Corticosteroid-induced remission, and mycophenolate maintenance therapy in GLILD: long-term, longitudinal change in lung function in a single centre cohort

Abstract

<b>Introduction:</b> Granulomatous lymphocytic interstitial lung disease (GLILD) is rare non-infectious complication in patients with common variable immunodeficiency disorders which significantly impacts morbidity and mortality. <b>Aim:</b> To evaluate the response of lung function to different treatment regimens. <b>Method:</b> This is a longitudinal retrospective cohort study. To assess the response to different treatments, we compared baseline lung function to post-treatment. <b>Results:</b> 14 patients with GLILD were included.7 patients were treated with acute corticosteroids for a mean duration of 4 months. The spirometry results were unchanged, but there was a significant improvement in DLCO% and KCO%(median change in DLCO%=5%, P=0.04, and KCO%=12%, P=0.02). Relapse occurred in 5/7 patients.5 patients were treated with long-term MMF&nbsp;+/−&nbsp;corticosteroids for a mean duration of 3.8 years.2 had relapsed after multiple courses of acute steroids. No changes were found in spirometry. However, there was a significant increase in DLCO% and KCO%(mean change in DLCO%=10%, P=0.04, and KCO%=11%, P=0.04).4 patients on steroids with MMF successfully weaned the Prednisone dose over 12 months. 4 patients had never received immunosuppression therapy. A significant decline was found in lung function over 7.8 years. The median reduction in the FVC%, FEV1%, and DLCO% were 20%, 10%, and 11%, respectively. <b>Conclusion:</b> Corticosteroids improve gas transfer, but patients often relapse. The use of MMF was associated with long-term effectiveness in GLILD and permits weaning of corticosteroids. A delay in initiating and continuing maintenance treatment could lead to disease progression.