Recent advances in connective tissue disease related interstitial lung disease

ABSTRACT

Introduction: Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD). Although the majority of patients with CTD-ILD are stable or slowly progressive, a significant group exhibits a more severe and progressive decline. Interstitial pneumonia with autoimmune features (IPAF) describes the subset of patients with interstitial pneumonia who have features suggesting underlying autoimmunity, but whose features fall short of a clear diagnosis of CTD.

Areas covered: In this focused review, we discuss recent advances in early detection, prognostic evaluation, and management of autoimmune forms of ILD.

Expert commentary: Early detection of ILD and a better understanding of factors that impact prognostication may be helpful when making decisions regarding therapeutic interventions. The treatment of CTD-ILD should be comprehensive, is often fraught with challenges and can be complicated by comorbid conditions and extra-thoracic disease activities. Several large randomized studies have examined the impact of immunosuppressive therapy for CTD-ILD, however, additional studies are needed to determine the optimal treatment strategies. Future studies may provide additional information about the best treatments in patients with IPAF.

KEYWORDS:

• Connective tissue disease
• interstitial lung disease
• CTD-ILD
• recent advances
• interstitial pneumonia with autoimmune features

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

This paper was not funded.