Diffuse idiopathic pulmonary endocrine cell hyperplasia (DIPNECH) requiring bilateral sequential lung transplant

Introduction: DIPNECH is a rare disorder with only around 50 cases published. It is characterised by abnormal proliferation of neuroendocrine cells along respiratory epithelium. Case Study: A sixty-three year old lady, an ex-smoker, underwent bilateral sequential lung transplant for presumed emphysema in November 2011. She was first referred to a Respiratory Physician in 1993 with persistent cough and dyspnoea and occasional hamoptysis. An initial diagnosis of asthma was made, later replaced by COPD. Her breathlessness increased with severe disability. She commenced oxygen therapy in 1997 and she developed type 2 respiratory failure in 2000. Disabling breathlessness prompted referral for lung transplantation in 2008. Histology of the explanted lungs showed DIPNECH. Discussion: DIPNECH occurs predominantly in non-smoking, middle-aged women. Presenting symptoms include cough and dyspnoea. Diagnosis is often delayed. A lack of diagnostic radiological features makes tissue biospy the gold standard for diagnosis. Outcomes in a previous Systematic Overview were variable with many patients achieving clinical stability or improvement¹. Our case was characterised by a progressive decline in lung function and respiratory failure. Conclusion: We present the first confirmed case study in medical literature of a patient undergoing bilateral lung transplantation for DIPNECH. References: 1. Nassar A. Jarpszewslo E. et al.Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia. A systematic Overview. Am J Respir Crit Care Med 2011 Vol 184. pp8-16.