AbstractIntroduction and Objective
Idiopathic pulmonary fibrosis (IPF) is a chronic, irreversible fibrotic disease with more than 5000 incident cases annually in UK. IPF related cough is present in 80% cases and is often refractory to current treatments. Cough has a significant impact on quality of life, causing sleep disturbance, difficulties at work and stress incontinence. A previously published prospective open label phase II trial of thalidomide for treatment of pulmonary fibrosis suggested that IPF associated cough responded well to thalidomide. We have been using thalidomideas an “off-license” indication for selected IPF patients. The objective of this study was to review our experience of using Thalidomide as treatment for cough in IPF.Methods
Nine patients were referred to Nottingham Academic Interstitial Lung Disease clinic between 2009 and 2011 for assessment of their cough. A modified version of Leicester Cough Questionnaire was used, in conjunction with subjective symptoms, to clinically assess their cough. A trial of PPI (omeprazole 40 mg) and Prednisolone (10 mg) for 6 weeks was given to all subjects. Two were excluded as did not have significant cough, one patient declined thalidomide after initial screening.Results
Six patients were treated with thalidomide. Four had IPF, one had Hypersensitivity Pneumonitis and one had fibrotic Cryptogenic Organising Pneumonia. 72% were males with mean age 69 years (range 51–88 years). The median pre-thalidomide cough score was 74.5 (IQR 13.25) and post treatment cough score was 51.5 (IQR 49.25). This was statistically significant (p=0.046). Three stopped thalidomide subsequent to rash. Two patients are currently stable with 50 mg once daily, and 1 with 50 mg alternate daily of thalidomide.Conclusion
Our observation of a carefully selected cohort of patients suggests that thalidomide has potential for treatment of IPF associated cough. It works quickly (within days) as reflected both subjectively and objectively via questionnaire, even when prednisolone has failed. However, it does have a significant side-effect profile. Identification of thalidomide's mechanism of action may aid the development of novel, effective anti-tussive therapy for this debilitating aspect of IPF. We will assess this in randomised open label trial comparing thalidomide vs prednisolone for treatment of IPF associated cough.