Relapsing polychondritis (Polychondritis recidivans) is a rare inflammatory disease of unknown etiology and sudden onset. It affects the cartilage of the ears, nose, larynx, trachea and bronchi. What are the symptoms of recurrent cartilage inflammation and how is it treated?

Recurrent cartilage inflammation( Polychondritis recidivans, relapsing polychondritis ) has a characteristic, very variable course, with periods of exacerbations lasting several days up to several weeks, followed by periods of remission. The disease affects the cartilage of the ears, nose, larynx, trachea and bronchi. The peak incidence occurs in the fourth and fifth decade of life, but it can also develop in children and the elderly. It occurs in humans all over the world, with the same frequency in both sexes. Immunological mechanisms play an important role in the pathogenesis of recurrent cartilage inflammation. Deposits of immunoglobulins and complement can be found at the site of inflammation, and antibodies to type II collagen and matrilin I and the presence of immune complexes in the serum of some patients. The process of cartilage destruction begins from its outer surface and progresses deeply. In these places, her sinus damage and loss of chondrocytes occur. Damaged cartilage is replaced with granulation tissue, which then undergoes fibrosis and focal calcification. There may also be small foci of cartilage regeneration.

Recurrent cartilage inflammation: symptoms

The onset is sudden and involves cartilage in one or two places. Interestingly, general symptoms such as fever, fatigue and weight loss may precede organ changes by several weeks. The first symptom of recurrent cartilage inflammation is unilateral or bilateral inflammation of the cartilage of the ears. Patients complain of symptoms such as sudden pain, tenderness and swelling in the cartilaginous part of the ear. The skin at the site of the lesions is bright red or violet. Prolonged or recurring exacerbations of the disease due to the destruction of the cartilage lead to the sagging and drooping of the auricles. Additionally, the resulting swelling may obstruct the Eustachian tube or the external auditory canal, contributing to hearing impairment.Nasal cartilage may appear during the first flare of the disease or during subsequent exacerbations. Symptoms such as stuffy nose, runny nose and nosebleed are characteristic. The bridge of the nose is red, swollen and tender, and its collapse can lead to a saddle nose. Arthritis is most often asymmetrical, sparse and polyarticular, and affects both large and small peripheral joints. The relapse of inflammation lasts from a few days to several weeks and is self-limiting. The examination of the affected joints shows their excessive heat, painful palpation and swelling. It is also possible to involve the costal cartilages, upper sternal joints and sternoclavicular joints. Then a funnel-shaped, and even flail-like chest is formed.

In the eyes, there may be inflammations of the conjunctiva, epidural, sclera, iris and cornea. Due to the risk of developing blindness, ulceration and perforation of the cornea are particularly dangerous. Other common symptoms include eyelid edema and periorbital edema, exophthalmia, cataracts, optic neuritis, paralysis of the external eye muscles, retinal vasculitis, and retinal vein thrombosis. Hoarseness, coughing without expectoration of secretions, and tenderness in the projection of the larynx and proximal part of the trachea. Swelling of the mucosa, narrowing and / or collapse of the laryngeal and tracheal cartilages can lead to the onset of stridor and life-threatening airway obstruction requiring tracheostomy. In addition, collapsing bronchial cartilages promote the development of pneumonia, and with extensive involvement of the bronchial tree, it leads to respiratory failure. Aortic valve insufficiency occurs in approximately 5% of patients and results from progressive dilatation of the valve annulus or destruction of its leaflets. Other cardiac symptoms include pericarditis, myocarditis, and conduction disturbances. Aortic arch, thoracic and abdominal aortic aneurysms may coexist. Recurrent cartilage inflammation may be accompanied by systemic vasculitis, which may appear as leukocytoclastic vasculitis, polyarteritis nodosa or Takayasu's disease. Against their background, neurological disorders may develop in the form of epileptic seizures, strokes, ataxia and neuropathy of the cranial and peripheral nerves. Skin lesions are not so characteristic of recurrent cartilage inflammation, but if they occur, they take the form of purpura, erythema nodosum or multiforme, angioedema. hivesreticular cyanosis and adipose tissue inflammation Approximately 30% of patients with recurrent cartilage inflammation are diagnosed with other rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome or ankylosing spondylitis.

Other conditions associated with recurrent cartilage inflammation include inflammatory bowel disease, primary biliary cirrhosis, and myelodysplastic syndromes.

Recurrent cartilage inflammation: diagnosis

Currently, the McAdam criteria or the modified Damiani and Levine criteria are used to diagnose recurrent cartilage inflammation.

The criteria proposed by McAdam include:

  • recurrent cartilage inflammation of both auricles
  • non-erosive arthritis
  • nasal cartilage inflammation
  • inflammation of the eyeball structures (conjunctiva, cornea, sclera or sclera and / or uve)
  • laryngitis and / or tracheitis
  • damage to the cochlea and / or vestibular organ, manifested by neurosensory hearing impairment, tinnitus and / or dizziness

The diagnosis is certain if at least three of the above-mentioned symptoms are present, with a positive result from a cartilage biopsy of the ear, nose or respiratory tract. According to the modified Damiani and Levine criteria, the diagnosis can be established when one or two of the the above-mentioned symptoms and a positive biopsy result, or when a reduction in cartilage inflammation was obtained in at least two locations after the use of glucocorticosteroids or dapsone, or in the presence of at least three of the above-mentioned symptoms. It is important that in patients with a clear clinical picture, biopsy is usually not necessary In terms of laboratory test results, patients often have moderate leukocytosis, normocytic and normochromic anemia, and elevated ESR and C-reactive protein levels.

Some people can also detect circulating immune complexes, increased levels of gamma globulin and antibodies to the cytoplasm of neutrophils c-ANCA and p-ANCA. Many diagnostic methods are used to diagnose recurrent cartilage inflammation, for example:

  • the involvement of the airways can be demonstrated by performing computed tomography and bronchoscopy
  • MRI is especially useful in imaging the larynx and trachea
  • bronchography is performed to look for bronchial strictures
  • spirometry can detect a narrowing of the airways insidechest
  • chest X-ray shows narrowing of the trachea and / or main bronchi, aneurysm dilatation of the ascending or descending aorta, and enlargement of the cardiac figure in aortic regurgitation
  • X-rays can also show calcifications resulting from the destruction of the cartilage of the ears, nose, larynx and trachea

Recurrent cartilage inflammation: treatment

Prednisone in the doses of 40-60 mg daily is used in patients with active cartilage inflammation. Provided that disease activity is properly controlled, the dose of the drug is reduced and, in some cases, even complete drug withdrawal is possible. In the case of chronic use, 10-15 mg a day is taken to control the symptoms of the disease. Dapson can be used instead of prednisone.

Immunosuppressive drugs - methotrexate, cyclophosphamide, azathioprine and cyclosporine, are used when you do not respond to prednisone treatment or if you need to use high doses of prednisone to control disease activity.

In case of severe eye symptoms, intraocular administration of glucocorticosteroids and the use of high doses of prednisone may be necessary.

In patients with aortic valve involvement, valve replacement is performed, and in the case of aortic aneurysm - arterial repair is performed. In patients with symptoms of severe airway obstruction, a tracheostomy is necessary, and in the case of collapsed cartilages of the trachea and bronchi - implantation of stents.

Category:

Immune system