Local experience of patients with connective tissue associated interstitial lung disease who were treated with cyclophosphamide.

Abstract

Introduction: Interstitial lung disease (ILD) is a major cause of death amongst individuals with connective tissue diseases (CTD). Although there is no cure for CTD-ILD the need to retard disease progression is vital, hence early detection and treatment is necessary. Cyclophosphamide (CYC) is a potent immunosuppressant that has efficacy in inducing and maintaining remission in autoimmune diseases. Objectives: The purpose of this study was to assess the clinical, radiological and pulmonary function responses of patients who received intravenous CYC for CTD-ILD at Inkosi Albert Luthuli Central Hospital (IALCH) in Durban, Kwa-Zulu Natal, South Africa over a ten year period from January 2009 to December 2018. Methodology: This was a retrospective electronic chart review conducted at IALCH, the main quaternary public sector hospital in the province of Kwa-Zulu Natal, South Africa. Patients 18 years and older with CTD-ILD treated with CYC were included. Patients were given CYC every 2 weeks for 9 months with a total of 18 doses. Demographic and clinical data, as well as data from special investigations, were captured from medical records. Treatment outcomes were assessed using symptoms, pulmonary function and HRCT changes. Results: There were 62 subjects, 88.7% being female with the majority between the ages of 40-59 years old (64.5%). Approximately 50% were black Africans followed by ethnic Indians at 43.5% and then Whites at 6.5%. Most patients had Systemic Sclerosis, followed by Mixed Connective Tissue disease and then Systemic Lupus Erythematosus. There was no significant difference in pre and post treatment, symptoms, lung function and HRCT in those who were treated with CYC. Conclusion: In our setting, the use of 18 doses of CYC every 2 weeks, did not have a significant impact on disease progression.