Update on diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a disorder characterized by neuroendocrine cell hyperplasia, tumorlets and tumors, manifesting as lung nodules, chronic cough, and airflow obstruction. Often misdiagnosed as asthma, DIPNECH lies at the cross-section of oncology, obstructive airway disease and interstitial lung disease. With the increasing use of CT scans leading to higher rates of lung nodule detection, it is important for clinicians to be familiar with DIPNECH.

The evidence base in DIPNECH is sparse and limited to retrospective case series. In the last 5 years, clinical experiences in large academic centers have been published describing variability in clinical presentation and pulmonary function, use of DOTATATE scans, efficacy of somatostatin analogs, and principles of surveillance imaging.

More awareness of DIPNECH is needed among pulmonary clinicians. Apart from the usual presentation of cough with lung nodules and spirometric obstruction in women, patients also present with dyspnea and/or restrictive patterns on PFTs. Variability in diagnosis and management is widespread. Multidisciplinary assessment is helpful in guiding management. Multicenter and multispecialty collaboration is needed to establish best practices and improve clinical management.