Given the small number of patients, no controlled trials were performed in the PCA. The treatment is adapted, individually, depending on the activity and the severity of the clinical form.Corticosteroid therapy remains the basis of treatment and most patients require long-term treatment. It usually allows the regression of chondrites and articular, ocular and cutaneous manifestations.
After an attack dose, it is reduced to the lowest dosage possible to try to control the disease. In potentially severe forms (severe laryngeal or tracheobronchial chondritis, sudden onset hearing loss, systemic vasculitis with gravity factor), it is initially administered as one to three infusions with oral corticosteroid high dose. The decay of corticosteroids is then progressive.
Dapsone and colchicine have been suggested for the treatment of chondritis and minor skin manifestations.
Immunosuppressants are used immediately in severe respiratory or vascular forms or secondarily in case of corticoresistance or corticodependence. Their use should be cautious because of the risk of association with myeloid hemopathy and the lack of controlled studies. Methotrexate, at 0.3mg / kg weekly, is often effective. Cyclophosphamide, azathioprine, but also mycophenolate mofetil, ciclosporin, leflunomide, chlorambucil were tested with inconsistent results.
Biomedicines (anti TNF alpha, abatacept, rituximab) have been tried in a small number of cases with variable results that do not allow to decide on a possible effectiveness.
Local treatments are often necessary: corticosteroid (articular, ocular, inhalation) administration, tracheotomy, dilation or tracheobronchial stent, nasal plastic surgery. Respiratory physiotherapy helps drain bronchial secretions.
The PCA is monitored in internal medicine departments specializing in the management of autoimmune diseases. The site orphanet recence the specialized services and the tests in progress. It is very important to report that you have this condition if anesthesia has to be performed because the damage to the respiratory system can complicate intubation.
PCA is not part of the list of ALD (Long Term Conditions) because it is exceptional). But care in ALD off list is to be done by the doctor.You want to react, to give your testimony or to ask a question? Appointment in our thematic FORUMS or A doctor answers you !
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