We appreciate the support of Teramoto and colleagues for the concepts outlined in our recent commentary in CHEST (August 2010),1 which include the importance of a multidisciplinary approach to evaluating interstitial pneumonia (IP) and emphasize the usefulness of specific autoantibodies, such as anti-cyclic citrullinated peptide (CCP), as part of the assessment for connective tissue disease-associated interstitial lung disease (CTD-ILD). Teramoto and colleagues also briefly describe a patient without synovitis who presented with organizing pneumonia and anti-CCP antibody positivity, who later developed the articular manifestations of rheumatoid arthritis (RA). In our and others’ experience,2 this is not an uncommon scenario, and it supports the notion that lung disease may be the first manifestation of a CTD and that surveillance for evolving extrathoracic features is needed when caring for patients with “idiopathic” IP. Interestingly, we have recently identified a sizable cohort of patients with anti-CCP positivity and IP without prior history of CTD or the articular features of RA.3 Although a few of these patients have developed the synovitis of RA within a short interval of follow-up, the vast majority have not. Because these patients with ILD have a highly specific autoantibody in the absence of defining extrathoracic CTD features, and because without inflammatory arthritis they cannot be defined as having RA, we have proposed that it is important to distinguish them from the category of idiopathic IP and consider them to have lung-dominant CTD.1 With prospective assessments of these types of more precisely characterized and classified phenotypes, we hope that important questions regarding their pathobiology, natural history, and therapeutic responsiveness will be answerable. Finally, we emphasize that a classification of lung-dominant CTD conveys that this entity is distinct from both idiopathic IP and from definite CTD-ILD and that these patients require surveillance for evolution to more defined forms of CTD.