There are no placebo-controlled trials of therapy for SLE-related ILD; however, in our opinion, response rates are likely dictated by the underlying pathologic pattern. In general, glucocorticoids have been used with modest success. In one small, open-label study,35 prednisone (60 mg/d for at least 4 weeks) was administered to 14 subjects; pulmonary symptoms resolved entirely in 3 subjects, improved in 6 subjects, were unchanged in 1 subject, and worsened in 4 subjects.35 Immunomodulatory agents may hold additional promise.40 In subjects with SLE and clinically significant chronic ILD (eg, NSIP, usual interstitial pneumonia, or LIP pattern), our group generally uses an initial combination of oral glucocorticoids and an immunomodulatory agent, with the goal of weaning to low-dose corticosteroids, or an immunomodulatory agent alone. We use cyclophosphamide as initial therapy for the most severe and rapidly progressive disease and transition to an alternative agent (most often mycophenolate mofetil or azathioprine) after 12 to 18 months. For less severe cases, or when cyclophosphamide is not tolerated, we have found mycophenolate mofetil or azathioprine to be useful initial agents. We have also had anecdotal success with rituximab, especially in the setting of LIP or when glucocorticoids cannot be lowered because of antibody-mediated extrathoracic manifestations (eg, thrombocytopenia or hemolytic anemia).