Abstract
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with poor prognosis and treatment yet ineffective. Mycophenolate Mofetil (MMF) represents an immunomodulatory agent, acts by inhibiting lymphocyte proliferation and is commonly used to prevent rejection following organ transplantation. Objective: We sought to determine the safety and efficacy profile of MMF in IPF patients. Methods: We retrospectively identified ten patients, all males, who met the ATS/ERS 2000 criteria for IPF and received MMF 1,44gr/day for 12 months. All of them had routine laboratory, pulmonary function and radiological (high resolution computed tomography-HRCT) data available and therefore were enrolled in the study. Forced vital capacity (FVC), total lung capacity (TLC), diffusion capacity of the lung for carbon monoxide (DLCO), 6-minute walking distance (6MWD), alveolar-arterial gradient of oxygen tension (PA-aO2), HRCT scans and routine laboratory data at treatment onset were compared with respective values 12 months after treatment onset. Results: There were no significant alterations in FVC, TLC, DLCO, 6MWD and PA-aO2 pre- and 6 and 12 months post-treatment. HRCT evaluation showed a moderate deterioration of the total extent of disease (p=0.002). No cases of clinically significant infection, leucopenia, or elevated liver enzymes were recorded. Conclusions: The above data suggest that MMF is a safe therapeutic modality which resulted in overall stable disease regarding functional status while it demonstrated a moderate progression as assessed by radiological parameters in a small cohort of IPF patients. Larger, prospective studies are sorely needed.
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