Objective assessment of severity of cough and gastro-oesophageal reflux disease in patients with Idiopathic Pulmonary Fibrosis and efficacy of pulsed cyclophosphamide and methylprednisolone therapy in patients with progressive interstitial lung disease

Abstract

Cough is a disabling symptom in patients with Idiopathic pulmonary fibrosis (IPF). Reflux disease is frequently associated with IPF and implicated in pathogenesis of both IPF and cough. Therefore, acid suppression by omeprazole should ameliorate cough and improve quality of life in IPF patients, which is the hypothesis underlying the planned PPIPF study. My thesis is based on the baseline assessments of cough and reflux in IPF patients recruited for the study. 45 patients with mean age of 71.2 years; 35 (77.8%) male were recruited. 24-hour cough recording at baseline showed significantly raised cough frequency (mean 11.99/hour) with impaired quality of life (mean LCQ-total score 15.22). Reflux related health questionnaires [RSI (mean score 15.6,) and GIQLI (mean score 105.56,)] suggested impaired quality of life, possibly due to reflux disease in our study cohort [but not DeMRQ (mean score 1.16)]. Consent for GI studies and bronchoscopy was low. GI studies demonstrated oesophageal dysmotility in 4 (44%) and acid reflux in 6 (67%) out of the 9 IPF patients, who completed the assessment. BAL samples showed leucocytosis in all 8 participants with bacterial growth on cultures in 2 participants. Additionally, I undertook a case-series study to assess the outcome of pulsed cyclophosphamide and methyl-prednisolone therapy in patients with progressive interstitial lung disease (ILD) in our institute. Medical records of 53 patients with mean age of 60 years; 29 (55%) male were reviewed. The median number of cyclophosphamide pulses received was 6. The average rate of change of lung function was significantly less after cyclophosphamide therapy both for FVC (p=0.0004) and TLco (p=0.00015). In our single centre, retrospective study pulsed cyclophosphamide and methyl-prednisolone was associated with stabilisation of lung function in a mixed cohort of patients with progressive ILD. Adverse events were common but transient and managed with dose reduction and/or delayed schedule.