Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand*

Adelle S. Jee, Robert Sheehy, Peter Hopkins, Tamera J. Corte, Christopher Grainge, Lauren K. Troy, Karen Symons, Lissa M. Spencer, Paul N. Reynolds, Sally Chapman, Sally de Boer, Taryn Reddy, Anne E. Holland, Daniel C. Chambers, Ian N. Glaspole, Helen E. Jo, Jane F. Bleasel, Jeremy P. Wrobel, Leona Dowman, Matthew J.S. ParkerMargaret L. Wilsher, Nicole S.L. Goh, Yuben Moodley, Gregory J. Keir

Research output: Contribution to journalArticleResearchpeer-review

9 Citations (Scopus)

Abstract

Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmune-mediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, disease-specific approaches to treatment have been provided.

Original languageEnglish
Pages (from-to)23-51
Number of pages29
JournalRespirology
Volume26
Issue number1
DOIs
Publication statusPublished - Jan 2021

Keywords

  • clinical diagnosis and management
  • collagen vascular disease
  • connective tissue disease
  • interstitial lung disease

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