Chromogranin-A Levels and Refractory Bronchospasm in Interstitial Lung Disease: A Preliminary Report on the Favourable Response to Octreotide

Aims: A wide variety of lung pathologies are associated with pulmonary neuroendocrine cell hyperplasia (PNECH).
Presentation of Case: We report two patients with interstitial lung disease (ILD), severe persistent bronchospasm not responding to conventional therapy, and raised chromogranin-A (Cg-A) levels. A neuroendocrine tumour (NET) was suspected and both were given a therapeutic trial of octreotide. This led not only to a dramatic clinical improvement but also to the normalization of Cg-A levels. Cg-A levels were elevated in 6 additional patients with interstitial involvement but without bronchospasm. The raised Cg-A levels in these 8 patients and the response to octreotide in two who had bronchospasm supports a diagnosis of PNECH in ILD.
Conclusion: Cg-A levels should be measured in all patients with ILD. An octreotide trial should be considered in symptomatic patients with interstitial lung disease and elevated chromogranin levels.