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Current and Emerging Drug Therapies for Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD)

  • Adelle S. JeeEmail author
  • Tamera J. Corte
Review Article


Interstitial lung disease (ILD) can be associated with all connective tissue diseases and is an important cause of morbidity and mortality. The management of connective tissue disease-interstitial lung disease (CTD-ILD) is challenging due substantial heterogeneity in disease behaviour and paucity of controlled clinical trials to guide treating clinicians. Not all patients require treatment, and the decision to treat needs to be individualised based on a patient’s observed disease behaviour, baseline and longitudinal lung function measurements, extent of lung involvement on radiology and patient factors including age, co-morbidities and personal preference. If indicated, treatment of the CTD-ILD is largely with immunomodulation, with the aim to prevent progression of the ILD before further irreversible lung injury and disability occurs. Corticosteroids, cyclophosphamide, mycophenolate mofetil and azathioprine are the most common immunosuppressive agents currently used to treat CTD-ILD, demonstrating stability of lung function in case series and a small number of randomised controlled trials in ILD associated with systemic sclerosis. Biological and non-biological disease-modifying anti-rheumatic drugs, and the anti-fibrotics nintedanib and pirfenidone, have revolutionised the treatment of connective tissue diseases and idiopathic ILD, respectively. Furthermore, anti-fibrotics have recently demonstrated safety and efficacy in ILD associated with systemic sclerosis. There remains a critical unmet need to clarify when and in whom to initiate treatment, and which agent(s) to utilise to achieve optimal outcomes for CTD-ILD patients whilst minimising harms through prospective multicentre trials. This review highlights the challenges faced when treating patients with CTD-ILD and summarises available evidence for current, emerging and novel therapies.



ASJ would like to acknowledge financial support received through the Lung Foundation Australia/David Wilson Ph.D. Scholarship in Idiopathic Pulmonary Fibrosis Research.

Compliance with Ethical Standards


No sources of funding were used to assist in the preparation of this article.

Conflict of interest

Dr Adelle S. Jee declares travel fees, educational grant support and a speaker honorarium from Boehringer Ingelheim. Dr Tamera J. Corte declares consulting fees, advisory board membership, travel fees and speaker honorarium from Boehringer-Ingelheim and Roche, and unrestricted educational grants from Boehringer-Ingelheim, Roche, Bristol-Myers Squibb, Bayer and Galapagos.


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Respiratory and Sleep MedicineRoyal Prince Alfred HospitalSydneyAustralia
  2. 2.Central Clinical School, Faculty of Medicine and HealthUniversity of SydneySydneyAustralia

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