Experience of hydroxychloroquine in the treatment of pulmonary sarcoidosis in the west of Ireland: An insight into clinical practice

Introduction: Hydroxychloroquine is widely used in treatment of cutaneous sarcoidosis. There is limited literature regarding the experience of hydroxychloroquine in pulmonary sarcoidosis. Aim: To review the treatment and toxicity profile of hydroxychloroquine in the treatment of pulmonary sarcoidosis. Methods: Retrospective observational cohort study of sarcoidosis patients in the West of Ireland from 1983-2009. Remission was defined as successful withdrawal from steroids within one year, improvement in radiological staging of pulmonary disease (Scadding classification) and maintenance of pulmonary function. Relapse was defined as deterioration necessitating re-introduction of steroids. Results: 92/407 (22.6%) were treated with hydroxychloroquine 200mg BD. Mean age 38.1yrs (range 17-68) with M:F ratio 1:1. Mean length of treatment 2.9yrs. Mean follow-up 8.5yrs. 19.6%, 47.8%, 25% and 7.6% had stage 1-4 disease respectively. 95.6% received high-dose steroids prior to treatment. Indications for treatment were: steroid-sparing 17.4%, refractory 18.5%, extra-pulmonary 47.8% and maintenance of remission 16.3%. 52.2% were steroid-free within one year; 23.9% showed improvement in radiological staging (normal radiology in 13%); there was no significant difference in lung function. Relapse rate was 14.1%. Adverse event rate was 11.9% (4.3% discontinued treatment). Conclusion: This study shows hydroxychloroquine is safe and effective in the treatment of pulmonary sarcoidosis, particularly multisystem disease. We conclude, therefore, that hydroxychloroquine should be considered in the treatment and maintenance of patients with chronic pulmonary sarcoidosis.