformations are the next symptoms of that disease. Changes in the nasal and auricular cartilages are sometimes single sings of RP. However the inflammatory process can develop in laryngeal and tracheal cartilages producing the respiratory insufficiency and it leads to the death of 25% patients. In the treatment of RP anti-inflammatory drugs are used in the first period of disease. Afterwards patients are treated with steroids, metothrexate and cyclophosphamide. The latest anticytokinne drug are applied by RP treatment.

METHODS

A 46 years old woman was admitted to Otolaryngology Department with edema chorda vocalis, dysphonia and dyspnoe. After operation she had respiratory insufficiency. This woman was admitted to Otolaryngology Department many times with dyspnea, which was treating with steroids and terminally with tracheotomy.

RESULTS

Histology of the tracheal cartilages showed the PR. From this time the woman was pharmacology treating in another department of rheumatology.

CONCLUSIONS

The RP is rarely occurs disease, who is very difficult to diagnosis end treatment, and many times leads to death.