How is recurrent polychondritis treated?

I. Diagnostic Points Based on the typical clinical manifestations and laboratory tests in considering the possibility of RPC, the diagnostic criteria of McAdam in 1975 can be followed: 1. Bilateral otitis cartilaginous; 2. Non-erosive polyarthritis; 3. Nasal chondritis; 4. Ophthalmitis including conjunctivitis, keratitis, sclera, superficial scleritis, and uveitis, etc.; 5. Laryngeal and/or tracheal cartilage inflammation; 6. Cochlear and/or vestibular damage as evidenced by hearing loss, tinnitus and vertigo. The diagnosis can be confirmed if 3 or more of the above criteria are present, and no histologic confirmation is required. Fewer than 3 criteria require a cartilage biopsy to confirm the diagnosis. When the lesion extensively involves the cartilage of the ear, nose, throat and trachea, it should be differentiated from many other diseases with similar clinical manifestations: 1. Auricular lesions and otitis externa: it should be differentiated from localized trauma, frostbite, erysipelas, chronic infection, systemic lupus erythematosus, gout, mycobacterial diseases, syphilis and leprosy. 2. Systemic vasculitis or other connective tissue disease can also cause chondritis, but its bilateral chondritis is rare. 2.Nasal chondritis: it needs to be differentiated from Wegener’s granuloma, lymphoid granuloma, lethal midline granuloma, congenital syphilis, leprosy, lymphoma, tuberculosis and other granulomas, as well as carcinoma and lymphosarcoma. Repeated biopsy, culture of pathogenic bacteria and serologic examination can help to identify. 3.Ophthalmia: It should be distinguished from Wegener’s granulomatosis, polyarteritis nodosa, Cogan’s syndrome, leukosis, primary or secondary dry syndrome, seronegative spondyloarthropathies, and other systemic diseases involving the eye. According to the systemic manifestations of these diseases and laboratory examination characteristics is not difficult to distinguish from them. 4, tracheobronchial stenosis deformation: should be differentiated from infectious diseases, tuberculosis, non-infectious granulomatous diseases, tumors, chronic obstructive pulmonary disease, amyloidosis and other diseases, generally the above diseases are clearly diagnosed by biopsy. rpc patients with cartilage lesions of the ear, nose and other cartilage lesions, which can be used to differentiate from them. 5, aortitis and aortopathy lesions: should be differentiated from syphilis, Marfan syndrome, Ehlers-Danlos syndrome, idiopathic mediastinal cyst necrosis, seronegative spondyloarthropathies complicated by aortopathy. 6, costochondritis lesions: must be differentiated from benign thoracic syndromes, the latter such as idiopathic, traumatic costochondritis, Tietze’s syndrome, costothoracic chondritis, chondromalacia of the raphe, etc., the above diseases have no systematic clinical manifestations, which can be used to differentiate from RPC. Treatment program and principles 1, general treatment Acute episodes should be bed rest, depending on the condition of the liquid or semi-fluid diet, so as not to cause epiglottis and laryngeal pain. Pay attention to keep the respiratory tract open, prevent asphyxia. For those who are agitated, sedatives can be used appropriately. Let the patient keep enough sleep. 2, drug therapy (1) non-steroidal anti-inflammatory drugs available anti-inflammatory pain 2.5mg / d, or diclofenac sodium 75mg-150mg / d, depending on the dosage form of three times a day or once orally; ibuprofen 0.6g three or four times a day orally, or choose other non-steroidal anti-inflammatory drugs. (2) glucocorticosteroids Glucocorticosteroids can inhibit the acute attacks of lesions, reduce the frequency and severity of relapses, used for more serious patients, the starting dose is: prednisone 30mg-60mg / d, divided or morning oral once. In severe acute attacks such as larynx, trachea and bronchial tubes, eyes, inner ear is involved in time, the dose of prednisone can be increased, or even methylprednisolone shock treatment. After the clinical symptoms improve, prednisone can be gradually reduced. The dose of 15mg/d or less can be maintained for 1-2 years. (3) Immunosuppressant Cyclophosphamide 400mg intravenously once a week, or 200mg intravenously every other day, and the dose is reduced after the condition is stabilized. Methotrexate 10mg-30mg once a week orally or intravenously. Immunosuppressive agents such as azathioprine can also be used orally. When using immunosuppressants, blood and urine routine, liver and kidney function should be checked regularly to prevent adverse reactions (see the chapter on rheumatoid arthritis and others). (4) Amphetamine sulfone Amphetamine sulfone inhibits complement activation and lymphocyte transformation in humans, and also inhibits lysozyme-involved cartilage degeneration. The average dose of aminophenyl sulfone is 75mg/d, the dose range is 25mg-200mg/d. It is tried in small doses at the beginning and gradually increased later, and its efficacy has not been confirmed. Because of the accumulation effect, the drug needs to be discontinued for 1 day after 6 days of taking the drug, and lasts for about 6 months. The main side effects of Aminosulfone are nausea, drowsiness, hemolytic anemia, drug-induced hepatitis, and leukopenia. Third, symptomatic treatment 1, eye symptoms local use of prednisone ophthalmic ointment, or with hydrocortisone eye drops. Pay attention to prevent secondary infection. When secondary cataract or glaucoma occurs, targeted treatment can be given. 2, on the tracheal cartilage collapse caused by severe dyspnea patients, should be immediately performed tracheotomy, if necessary, with artificial ventilator assisted ventilation, in order to obtain further drug treatment opportunities. Surgical resection of limited tracheal stenosis due to chondritis has been reported. Lung inflammation should be actively prevented and treated, and effective antibiotics should be used once lung infection occurs. 3, RPC patients with refractory cardiac insufficiency due to heart valve disease, should use cardiac tonic and drugs to reduce the heart load. Valve repair or valvuloplasty is feasible if available. Successful aortic aneurysm resection has also been reported. Prognosis: The mortality rate is close to 1/3 in 5 years, and the patients usually die of laryngeal and tracheal cartilage support structure collapse, or cardiovascular lesions (aneurysm of large arteries, cardiac valve insufficiency) or systemic vasculitis. To minimize mortality and improve prognosis, early diagnosis and prompt treatment are required.