No abstract

Purpose of review Interstitial lung disease (ILD), though a common and often a severe manifestation of many connective tissue diseases (CTD), is challenging to manage due to its variable presentation and the relative lack of guidelines to assist the clinician. In this review, we discuss the approach to diagnosis, treatment, and monitoring patients with CTD-associated ILD, with a focus on systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathy (IIM). Recent findings High-resolution CT scan and pulmonary function testing can be reliably used to diagnose ILD and monitor progression, and often to determine its likely histologic subtype and severity. In SSc-ILD, randomized controlled trials show ILD stabilization with cyclophosphamide treatment; preliminary data from another randomized controlled trial demonstrates similar findings with mycophenolate. There are no robust clinical trials supporting specific treatments for RA-ILD or IIM-ILD, but rituximab in RA-ILD, and cyclophosphamide, mycophenolate and calcineurin inhibitors in IIM-ILD show promise. Summary Though ILD contributes substantially to morbidity and mortality in patients with CTD, there is minimal data to guide its management except in SSc-ILD.

Imaging plays an integral role in the diagnosis and management of suspected pulmonary infections and may reveal useful signs on chest radiographs and CT scans. Detected early, these signs can often be used to predict the causative agent and pathophysiologic mechanism and possibly to optimize patient care.