Usefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases

Abstract

Background: Interstitial lung diseases (ILDs) are a group of disorders characterised by progressive lung function decline. Stabilisation of lung function under intermittent i.v. cyclophosphamide was shown in patients suffering from systemic sclerosis, yet data in ILD patients are scarce. Objectives: To retrospectively evaluate the usefulness of cyclophosphamide pulse therapy in ILD. Methods: We retrospectively analysed all patients who received i.v. cyclophosphamide in our centre from 2002 to 2012. Lung function, survival status, and bronchoalveolar lavage cytology were recorded during a follow-up period of 18 months. Results: Twenty-six patients with idiopathic pulmonary fibrosis, 6 with lymphocytic interstitial pneumonia (LIP), 8 with idiopathic non-specific interstitial pneumonia (NSIP), 7 with rheumatoid arthritis-associated ILD, and 7 with perinuclear anti-neutrophil cytoplasmic antibody-positive ILD (pANCA+ ILD) were included. Patients with LIP and NSIP had the best survival outcome, those with pANCA+ ILD the worst. In the total cohort, we found a significantly higher total lung capacity decline in the year before treatment compared to the year after treatment. Conclusions: This retrospective analysis of cyclophosphamide treatment shows a stabilisation of lung function in most patients with fibrotic ILDs, yet prospective studies in clearly defined diagnoses are urgently needed.