Interstitial Lung Disease in Myositis

The clinical, radiographic, and histopathologic features of ILD in myositis are similar to idiopathic ILD. Patients with a known diagnosis of inflammatory myopathy require a prompt clinical evaluation and the assessment of myositis-associated autoantibodies. Patients possessing autoantibodies associated with ILD or those with any pulmonary symptoms should have pulmonary functions test and high resolution CT (HRCT) scanning of their lungs. Despite the lack of placebocontrolled trials, systemic glucocorticoids are considered the mainstay of initial treatment of myositisassociated ILD. Glucocorticoid-sparing agents are often started concomitantly with glucocorticoids, particularly in patients with severe disease. The first-line conventional immunosuppressive drugs include either mycophenolate mofetil or azathioprine. If these agents fail or if the features are more severe or rapidly progressive, then more aggressive immunosuppressive or immunomodulatory therapy including cyclophosphamide, tacrolimus or cyclosporine, or rituximab should be considered. Further investigations are required to assess the role of novel therapies in the treatment of myositis-associated ILD.