Answer to CC #14

Case Challenge #14 presented a 46 year old female presenting with a persistent cough for 10 years, no significant exposures, no smoking history, no travel, and an otherwise negative ENT, allergy, GI, speech, etc. work-up. PFTs reveal obstructive pattern, but standard COPD medications do not prove to be helpful. CT reveals bilateral nodular disease and mosaicism. Biopsy of the lung reveals neuroendocrine cell hyperplasia which stain for carcinoid.

What is the best initial management strategy?

Serial CT Scans! The patient has DIPNECH (Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia)

  • Definition: Hyperplasia of bronchial neuroendocrine cells in airway epithelium with aggregates of cells (“tumorlets”) extending beyond the basement membrane
    • first described in 1992 (Aguayo et al, NEJM)
    • typically female nonsmokers, mean age 45 years
    • absence of other pulmonary processes or environmental exposures to explain symptoms
  • Presentation:
    • years of cough, +/- wheeze and dyspnea
    • PFTs: irreversible airflow obstruction & a third of patients had restriction
    • CT: multiple nodules and air-trapping due to bronchiolitis. Moasaicism.
  • Management: “…longterm follow-up and treatment remains incomplete…” (Am J Resp Crit Care 2011)
    • COPD management: BA +/- inhaled or systemic steroids for symptomatic relief
    • Serial CTs to watch for development of carcinoid tumors
      • “Given its only minor risk of invasive metastatic spread, serial imaging for timely detection of progressing lesions and optimizing of concomitant obstructive pulmonary disease are the preferred treatment approaches.” (Dtsch Med Wochenschr. 2014 Jun;139(23):1245-8. doi: 10.1055/s-0034-1370073. Epub 2014 May 27.)
    • There is some evidence that somatostatin analogues are helpful, but long-term safety and efficacy data are lacking

Case challenge #14 (aka the Parkland Files!) will be posted next week!