Enfermedad pulmonar intersticial difusa por metaplasia peribronquiolar

Abstract

Introduction: Interstitial lung diseases are a group of rare diseases related to important morbidity and mortality. Peribronchiolar metaplasia, also known as lambertosis, could be found in some of these illnesses. Clinical case: We present a child with respiratory symptoms and need of oxygen since he was an infant. An interstitial alveolar infiltrate was seen in the computarized tomography. According to the clinic and imaging, the first diagnostic approach was an interstitial lung disease. The first lung biopsy was normal. He initially received treatment with cortico-steroids and bronchodilators during years, with no clear improving. A second lung biopsy was performed in which we found changes compatible with lambertosis. Finally, he started treatment with hidroxychloroquine and currently he has a normal life and rarely needs oxygen at home. Discussion: In our patient, despite the normal result of lung biopsy, the diagnosis of interstitial lung disease was not discarded because of the suggestive symptoms, signs and imaging tests. Lambertosis in adults is related to tobacco and infectious agents, but in children genetics seem to be important. The initial treatment were corticosteroids and then hidroxychloroquine. Conclusions: Although the diagnosis is histopathological, a normal lung biopsy does not discard an interstitial lung dis-ease. There is need of more clinical trials to establish the appropriate treatment for these patients.