Computed Tomographic Imaging in Connective Tissue Diseases

Joseph Barnett, FRCR; Anand Devaraj, MD, MRCP, FRCR


Semin Respir Crit Care Med. 2019;40(2):159-172. 

In This Article

Abstract and Introduction


Pulmonary disease represents a significant extra-articular manifestation in the majority of connective tissue diseases (CTDs). The identification, classification, and staging of pulmonary involvement are centrally important to the management of patients, aiding the prognostication of disease behavior and treatment decisions. We present a review of the high-resolution computed tomographic pulmonary features of CTD in the lung and their significance to the reporting radiologist.


Pulmonary involvement in connective tissue diseases (CTDs) represents a unique challenge in its variability. Disease can affect all compartments of the lung, namely, the airways, interstitium, pulmonary vessels, pleural space, cardiac tissues, and chest wall structures. Further diversity is encountered within each structure; within the interstitium, all classes of the idiopathic interstitial pneumonias can be encountered; and within the pulmonary arteries, many classes of pulmonary hypertension (PH) are attributable to CTDs. Furthermore, the chest can represent the first manifestation of a CTD in a significant minority of patients. The significance of pulmonary abnormalities in CTD is also highly heterogeneous, varying from incidental to life threatening.