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

Heba M Bintalib; David M Lowe; Gaia Mancuso; Georgia Gkrepi; Suranjith L Seneviratne; Siobhan O Burns; John R Hurst

doi:10.1183/23120541.00024-2022

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

ERJ Open Res. 2022 Oct 17;8(4):00024-2022. doi: 10.1183/23120541.00024-2022. eCollection 2022 Oct.

Authors

Heba M Bintalib^{1

2

3}, David M Lowe^{4

5}, Gaia Mancuso⁶, Georgia Gkrepi⁷, Suranjith L Seneviratne⁵, Siobhan O Burns^{4

5}, John R Hurst¹

Affiliations

¹ UCL Respiratory, University College London, London, UK.
² Department of Respiratory Care, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
³ King Abdullah International Medical Research Center, Jeddah, Saudi Arabia.
⁴ Institute of Immunity and Transplantation, University College London, London, UK.
⁵ Department of Immunology, Royal Free London NHS Foundation Trust, London, UK.
⁶ Unit of Immunology, Rheumatology, Allergy, and Rare Diseases (UnIRAR), Vita-Salute San Raffaele University, Milan, Italy.
⁷ Respiratory Medicine Department, University Hospital of Ioannina, Ioannina, Greece.

Abstract

Aim: The aim of the study was to evaluate the response in lung function to different treatment regimens for common variable immunodeficiency patients with granulomatous lymphocytic interstitial lung disease (GLILD).

Method: A longitudinal retrospective cohort study was carried out. Patients were divided into three groups. To assess the response to different treatments, we compared baseline lung function with post-treatment tests.

Results: 14 patients with GLILD were included, seven of whom were treated with acute corticosteroids for a mean duration of 132±65 days. Spirometry results were unchanged, but there was a significant improvement in diffusing capacity of the lung for carbon monoxide (D _LCO)% and transfer coefficient of the lung for carbon monoxide (K _CO)% (median change in D _LCO%=7%, p=0.04, and K _CO%=13%, p=0.02). Relapse occurred in three out of seven patients. Five patients were treated with long-term mycophenolate mofetil (MMF) with/without corticosteroids for a mean duration of 1277±917 days. No changes were found in spirometry; however, there was a significant increase in D _LCO% and K _CO% (median change in each of D _LCO% and K _CO%=10%, p=0.04). Four patients on steroids with MMF successfully weaned the prednisone dose over 12 months. Four patients never received immunosuppression therapy. A significant decline was found in their lung function assessed over 7.5 years. The median reduction in the forced vital capacity (FVC)%, forced expiratory volume in 1 s (FEV₁)% and D _LCO% was 15%, 7% and 15%, equivalent to 2%, 1% and 2% per year, respectively.

Conclusion: Corticosteroids improve gas transfer in GLILD, 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.