SESSION TITLE: Obstructive Airway Disease Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 26, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: DIPNECH (Diffuse idiopathic pulmonary neuroendocrine hyperplasia) is a rare cause of COPD. We present a case of successful use of azithromycin for the same.
CASE PRESENTATION: A 68 year old female presented to the hospital with chronic shortness of breath of several years duration. She had been treated with different antibiotics and steroids, with only transient benefit. She had a history of smoking almost 30 pack year, but had quit for several years. PFT revealed severe obstruction with FEV1 of 0.95 liters (41% of predicted) with air trapping. HRCT of the chest revealed multiple bilateral irregular nodular opacities with areas of ground glass attenuation. ANA, ANCA, RF, ESR, CRP, fungal serologies were all within normal limits. Bronchoscopy with BAL and TBBX was done. Lavage fluid was negative for infection. TBBX was non-diagnostic. Pt was persistently symptomatic and a decision was made to perform open lung biopsy which showed diffuse neuroendocrine cell hyperplasia, with large aggregates consistent with tumorlets at places. None of the aggregates were however larger than 5 mm and hence didn’t classify as carcinoid tumors. The patient was subsequently started on low dose Azithromycin three times a week, in addition to her usual steroid, LABA and anticholinergic inhalers. Patient has had significant response to this regimen. She has currently been on this regimen for 4 years. Her FEV1 is stable, and she has had very infrequent exacerbations.
DISCUSSION: DIPNECH is a very rare disorder which presents in middle aged women, mostly nonsmokers with symptoms of COPD. Fewer than 50 cases have been reported in literature. Pulmonary function testing shows an obstructive pattern. CT chest usually shows a combination of pulmonary nodules, mosaic attenuation with ground glass and minimal bronchiectasis. Diagnosis is usually made on open lung biopsy, and demonstrates neuroendocrine cells with associated tumorlets in many cases. Upto 40% of patients are found to have carcinoid tumors. Most patients do well on oral or inhaled steroids. Patients have been treated with chemotherapy, surgical resection and lung transplantation.
CONCLUSIONS: To date, this is the first report of successful use of Azithromycin in this disease, which might act by its immunomodulatory effect. Further studies are needed before this can be accepted as a common practice.
Reference #1: Nassar AA et al. Am J Respir Crit Care Med. 2011;184(1):8-16.
Reference #2: Aubry MC et al. Chest. 2007;131(6):1635-43.
Reference #3: Davies SJ et al. Thorax. 2007;62(3):248-52.
DISCLOSURE: The following authors have nothing to disclose: Santosh Dhungana, James McCormick
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