[A case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) syndrome].

INTRODUCTION DIPNECH is a strictly histological entity according to the WHO 2015 classification and is considered to be at pre-neoplastic risk. It has been proposed that DIPNECH syndrome should be used to describe patients have clinical symptoms, an obstructive ventilatory disorder and compatible radiological abnormalities. The diagnosis is histological and usually based on a surgical lung biopsy. CASE REPORT We report the case of a 58-year-old woman with a chronic cough for over 20years who had an obstructive airway pattern on spirometry. Diagnoses of asthma and COPD had been discussed. After 7years of follow-up, the DIPNECH hypothesis was evoked on the scanning aspect of mosaic attenuation, expiratory trapping and micronodules, which was subsequently confirmed by surgical pulmonary biopsy. CONCLUSION It is necessary to consider the possibility of this rare disease in order to avoid inappropriate treatments and in the hope that future therapeutic advances (somatostatin analogs, mTOR inhibitors) improve patients' experience and the progression of respiratory function.

• Publication year

  2019

• Venue

  Revue des maladies respiratoires

• Publication date

  2019-12-31

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/j.rmr.2019.10.011PMID 31901370
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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