Diagnosis of Respiratory Bronchiolitis associated interstitial lung disease

Abstract

Background. Respiratory Bronchiolitis (RB), described by Niewoehner et al in 1974, is a common finding in heavy smokers and is characterised by the presence of pigmented macrophages within respiratory bronchioles and adjacent alveoli. In the 1980s, Myers et al described RB associated with interstitial lung disease (RBILD), which is a rare entity expressed as an amplified respiratory bronchiolitis in response to cigarette smoke. Methods. The authors studied a group of 8 patients with biopsy-proven RBILD diagnosed within the last five years, and assessed epidemiological data, clinical and imaging features, lung function tests, bronchoalveolar lavage findings, therapeutic approaches and clinical evolution. Results and Conclusions. The most difficult differential diagnosis is between RBILD and Desquamative Interstitial Pneumonia (DIP), which seems to suggest that these disorders are either end of the same spectrum, although the authors claim that there are some clinical, morphological and prognostic distinctions. The diagnosis of RBILD requires an appropriate clinical setting (including smoking habits), characteristic image findings (like ground glass shadowing and centrilobular nodules), and Bronchoalveolar Lavage (BAL) data to exclude other diagnosis. Pathological confirmation may also be important, not only to exclude more adverse interstitial lung disease, such as idiopathic pulmonary fibrosis, but also, as in the eight cases presented, to illustrate some specific features such as the prevalence of lymphocytosis in BAL.