26th July 2019, Volume 132 Number 1499

James Fulforth, Donna Thomson, Gordon Maxwell, Rachel Wiseman, Adrienne Edwards

Idiopathic pulmonary fibrosis (IPF) is an incurable progressive lung disease characterised by cough and exertional dyspnoea. Although disease course varies, the majority develop progressive respiratory failure, culminating in death within…

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Summary

Idiopathic pulmonary fibrosis is a condition of unknown cause which results in progressive reduction in lung function causing breathlessness and often resulting in death. Historically, treatments have been ineffective, but newer agents have shown some promise. This paper highlights the estimated number of patients living with this condition and highlights ways in which resources could be better utilised to help them. Hopefully this may result in better overall care for patients living with this condition.

Abstract

Aim

In light of new therapies and guidelines for the management of idiopathic pulmonary fibrosis (IPF), and in the absence of local epidemiological data, we sought to ascertain a current estimate of the prevalence of IPF in Canterbury and to audit local practices.

Method

We performed a retrospective observational study of patients with IPF in Canterbury, New Zealand and the wider region. Patients were identified through a systematic search of hospital records and included if they were alive on 1 January 2017, had a histological or radiological diagnosis of usual interstitial pneumonia and clinical correlation consistent with a diagnosis of IPF. Clinical data was extracted from the clinical record. Follow up was complete until April 2018.

Results

Sixty-eight patients were included, median follow up 33 (14–49) months. Fifteen (22.1%) patients died during follow up, median survival 19 (6.5–54) months. Estimated prevalence of IPF was 6.53/100,000 persons. Six (8.8%) patients were discussed at the Interstitial lung disease multi-disciplinary meeting. Resting Sp02 and end-of-life discussions were documented in 44 (64.7%) and 19 (27.9%) patients respectively, while oxygen therapy was prescribed to 15 (22.7%). 20/36 (55.5%) patients eligible for pirfenidone were treated. Those treated were more likely to have undergone a six-minute walk test (5/20 vs 3/48, p<0.05) or have been hospitalised in the last 12 months (12/20 vs 3/48, p<0.05). 7/20 patients remained on treatment at the end of follow-up (eight discontinued, five deceased).

Conclusion

In this study the estimated prevalence of IPF in the Canterbury region is 6.53/100,000 persons. Furthermore, we have identified limitations in local practice relevant for service development.

Author Information

James Fulforth, Senior Registrar, Department of Respiratory Medicine, Christchurch Hospital, Christchurch; Donna Thomson, Clinical Nurse Specialist, Department of Respiratory Medicine, Christchurch Hospital, Christchurch; Gordon Maxwell, Registrar, Department of Respiratory Medicine, Christchurch Hospital, Christchurch; Rachel Wiseman, Consultant Respiratory and Palliative Care Physician, Department of Respiratory Medicine, Christchurch Hospital, Christchurch;
Adrienne Edwards, Consultant Respiratory Physician, Department of Respiratory Medicine, Christchurch Hospital, Christchurch.

Correspondence

Dr Adrienne Edwards, Canterbury District Health Board, Canterbury Respiratory Service, Christchurch, Canterbury.

Correspondence Email

adrienne.edwards@cdhb.health.nz

Competing Interests

Nil.

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