SESSION TITLE: Interstitial Lung Disease
SESSION TYPE: Original Investigation Slide
PRESENTED ON: Wednesday, October 30, 2013 at 02:45 PM - 04:15 PM
PURPOSE: Our objective was to evaluate the safety and efficacy of the addition of tacrolimus to conventional immunosuppressive therapy in patients with progressive connective tissue disease-related interstitial lung disease (CTD-ILD).
METHODS: We included patients from our ILD database with progressive CTD-ILD despite treatment with prednisone and an additional immunosuppressive agent. Demographics, clinical features, lung function, radiographic images, pathologic findings and adverse advents were reviewed. Response to therapy was assessed by percent change in forced vital capacity (FVC) and difference in six-minute walk test (6MWT) distance before tacrolimus treatment as compared to the best lung function during treatment.
RESULTS: Eleven patients were included; three had undifferentiated connective tissue disease and eight had an idiopathic inflammatory myopathy. Pathologic and/or radiographic findings showed organizing pneumonia and/or nonspecific interstitial pneumonia in nine patients and usual interstitial pneumonia in two patients. Tacrolimus was added as an adjunctive agent to standard immunosuppressive therapy in doses of 1 mg BID to 6 mg BID, targeting a serum trough level of 5-10 ng/mL. Length of tacrolimus treatment ranged from 2 to 98 months (mean 34.8 months ± 30.0, median 29 months). The mean of the percent change in FVC was 8.36% ± 10.69 with a median improvement of 6%. Eight of eleven patients had an improvement in FVC ranging from 3 to 32%; the remaining three patients experienced a decrease in FVC by 1 to 3%. Eight of eleven patients performed a 6MWT pre and post-tacrolimus treatment. Five of eight patients had an improvement in their 6MWT distance while three walked a shorter distance (mean change in distance was 52.3 meters ± 119.5, median improvement of 30.2 meters). No patient had a life-threatening adverse event. Most common side effects included hypertension, hyperglycemia and pulmonary infections. No patient had a sustained elevation in creatinine.
CONCLUSIONS: This case series demonstrates that tacrolimus can be efficacious and is well tolerated as an adjunctive therapy to corticosteroids and an additional immunosuppressive agent.
CLINICAL IMPLICATIONS: Tacrolimus should be considered in patients with progressive CTD-ILD.
DISCLOSURE: The following authors have nothing to disclose: Leah Witt, Carley Demchuk, James Curran, Mary Strek
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