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Anti-synthetase syndrome treatment
Diagnosis is undertaken via a comprehensive history and physical examination focused on CTD signs and symptoms that include Raynaud’s phenomenon, mechanic’s hands, unexplained fever, myositis, and non-erosive arthritis. HRCT imaging along with pulmonary function testing is crucial in screening for and characterizing the extent of pulmonary involvement. Muscle and lung biopsies are infrequently required for diagnosis but can aid in understanding severity of disease and the likelihood of response to therapy. Immunosuppressive agents, such as azathioprine, mycophenolate mofetil, and/or tacrolimus, are usually required in addition to corticosteroids for management of the myositis and pulmonary manifestations of the anti-synthetase syndrome. No study has demonstrated the superiority of a particular agent, so the choice of therapy is guided by practitioner comfort and a careful assessment of potential risks of therapy in an individual patient. Our practice is to initiate prednisone and azathioprine or mycophenolate mofetil in patients with anti-synthetase syndrome-related ILD, with the addition of tacrolimus for rapid disease control in more severe ILD. In the setting of the anti-synthetase syndrome, we use rituximab for patients with severe progressive and/or refractory ILD and have used cyclophosphamide in ARDS.
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